Acute hemiplegia in childhood …

Stroke & Vascular Disorders

Updated 10.10.2023
Released 06.19.1995
Expires For CME 10.10.2026

Acute hemiplegia in childhood

Authors: Uluc Yis MD, Haluk Topaloglu MD

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Editor: Nina F. Schor MD PhD

Acute hemiplegia in childhood

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Introduction

Overview

Acute hemiplegia in childhood is a diagnostic and management challenge for the clinician. Hemiplegia is a total paralysis of arm, leg, and sometimes the face on 1 side of the body whereas hemiparesis is partial paralysis on 1 side of the body. Hemiplegia/hemiparesis is not a disease but a response of the central nervous system to a variety of insults. Underlying etiologies are more diverse in children than in adults. This review is a clinical approach to the child with acute hemiplegia. It includes a staged approach towards clinical assessment, diagnostic workup, and management of specific causes of hemiplegia.

Key points

	• Acute hemiplegia in children is a clinical syndrome with various causes.
	• The immediate priority is to exclude a neurosurgical condition like intracranial hemorrhage, brain tumor, hydrocephalus, and massive ischemic stroke.
	• Acute hemiplegia in children is the most common presentation of vascular stroke syndromes.
	• About 20% to 30% of children with acute hemiplegia have “stroke mimics” like hemiplegic migraine, alternating hemiplegia, Todd paralysis, reversible vasoconstriction syndrome, posterior reversible encephalopathy, and conversion disorder.
	• Clinical data and neuroimaging help to establish the diagnosis in most of the cases.
	• Management and prognosis of acute hemiplegia in children depend on the etiology.

Historical note and terminology

The occurrence of unilateral weakness related to contralateral brain injury was already familiar to ancient physicians like Hippocrates and Aretaeus. Jusepe Ribera, a 17th century Spanish artist, painted a portrait of young soldier with hemiplegia. Early observations of acute hemiplegia were based on experience with penetrating head injury, intracranial hemorrhage, and epileptic seizures. In the late 18th century, Darwin experimented with electrical therapy for children with hemiplegia (40). Todd described a post-epileptic hemiplegia in 1865 (91), and in 1887 Freud described acute childhood hemiplegia associated with epilepsy (73). In 1916, Higier described hemiplegic seizures (45). Seminal papers by Bickerstaff (11), Aicardi and colleagues (02), and Carter (84; 46) focused mainly on childhood stroke and heralded the modern approach to evaluating acute childhood hemiplegia, caused by stroke. Our knowledge of the causes and treatment of both transient and permanent acute childhood hemiplegia have increased exponentially in the past decade due in no small measure to the International Pediatric Stroke Study initiative (58). In addition, structural and functional brain MRI, as well as traditional and MR angiography, have contributed to our understanding of the multiple causes and pathophysiology of acute hemiplegia in childhood. Advances in genetics have enabled us to understand the pathophysiology of familial hemiplegic migraine and alternating hemiplegia of childhood.

Clinical manifestations

Presentation and course

Clinical manifestations, course, and outcome of acute hemiplegia vary depending on the etiology.

Stroke. Acute hemiplegia, a common presentation of arterial ischemic stroke (60; 39), appears suddenly in 50% of cases but is subacute (evolving over hours) in 35% and stuttering in onset in 15% (30). Arteriopathy in contrast to other causes of stroke almost always presents with hemiparesis as opposed to multifocal or bilateral symptoms (59). Subacute onset also suggests an underlying arteriopathy (13). Duration of hemiplegia varies from days to weeks; deficits can be permanent. Etiology and location of lesion determine outcome. Seizures and headache may precede, present with, or follow arterial ischemic stroke in as many as a third of patients or more (30; 18; 43; 01; 83) and may delay the proper diagnosis (82). Seizures are particularly common in children younger than 1 year of age who may or may not have an apparent hemiparesis (97). Other neurologic signs can co-occur with hemiplegia, eg, hemisensory or visual deficit, aphasia, or constructional apraxia, depending on the location and type of the lesion. When seen in cerebral venous sinus thrombosis with associated infarction, acute hemiparesis is often accompanied by altered consciousness, headache, and seizures. Predisposing conditions, eg, dehydration and systemic diseases, are common (27).

Hemorrhage. Hemorrhage can occur in the setting of stroke (arterial or venous), vascular malformations and aneurysm, neoplasms, congenital heart disease, head and neck trauma, and hematologic disorders. Hemiplegia is generally abrupt in onset, except when it occurs in the setting of hemorrhagic stroke. Headaches and seizures are common (57; 18). In younger children, the clinical presentation may be nonspecific, whereas in older children (over 6 years of age) a focal deficit or hemiplegia is the rule (57).

Hemiplegic migraine. Hemiplegic migraine usually has a slowly progressive onset evolving over 5 to 30 minutes, but in 5% to 10% of cases onset is abrupt, peaking in less than 1 minute (90). In about 15%, the hemiplegia shifts sides during an attack. Weakness lasts less than 1 hour in 51% of cases, 1 to 24 hours in a subsequent 41%, and more than 24 hours in an additional 8% (90). In hemiplegic migraine, positive symptoms (particularly paresthesias) often precede the hemiplegia (41), and more typical auras (visual, sensory, or dysphasic) often co-occur with the hemiplegia. Headache can precede or occur at the same time as the aura or follow it--generally within an hour. A structural lesion needs to be ruled out as both arterial ischemic stroke and cerebral venous sinus thrombosis occur in migraineurs, at least in adulthood, at a greater frequency than in the general population, and headache, especially in children, can occur at the onset of stroke. It is not known whether prolonged aura (more than 1 hour but less than 7 days) or persistent aura (more than 7 days) increases the risk for stroke. However, hemiplegic migraine does not specifically confer a greater risk of stroke than migraines with other auras (51).

Seizures: ictal and Todd postictal paralysis. Todd postictal paralysis is identified by its context; it occurs after a seizure. Duration ranges from minutes to days but in most instances is less than 30 minutes (38). Hemiplegia lasting more than 6 hours is unusual and should not be assumed to be a Todd paralysis (41). However, Todd himself reported episodes lasting for days (91). Rarely, focal weakness may be the only manifestation of a seizure (72; 71). A hemiconvulsion-hemiplegia-epilepsy syndrome has been described and is characterized by a prolonged clonic seizure with unilateral predominance occurring during a febrile illness and followed by hemiplegia (05). Mutations in sodium channel genes SCN1A and SCN2A are a predisposing factor for this syndrome. It is hypothesized that altered channel activity resulting from these mutations provokes both seizures and subsequent excitotoxic brain damage (80).

Demyelinating disorders. Onset of hemiplegia in disorders affecting white matter, such as acute disseminated encephalomyelitis and leukoencephalopathy due to toxin or medication side effects, is more often subacute than acute and is often associated with an encephalopathy.

Conversion disorder. In conversion disorder, the hemiplegia usually appears suddenly and is typically temporally related to a psychological stressor (04). Other psychiatric disorders (ie, depression and anxiety) may coexist.

Asthmatic amyotrophy. Sudden, flaccid paralysis of 1 or more limbs may occur during recovery from an asthmatic attack. It resembles poliomyelitis. The interval between the asthmatic attack and the paralysis is 1 to 11 days. Monoplegia frequently involving the arm occurs in 90% of cases whereas 10% have hemiplegia or diplegia. Sensation is intact but there is pain in the paralyzed limb in 50% of cases. Recovery is incomplete (56).

Diabetes mellitus. Acute transitory attacks occur in children with insulin-dependent diabetes mellitus. Attacks frequently occur during sleep. Hemiparesis is present on awakening and lasts for 3 to 24 hours. Weakness is greater in the face and arm. Headache is a constant feature. Recovery is complete (67).

Alternating hemiplegia of childhood. Onset is from birth to 18 months. Mild developmental delay and abnormal eye movements are initial features. Young infants have more dystonic features and older children have flaccid hemiplegia. Brief attacks of nystagmus accompany attacks. Duration of attacks varies from minutes to days. Hemiplegia may shift from side to side. Arm is generally more severely affected than leg. Hemiplegia disappears with sleep. Neurologic impairment occurs as the disease progresses (88). Adults with this disorder can have other manifestations, including parkinsonism (see MedLink Neurology article Alternating hemiplegia of childhood).

Meningitis/meningoencephalitis. The clinical manifestations of CNS infection differ between the meningitic form and the encephalitic form. Fever, headache, vomiting, and stiffness of the neck dominate the meningitic form and altered state of consciousness, focal neurologic signs (including hemiplegia), and seizures dominate the encephalitic form.

Reversible cerebral vasoconstriction syndrome. Reversible cerebral vasoconstriction syndrome is a clinical and radiographic syndrome that is characterized by thunderclap headache, seizures, and focal neurologic deficits. There is narrowing of the cerebral arteries on cerebrovascular imaging. This usually diminishes within 12 weeks after the onset of symptoms. Reversible cerebral vasoconstriction syndrome is associated with stroke (ischemic or hemorrhagic) in 10% of cases. It is frequently associated with ingestion of vasoactive drugs such as nasal decongestants (29).

Posterior reversible encephalopathy syndrome. Posterior reversible encephalopathy syndrome is an emerging neurologic disease characterized by headache, altered mental status, visual disturbance, seizures, and focal neurologic findings including hemiplegia. Hypertension, cytotoxic agents, and corticosteroids are well-identified triggers. When it is managed in a timely fashion, the prognosis is benign with complete recovery within days or weeks. Typical radiographic findings include vasogenic edema in posterior cerebral regions (75).

Metabolic or genetic disorders. Metabolic abnormalities such as hyper- and hypoglycemia and hypocalcemia result in inadequate energy supply to neurons. Inherited metabolic disorders can cause stroke-like episodes with hemiplegia, which are mediated by cellular energy failure, for example, in mitochondrial disorders like MELAS (mitochondrial encephalopathy and lactic acidosis syndrome) and organic acidemias. Disease-specific mechanisms for stroke have been documented in some genetic disorders (89; Testai and Gorelick 2010b; 68).

Prognosis and complications

Outcome after acute hemiplegia is highly variable and depends on the underlying cause. Pooled data from several studies of children with arterial ischemic stroke indicate that 30% were neurologically normal, 61% had motor or cognitive problems, and 9% died (58). Epilepsy develops in 10% to 15% of children with arterial ischemic stroke (25). Following intracerebral hemorrhage, 42% of children are normal and 32% have persistent deficits, with 25% mortality, and epilepsy in 11% (58). Imaging studies suggest that acute involvement of the descending corticospinal tract on diffusion-weighted imaging is a harbinger of poor motor outcome (28). AHE causes mild to severe cognitive problems. Almost all affected children have some level of developmental delay and intellectual disability (88). Prognosis of intracranial infections and acute demyelinating disorders is excellent if diagnosed and treated early. Most patients with reversible cerebral vasoconstriction syndrome show resolution of symptoms within days to weeks. Fifteen to twenty percent are left with residual deficits from stroke (47). Prognosis in posterior reversible encephalopathy syndrome is usually excellent. Symptoms are most often fully reversible after removal of the inciting factor and control of the blood pressure. Serious neurologic disability and death have also been reported (Raj et al 2013; 81). Rarely, some patients develop epilepsy after recovery. The prognosis is asthmatic amyotrophy is uniformly poor (56). Most of the patients later develop marked limb amyotrophy and remain handicapped. The outcome in hemiconvulsion hemiplegia epilepsy syndrome depends on the cause and immediate seizure control. Most patients develop epilepsy and attacks of status epilepticus. Cognitive deficits are the rule (05). In contrast, the outcomes in hemiplegic migraine, acute hemiplegia in diabetes mellitus, Todd postictal paralysis, and conversion disorder are generally excellent.

Clinical vignette

A 9-year-old girl presented with sudden onset right-sided weakness. She was previously healthy and had no history of recent trauma, infection, or vaccination. There was no family history of premature stroke, myocardial infarction, or deep venous thrombosis. The patient was afebrile and vital signs were normal. Blood pressure was 90/60 mmHg. On neurologic examination, she was fully conscious and cooperative. Cranial nerve examination revealed left central facial paralysis. Strength in the right upper and lower extremities was normal (5/5), but greatly diminished on the left (1/5). Deep tendon reflexes were hyperactive on the left and left plantar response was extensor. Skin examination revealed no café-au-lait spots, neurofibromas, or freckling of the groin or the axilla. The remainder of the physical examination was normal. Diffusion-weighted magnetic resonance imaging revealed diffusion restriction in the right putamen, nucleus caudatus, and posterior limb of the internal capsule consistent with acute infarction. Time of flight magnetic resonance angiography showed beading and decrease in caliber in the right supraclinoid segment of the carotid artery and middle cerebral artery. In the superior-anterior part of the M1 segment of the middle cerebral artery, there seemed to be a second lumen with a signal intensity lower than that of the primary lumen but higher than that of the parenchyma. These findings suggested dissection superimposed on fibromuscular dysplasia in the middle cerebral artery, which compromised the origin of the lenticulostriate arteries and led to striatocapsular infarct. Digital subtraction angiography performed 1 week after the MRA showed the typical “string of beads” appearance of fibromuscular dysplasia in the right supraclinoid segment of the carotid artery, M1 and M2 segment of middle cerebral artery, and A1 segment of anterior cerebral artery. Dissection, however, was not confirmed. It was assumed that the dissected intima had sealed during the interval between the MRA and the digital subtraction angiography or that the second lumen appearance resulted from motion artifacts. Abdominal magnetic resonance imaging of the patient was normal. Complete blood count, coagulation parameters, antiphospholipid antibodies, antithrombin III, protein C, protein S, and homocysteine were normal. Factor V Leiden, methylene tetra-hydrofolate reductase, and prothrombin G20210A mutations were negative. Serum cholesterol, triglycerides and low- and high-density lipoproteins (LDL, HDL) were 303 mg/dl (122-209), 122 mg/dl (35-114), 221 mg/dl (60-150), and 58 mg/dl (35-84), respectively. Cardiac examinations, including echocardiography and electrocardiography, were normal. The lipid profile of both parents also showed increased levels of cholesterol, triglycerides, and LDL. Familial combined hyperlipidemia was suggested, and a low-cholesterol, low-saturated fat diet was started. Aspirin at a dosage of 3 mg/kg/day was started for its antiplatelet effect and physical rehabilitation was instituted for hemiparesis. No further strokes appeared in the follow-up period of 6 months, and at present, she has mild hemiparesis and is able to walk independently.

Biological basis

Anatomic localization

The corticospinal tracts arise from somatotopically organized areas of primary motor cortex, lateral premotor cortex, and supplementary motor area as well as the primary sensory cortex in the postcentral gyrus, ie, the anterior paracentral gyrus, superior parietal lobule, and areas of the cingulate gyrus. The corticospinal tract descends in the corona radiata, the posterior limb of the internal capsule, the middle three fifths of the cerebral peduncle, the pons, and the medullary pyramids. Thus, lesions in any of these areas can result in hemiparesis of varying degree and distribution. Corticospinal neurons within the motor cortex are somatotopically organized in accordance with their functional importance; the size of the cortical representation in the motor homunculus varies with the functional importance of the body part represented. For example, isolated hand weakness of cortical origin may present with loss of thumb and finger movements and impaired hand flexion and extension, or it may occur with only partial involvement of a few digits. The corticospinal tract is also organized somatotopically in the posterior limb of the internal capsule with hand fibers lateral and slightly anterior to foot fibers, in the pons with fibers controlling the proximal muscles placed more dorsally than those controlling more distal muscles, and in the medullary pyramids with fibers of the lower extremities placed more laterally and decussating more rostrally than those of the upper extremities. Depending on the location of the lesion relative to the corticospinal tract pathway, a hemiparesis of vascular origin may predominantly involve the upper or lower extremity with or without other associated findings, except for the pure motor hemiparesis of pontine lesions where the deficit is equally arm and leg and spares the face (14). Cervical cord lesions can also cause hemiparesis secondary to compression of the corticospinal tracts.

Pathophysiology

Acute hemiplegia in childhood is caused by an alteration in cerebral metabolism in a brain region involved in contralateral motor function. The specific mechanism of this metabolic derangement varies depending on the underlying cause.

Stroke. The pathogenesis of childhood stroke is multifactorial and can be divided into several categories: cardiac, extracranial arteriopathies, intracranial arteriopathies, thrombophilia, sickle cell disease, and systemic causes. Cardioembolic stroke occurs as a result of congenital heart disease, acquired heart disease, and procedure-related events. The pathophysiology of stroke in children with cardiac disease is usually thromboembolic. Extracranial arteriopathies are usually caused by craniocervical arterial dissection. Dissection promotes platelet activation and activation of clotting cascade in the intimal layer of the artery. Dissection also causes aneurysmal dilatation of the affected artery. The most common cause of childhood stroke is intracranial arteriopathies. They are mainly divided into dissection and inflammatory types. Dissection type occurs in the anterior circulation, usually with trauma. Inflammatory type is a focal vasculitis. An infectious or parainfectious process leads to localized vessel inflammation and secondary thrombus formation. Progressive disease of the distal middle cerebral artery/proximal internal carotid artery is called moyamoya disease. Moyamoya disease is associated with 6% to 10% of childhood strokes and transient ischemic attacks. Thrombophilia leads to hypercoagulable states resulting from inherited or acquired conditions. In fact, protrombotic conditions may act as triggers for stroke in the presence of other risk factors. Systemic causes of childhood stroke include inflammatory causes and genetic/metabolic syndromes. Typical examples include primary angiitis of the central nervous system, systemic lupus erythematosus, and polyarteritis nodosa. Clues for inflammatory causes include proteinuria, presence of persistent serum inflammatory markers, frequent fevers, and livedo reticularis. Genetic and metabolic causes of stroke are rare. ACTA2 syndrome, COL4A1 mutations, and PHACE syndrome are specific syndromes. Stroke in mitochondrial disorders do not follow typical arterial distributions (32). Common causes of pediatric stroke are shown in Table 1.

Table 1. Common Causes of Pediatric Stroke

	Cardiac	Patent foramen ovale Congenital cardiac anomaly Acquired cardiac anomaly Arrhythmia
	Arteriopathy	External dissection Focal cerebral arteriopathy inflammation type Focal cerebral arteriopathy dissection type Moyamoya disease Fibromuscular dysplasia ACTA2-related vascular disease Dynamic vertebral artery compression/V3 pseudoaneurysm
	Thrombophilia	Inherited thrombophilia Acquired thrombophilia
	Inflammatory	Lupus Takayasu arteritis Childhood primary angiitis of central nervous system Adenosine deaminase deficiency Polyarteritis nodosa Wegener disease P-ANCA, C-ANCA related vasculitis
	Genetic/metabolic	PHACE syndrome Fabry disease MELAS POLG1
	Connective tissue disorder	Ehler Danlos type IV Loeys-Dietz syndrome

Hemorrhage. Hemorrhage causes hemiplegia as a result of mass effect or by specific involvement of the corticospinal tracts. Hemorrhages can be caused by vascular malformations (about 50%), brain tumors (10%), cardiac disease, hematological disorders, and medical illnesses (25%) (57). In some cases, there is no definable cause. Intraparenchymal hemorrhages are generally due to vascular malformations or brain tumors (57). Saccular aneurysms are 3 times more likely to hemorrhage than fusiform aneurysms (44). Subdural hemorrhages generally occur in the setting of hematological disorders or cardiac surgery. Subarachnoid hemorrhage is not associated with specific risk factors (57). Sturge-Weber syndrome has been associated with spontaneous intracranial hemorrhages in some children (69).

Metabolic or genetic disorders. Metabolic abnormalities such as hyper- and hypoglycemia and hypocalcemia result in inadequate energy supply to neurons. Inherited metabolic disorders can cause stroke-like episodes with hemiplegia, which are mediated by cellular energy failure, for example, in mitochondrial disorders like MELAS and organic acidemias. Disease-specific mechanisms for stroke have been documented in some genetic disorders (89; Testai and Gorelick 2010b; 68).

Hemiplegic migraine. Migraine auras of all types are due to cortical spreading depression characterized by brief neuronal excitation, which initiates a depolarization wave that moves across the cortex at a rate of 3 to 5 mm/minute and is followed by a prolonged inhibition of neuronal activity. Whether hemiplegic migraine is qualitatively different from other migraines with aura is debatable. Mutations in 1 of 3 genes, ie, voltage-gated channels CACNA1A and SCN1A genes and the Na/K pump ATP1A2 gene, are responsible for 50% to 70% of familial hemiplegic migraine (79). These genes are inherited in an autosomal dominant pattern; the child of a parent with familial hemiplegic migraine has a 50% chance of inheriting the mutation.

Seizures: Todd postictal hemiplegia and ictal hemiplegia. “Neuronal exhaustion” is considered the most likely cause of Todd postictal hemiplegia. Todd believed in the context of his electrical theory of epilepsy that the hemiplegia resulted from undue exultation resulting in a state of depression or exhaustion (12). Excessive inhibition is another proposed mechanism. Epileptic activity in the supplementary motor area or somatosensory cortex (23) or temporoinsular area (94) has been implicated in epileptic hemiparesis. Periodic lateralizing epileptiform discharges on EEG may be disproportionately frequent during ictal hemiparesis. Although the pathophysiological mechanism of hemiconvulsion-hemiplegia-epilepsy syndrome is not known, there are several factors that are believed to contribute to the pathogenesis. The proposed mechanism is neuronal lesion induced by venous thrombosis and/or by neuronal toxicity. The role of cerebral malformation and/or cortical dysplasia has also been suggested. Inflammation and hyperthermia are known to worsen status epilepticus and prolonged status epilepticus damages the blood-brain barrier. The unilaterality may be due to regional maturation of the corpus callosum, focality of a neurotropic virus, and genetic predisposition (05). New research has identified missense mutations in sodium channel SCN1A and SCN2A that predispose children to encephalopathy with severe febrile seizures (88; 80).

Demyelinating disorders. Demyelination in the cerebral white matter or in the spinal cord results in hemiplegia primarily by slowing conduction through motor fibers.

Infection. Meningitis and encephalitis can cause hemiplegia via destruction of brain parenchyma, abscess formation, local inflammation, or vasculopathy (19).

Neoplasm. A tumor may cause hemiplegia through compression of brain or cervical cord structures responsible for motor control by the mass lesion itself or surrounding edema or hemorrhage.

Head or neck trauma. Head trauma can cause acute hemiplegia through a variety of mechanisms, including shearing injury; intraparenchymal, epidural, subdural, or subarachnoid hemorrhage; or ischemic injury, which can be a primary effect due to dissection or secondary to vasospasm following subarachnoid hemorrhage. Repeated head trauma, as occurs in the setting of contact sports, may increase the risk of brain ischemia over time due to long-term vascular and neuronal changes (15). Acute cervical cord compression causing hemiplegia may be spontaneous or the result of trauma (36).

Toxin or medication side effects. Toxic agents produce stroke via a range of mechanisms like vasculitis (eg, cocaine) or cause hemiplegia through myelin and axon loss, gliosis, and necrosis (eg, methotrexate leukoencephalopathy) (53).

Conversion disorders. Atypical activation in motor cortex, basal ganglia, and thalamus in response to paretic limb stimulation (55) and atypical connectivity between the amygdala and the supplementary motor area (95) as well as between the ventromedial and dorsolateral prefrontal cortex (24) are present in patients with conversion disorder. Motor mental imagery is impaired (16). Together these data suggest an atypical relationship between emotion and motor function (95).

Posterior reversible encephalopathy syndrome. Previous reports suggest that vasogenic rather than cytotoxic edema plays a more significant role in the pathophysiology. When hypertension exceeds cerebrovascular autoregulatory limits, it results in endothelial dysfunction and failure of compensatory vasoconstriction to prevent hyperperfusion and subsequent fluid extravasation. Because sympathetic innervation is relatively scant in the posterior circulation, parieto-occipital lobes are affected frequently. Antineoplastic and immunosuppressive drugs may also cause a direct toxic effect on the cerebrovascular endothelium (37).

Reversible cerebral vasoconstriction syndrome. The pathophysiology of the abrupt-onset headache and the prolonged-reversible vasoconstriction in RCVS is not known. An abnormality of the cerebrovascular tone is suggested. Cerebral vasoconstriction may result in ischemic stroke or hemorrhages in some cases (93).

Alternating hemiplegia of childhood. About 70% of cases are caused by mutations in ATP1A3 gene. Other genes which cause alternating hemiplegia of childhood or similar symptoms include the CACNA1A, SLC1A3, and ATP1A2. The ATP1A3 gene is responsible for the production of ATPase, Na+K+ transporting, that is required for the normal function of nerve cells in the brain. This protein plays a critical role in the transport of sodium and potassium ions across a channel that connects nerve cells which helps to regulate brain activity. Alternating hemiplegia of childhood is therefore a channelopathy (62).

Global perspective. A retrospective review of 25 children with acute hemiplegia admitted to the Neurology Unit of the Department of Pediatrics of University of Calabar Teaching Hospital in Nigeria showed that prolonged seizures, speech defect, cranial nerve deficit, and loss of consciousness were the most common clinical findings (31). Viral encephalitis was the most common etiology, followed by meningitis and sickle cell anemia. Only 16% of the patients recovered completely, 8% died, and 76% had varying degree of weakness.

Differential diagnosis

The differential diagnosis of acute hemiplegia is extremely broad, although stroke, migraine, and Todd postictal paralysis are the most common causes (Table 2).

Table 2. Differential Diagnosis of Acute Hemiplegia in Children

Condition
Intracranial hemorrhage	Abrupt onset, signs of increased intracranial pressure	Rapid evolution of neurologic findings, altered state of consciousness, neurocutaneous signs	Imaging, CT is preferred in acute setting
Stroke	Abrupt or stuttering onset. Preceding infections, cardiac pathology, head-neck trauma	Focal neurologic findings, consciousness depends on the extent of infarct area	Imaging, MRI is preferred, vascular imaging of brain and neck
Intracranial infection	Fever, headache	Signs of meningeal irritation	Cerebrospinal fluid examination after excluding increased intracranial pressure
Acute demyelinating conditions	Subacute onset, altered state of consciousness, fever, headache	Encephalopathy, signs of meningeal irritation, focal neurologic findings	Imaging, MRI is preferred, cerebrospinal fluid examination
Posterior reversible encephalopathy	Seizures, drugs (tacrolimus, cyclosporine A, cyclophosphamide)	Hypertension, altered state of consciousness	Imaging, MRI is preferred
Bleeding or edema associated with CNS tumor	Subacute history of neurologic symptoms followed by acute change	Altered state of consciousness, focal neurologic findings	Imaging, CT is preferred in acute setting
Metabolic disease	Hypotonia, psychomotor retardation, vomiting, encephalopathy, recurrent cases in a family, consanguinity	Movement disorder, seizure	Imaging, MRI is preferred, MRI spectroscopy, extensive metabolic investigations
Reversible cerebral vasoconstriction	Thunderclap headache, history of vasoactive drugs	Altered state of consciousness, focal neurologic findings	Vascular imaging
Hemiplegic migraine	Recurrent headache, family history	Hemiparesis	Imaging, MRI with vascular imaging, genetic testing
Postictal state	Focal motor seizure, history of epilepsy	Focal neurologic findings	Imaging in first seizure, electroencephalograpy
Conversion disorder	Disproportionate effect on function	Findings not conforming to a neuroanatomical condition	Normal examinations
Alternating hemiplegia	Developmental delay, abnormal eye movements	Binocular or monocular nystagmus, hemiplegic and dystonic attacks shifting from side to side, disappearing with sleep	Normal imaging and electroencephalography, genetic testing
Asthmatic amyotrophy (Hopkins syndrome)	History of asthmatic attack	Monoplegia in 90% of cases with arm involved twice as often as the leg, 10% have hemiplegia, sensation is intact	Imaging of brain, cervical spine and brachial plexus, electromyography, cerebrospinal fluid examination
Acute hemiparesis in diabetes mellitus	History of insulin-dependent diabetes mellitus	Headache, hemiparesis on awakening, sensation is intact, recovery in 3 to 24 hours	Normal imaging and electroencephalography

In the differential diagnosis of hemiplegia in children, the clinician should first distinguish between peripheral and central deficits. A competent neurologic examination is a key. Facial weakness, encephalopathy, and seizures should point the clinician to central nervous system. Increased tone and reflexes on the affected side are late signs in hemiplegia due to central deficits. Partial Bell palsy, sparing the upper face, can be mistaken for an ipsilateral upper motoneuron facial palsy. Facial palsy contralateral to the hemiplegia localizes the lesion to the pons. The signs in younger children are more diffuse and the clinician should be careful for asymmetry of movements, tone, posture, and reflexes. The immediate priority in the acute setting is to identify a neurosurgical emergency such as intracranial hemorrhage, malignant ischemic stroke, brain tumor, and hydrocephalus. An abrupt onset with headache and coma is suggestive for intracranial hemorrhage. Ischemic stroke may have a stuttering onset. Headache is common in hemiplegic migraine, central nervous system infections, and sinovenous thrombosis. Cases with hemiplegic migraine also have more typical auras (visual, sensory, or dysphasic) often cooccurring with the hemiplegia. Encephalopathy is common in central nervous system infections and acute demyelinating disorders and should be perceived as a warning sign in cases of arterial ischemic stroke who develop malignant middle cerebral artery syndrome. Thunderclap headache is common in cerebral vasoconstriction syndrome and subarachnoid hemorrhage. Children with moyamoya disease have transient ischemic attacks that are triggered by hyperventilation and dehydration. During attacks of alternating hemiplegia, paralysis alternates form 1 side of the body to the other. Symptoms completely disappear with sleep and reappear with awakening. Seizures are often the presenting manifestation associated with visual disturbances in posterior reversible encephalopathy syndrome (10).

Diagnostic workup

History and clinical examination provide crucial clues about the cause of hemiplegia. Both family and personal history are relevant. Funduscopic examination may reveal signs of retinal hemorrhage, suggesting cerebral trauma, as well as signs of a genetic disorder like Lisch nodules or cataracts. Examination of the oropharynx and neck may reveal evidence of injury or inflammation. Examination of the heart facilitates the diagnosis of cardiac disease. Delayed or diminished femoral pulses suggest coarctation of the aorta, which has been associated with moyamoya disease. Examination of the skin may reveal lesions of varicella or herpes infection, abnormalities of Ehlers-Danlos syndrome or other connective tissue disorder, stigmata of neurocutaneous disorders, or lesions of conjunctivae and skin, suggesting Kawasaki or Behçet disease.

Neuroimaging. Neuroimaging is essential in the evaluation of a child with acute hemiplegia. Computed tomography (CT) is rapid and appropriate for emergent evaluation, but it is inadequate, except for perfusion-weighted imaging, to detect early stroke or accurately distinguish the many mimickers of ischemic stroke in children (82; 01; 83). Magnetic resonance imaging (MRI) with diffusion-weighted imaging sequences with apparent diffusion coefficient maps is the most sensitive test for arterial ischemic stroke (03). Diffusion- and perfusion-weighted imaging can detect ischemia within minutes. Gradient echo imaging detects blood. Specific patterns on MRI may aid in defining etiology, especially in mitochondrial disorders. Stroke-like lesions that do not conform to vascular territories, which are bilateral, and the presence/absence of basal ganglia signal abnormalities may be clues to an underlying mitochondrial disorder. Subsequent MR spectroscopy is useful to aid in identifying certain mitochondrial disorders by evaluating for lactate peaks in noninfarcted areas (54). Typical findings of PRES are bilateral areas of white matter edema in the posterior cerebral hemispheres but variations do occur.

MRI can also provide some information about the presence of vasculopathies. Axial T1 MRI of the neck with fat saturation should be performed along with magnetic resonance angiography to evaluate for arterial dissection or other vasculopathy. Moyamoya disease can be diagnosed on MRI based on its dilated, tortuous enhancing signal voids in the basal ganglia and the severe stenosis of the distal internal carotid artery (06).

In hemorrhagic stroke, CT may not identify underlying vascular abnormalities such as arteriovenous malformations and cavernomas, and it may poorly differentiate tumor from secondary hemorrhage. Therefore, MRI of the brain with gadolinium should be obtained. Multiple areas of hemorrhagic infarction suggest emboli. Tumor, demyelination, leukoencephalopathy, and evidence of trauma are best detected using MRI. Diffusion-weighted MRI can be similar in leukoencephalopathy and arterial ischemic stroke. However, in leukoencephalopathy the changes may not be confined to specific vascular territories (07); abnormalities can be present in subcortical or deep periventricular white matter, corpus callosum, cortex, cerebellum, and thalamus (78; 34). Diffusion-weighted imaging changes may represent reversible cerebral dysfunction with associated cytotoxic edema and metabolic derangement rather than ischemic structural injury (78). Transient abnormalities have been described on MRI with both migraine and seizures. Longitudinal follow-up of a case of prolonged hemiplegic attack using MRI, DWI, MR spectroscopy, and single-photon emission computed tomography (SPECT) provided supportive evidence for primary neuronal dysfunction (92). The MRI revealed progressive signal alterations in the hemisphere affected. The magnetic resonance spectroscopy demonstrated neuronal loss (decreased N-acetylaspartate) without cerebral ischemia, and single photon emission computed tomography (SPECT) exhibited reversible impairment of neuronal function.

MRA is probably equivalent to standard angiography for carotid and middle cerebral artery angiopathies, but it is less sensitive for small vessel disease and distal angiopathy (13). In some cases, cerebral angiography may be required to better delineate suspected arterial dissection, moyamoya disease, fibromuscular dysplasia, or vasculitis (77). MRA can actually overestimate the length and severity of stenosis. MRA can also identify aneurysms larger than 3 mm. A 3-D CT angiography may have value in the evaluation of arterial ischemic stroke and arteriopathies, but the amount of radiation is relatively high, and its utility in pediatric cases is not well studied. Abnormal cerebral angiography is the primary diagnostic feature of reversible vasoconstriction syndrome. Smooth, tapered narrowing followed by dilated segments of second and third order of cerebral arteries, resulting in a “sausage on a string” appearance, is the most characteristic abnormality (64).

Electroencephalography (EEG). EEG distinguishes between an ictal and postictal basis for hemiplegia. In moyamoya disease, hyperventilation can produce bursts of high-amplitude delta. Following cessation of hyperventilation, the delta activity can resolve and spontaneously recur, called "re-build up phenomenon" (52). Prolonged video EEG monitoring may be high yield in arterial ischemic stroke, cerebral venous sinus thrombosis, or hemiplegic stroke as the reported incidence of non-convulsive seizures, including status epilepticus, in those who presented with seizure at the time of stroke ranges from 14% to 23% (01; 83).

Complete blood count is required to evaluate for infection and hematologic disorders. Blood cultures are necessary for diagnosing bacterial endocarditis. Erythrocyte sedimentation rate, prothrombin time, and partial thromboplastin time may also suggest an etiology in some cases. Serum chemistries may help distinguish neurologic complications of hyperglycemia or hypoglycemia from stroke, whereas urine drug screen for cocaine and amphetamines may identify a toxicological cause of stroke. Routine urinalysis provides information about possible renal disease and dehydration. Pregnancy tests should be performed in adolescent females. When embolic disease is suspected, chest x-ray and echocardiography are essential to evaluate for possible cardiac disease. Cardiac rhythm monitoring can identify arrhythmias as a source of emboli. When an acute or chronic meningitis or encephalitis is suspected, lumbar puncture is indicated. Children without evidence to suggest common causes of hemiplegia should undergo more extensive evaluation, such as a search for underlying prothrombotic state (49). A genetic workup should be undertaken in cases with familial hemiplegic migraine and alternating hemiplegia of childhood. Metabolic stroke is usually the result of metabolic defects that cause energy failure and intoxication. Serum and cerebrospinal fluid lactate is usually elevated in mitochondrial disorders. Serum amino acids and urine organic acids are helpful at the time of clinical deterioration. Genetic testing for common mitochondrial mutations and measurement of respiratory chain activities in muscle are required to establish the diagnosis of mitochondrial encephalomyopathies (10).

Prevention

Risk factors for stroke in children differ significantly from those in adults. Widespread vaccination against Haemophilus influenzae and Streptococcus pneumoniae has decreased the incidence of bacterial meningitis and its complications, including stroke. Similarly, vaccination against varicella may have decreased the frequency of post-varicella focal cerebral arteriopathy. Children with metabolic and genetic disorders underlying stroke may benefit from treatment of their underlying condition, including possible gene therapy. Medication management to prevent migraine and seizure presumably decreases the frequency of hemiplegic migraine and Todd paralysis. Primary prevention for stroke in children has been accomplished in sickle cell disease and intracardiac thrombus. Blood transfusion therapy decreases recurrence risk in sickle cell disease and anticoagulation or surgery prevents stroke in children with intracardiac thrombus. Aside from these high-risk situations, stroke prevention has not been achieved in pediatric patients. It is important to establish healthy behaviors in childhood to reduce the risk of obesity and sedentary lifestyle (32).

Management

Management depends on the cause of hemiplegia and differs significantly after stroke, hemorrhage, epilepsy, migraine, and infection as well as demyelinating, metabolic disease and genetic disease.

Stroke. Several consensus guidelines for the management of different types of stroke have been published. Treatment of hypertension, hypotension, fever, hyperglycemia, and seizures is essential. Decompressive hemicraniectomy should be considered for large strokes associated with mass effect and deterioration of consciousness (85). Early heparinization is reasonable pending evaluation for the cause of the stroke because the likelihood of arterial dissection, undiagnosed cardiac disease, and coagulopathy is fairly high in children. Once these etiologies are ruled out, anticoagulation can be stopped and aspirin substituted for secondary stroke prevention. The duration of aspirin therapy depends on the underlying condition. Most children are treated for 2 years to cover the time window when the vast majority of recurrent strokes occur. If arterial ischemic stroke is due to cardiac embolism, extracranial arterial dissection, or hypercoagulable state, long-term anticoagulation is recommended. In sickle cell disease, acute exchange transfusion followed by long-term transfusion therapy is standard care. Revascularization surgery is recommended for moyamoya disease if symptoms of ischemia increase or if cerebral perfusion is compromised (77). With strokes due to cerebral venous sinus thrombosis, the precipitating illness and seizures and elevated intracranial pressure, if present, require treatment (77). Anticoagulation both acutely and chronically is recommended (3 to 6 months) (66; 77), particularly given the fact that thrombus propagation may occur in over one third of untreated children (65). Treatment of stroke due to genetic and metabolic conditions varies by disorder (77).

Arterial recanalization therapy, including intravenous tissue type plasminogen activator (tPA) and intraarterial tPA or endovascular thrombectomy for childhood arterial ischemic stroke, remains controversial. They can be used in children with persistent disabling neurologic deficits (pediatric NIH score ≥ 6 at the time of intervention), radiographically confirmed cerebral large artery occlusion, and larger children. Treatment decision should be made with neurologists and endovascular surgeons with experience in treating children (32).

Traditional rehabilitation is appropriate for hemiplegia of any cause. Both unimanual constraint therapy and bimanual therapy have been adapted for children and appear to improve function of the hemiparetic hand (42). Motor imagery training appears to enhance recovery of hemiplegia (86). The use of daily inhibitory, low frequency, repetitive transcranial magnetic stimulation to the unaffected M1 region may improve motor function in children with chronic subcortical arterial ischemic stroke by rebalancing cortical inhibition of the contralateral motor cortex (50).

Hemorrhage. In the acute setting, children with cerebral hemorrhage should be stabilized medically. Surgical or endovascular intervention may be indicated in cases of large hemorrhage and arteriovenous malformations or aneurysm (09).

Seizures. Appropriate EEG monitoring and choice of antiepileptic medication therapy is crucial to seizure control.

Alternating hemiplegia of childhood. Flunarizine, a calcium channel blocker, has been shown to reduce duration, severity, and frequency of episodes of alternating hemiplegia, but has no abortive effects (63). Theoretically, flunarizine works because alternating hemiplegia is a channelopathy involving CACNA1A and ATP1A2 (20). However, flunarizine has not been shown to have a significant effect on the overall developmental outcome. Glutamate and N-methyl-D-aspartate (NMDA) receptors are thought to also be involved in the induction of attacks. Amantadine, an NMDA receptor antagonist, and topiramate are drugs that modulate ion channels and inhibit non-NMDA excitatory neurotransmission, and have been investigated as add-on agents (20). Seizures are a rare side effect of amantadine (87).

Hemiplegic migraine. Flunarizine, valproate, and steroids have been used to treat prolonged auras acutely (96). Intranasal ketamine has been used to treat hemiplegic aura (48). Calcium channel blockers and valproate are the most commonly used as preventative agents in hemiplegic migraine (96; 74). Beta blockers and triptans should be avoided (51; 41).

Demyelinating disorders. Acute disseminated encephalomyelitis generally responds to treatment with corticosteroids or intravenous immunoglobulins (22). Plasma exchange is suggested in cases who fail to respond steroids and intravenous immunoglobulins.

Conversion disorder. Physical therapy is an important part of treatment for conversion paralysis along with appropriate pharmacotherapy and psychotherapy to address the related psychological stressors and any comorbid psychiatric disorder (eg, depression, anxiety) (70).

Reversible cerebral vasoconstriction syndrome. There is no established therapy for RCVS. Most patients fully recover with time. It is reasonable to admit patients for observation, pain control, and supportive care. Calcium channel blockers such as nimodipine and verapamil, serotonin antagonists, dantrolene, and brief courses of magnesium sulfate have been helpful in some studies (29).

Posterior reversible encephalopathy syndrome. PRES should be promptly recognized because it is usually reversible with appropriate treatment. Gradual blood pressure lowering will often improve the patients dramatically. Overaggressive blood pressure lowering can lead to complications. Seizures are treated with antiseizure drugs. Reduction drug dose or prompt removal of the cytotoxic or immunosuppressive drug is recommended (35).

Pregnancy

Pregnancy is by definition a proinflammatory and hypercoagulable state. Postnatal infection, migraine, thrombophilias, lupus, heart disease, preeclampsia, smoking, obesity, older age, bed rest more than 4 days, and surgical delivery are specific risk factors (33). Migraine frequency can increase or decrease during pregnancy; and migraine can also increase the risk of stroke during pregnancy (17). Seizure control can be an issue during pregnancy.

Anesthesia

Specific anesthetic management is required in children who are at high risk for stroke due to moyamoya (08) or sickle cell disease (61).

Outcomes

There are no clinical trials evaluating the best treatment for childhood arterial ischemic stroke (anticoagulant or antiplatelet medication). It is well known that absence of antithrombotic therapy increases the risk of recurrent stroke by 1.5- to 2-fold. The use of anticoagulation is relatively safe in children with arterial ischemic stroke, with a 4% risk of intracerebral hemorrhage. Safety and efficacy data for hyperacute stroke therapies in children are lacking. Hemicraniectomy is a potentially lifesaving option in children with large supratentorial stroke. Decompressive craniotomy results in decreased mortality and improved outcomes in patients with large cerebellar infarctions (32). Flunarizine is associated with more than 50% reduction in attack frequency in 85% of pediatric hemiplegic migraine (74). Flunarizine can also reduce severity, frequency, and duration of dystonic and hemiplegic attacks in acute hemiplegia in childhood. The effect of flunarizine on long-term prognosis of disease is unknown (76). There is no proven therapy for RCVS and PRES. The clinical outcome is benign in 90% of cases with supportive care. Most patients with demyelinating disorder of central nervous system make a full but slow recovery with steroids over 4 to 6 weeks.

References

01: Abend NS, Beslow LA, Smith SE, et al. Seizures as a presenting symptom of acute arterial ischemic stroke in childhood. J Pediatr 2011;159(3):479-83. PMID 21429519
02: Aicardi J, Amsili J, Chevrie JJ. Acute hemiplegia in infancy and childhood. Dev Med Child Neurol 1969;11(2):162-73. PMID 5787715
03: Albers GW, Thijs VN, Wechsler L, et al. Magnetic resonance imaging profiles predict clinical response to early reperfusion: the diffusion and perfusion imaging evaluation for understanding stroke evolution (DEFUSE) study. Ann Neurol 2006;60(5):508-17. PMID 17066483
04: American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders. 4th edition. Washington DC: American Psychiatric Association (APA), 2000.
05: Auvin S, Bellavoine V, Merdariu D, et al. Hemiconvulsion-hemiplegia-epilepsy syndrome: Current understandings. Eur J Paediatr Neurol 2012;16(5):413-21. PMID 22341151
06: Bacigaluppi S, Dehdashti AR, Agid R, Krings T, Tymianski M, Mikulis DJ. The contribution of imaging in diagnosis, preoperative assessment, and follow-up of moyamoya disease: a review. Neurosurg Focus 2009;26(4):E3. PMID 19335129
07: Baehring JM, Fulbright RK. Delayed leukoencephalopathy with stroke-like presentation in chemotherapy recipients. J Neurol Neurosurg Psychiatry 2008;79(5):535-9. PMID 17682013
08: Baykan N, Ozgen S, Ustalar ZS, Dagcinar A, Ozek MM. Moyamoya disease and anesthesia. Paediatr Anesth 2005;15(12):1111-5. PMID 16324034
09: Beslow LA, Jordan LC. Pediatric stroke: the importance of cerebral arteriopathy and vascular malformations. Childs Nerv Syst 2010;26(10):1263-73. PMID 20625743
10: Bhate S, Ganesan V. A practical approach to acute hemiparesis in children. Dev Med Child Neurol 2015;57(8):689-97. PMID 25832616
11: Bickerstaff ER. Aetiology of acute hemiplegia in childhood. Br Med J 1964;2(5401):82-7. PMID 14147794
12: Binder DK. A history of Todd and his paralysis. Neurosurgery 2004;54(2):480-6. PMID 14744294
13: Braun KP, Rafay MF, Uiterwaal CS, Pontigon AM, DeVeber G. Mode of onset predicts etiological diagnosis of arterial ischemic stroke in children. Stroke 2007;38(2):298-302. PMID 17194885
14: Brazis PW, Masdeu J, Biller J. Localization in Clinical Neurology. 5th edition. Philadelphia: Lippincott Williams & Wilkins, 2007.
15: Brosch JR, Golomb MR. American childhood football as a possible risk factor for cerebral infarction. J Child Neurol 2011;26(12):1493-8. PMID 21968983
16: Burgmer M, Konrad C, Jansen A. Abnormal brain activation during movement observation in patients with conversion paralysis. Neuroimage 2006;29(4):1336-43. PMID 16213162
17: Bushnell CD, Jamison M, James AH. Migraines during pregnancy linked to stroke and vascular diseases: US population based case-control study. BMJ 2009;338:b664. PMID 19278973
18: Chadehumbe MA, Khatri P, Khoury JC, et al. Seizures are common in the acute setting of childhood stroke: a population-based study. J Child Neurol 2009;24(1):9-12. PMID 18923086
19: Chang CJ, Chang WN, Huang LT, et al. Cerebral infarction in perinatal and childhood bacterial meningitis. QJM 2003;96(10):755-62. PMID 14500862
20: Chi L, Zhao X, Liu X, et al. Alternating hemiplegia of childhood in Chinese following long-term treatment with flunarizine or topiramate. Int J Neurosci 2012;122(9):506-10. PMID 22494152
21: Chinnabhandar V, Singh A, Mandal A, Parmar BJ. Acute hemiplegia in children: a prospective study of etiology, clinical presentation, and outcome from Western India. J Neursci Rural Pract 2018;9(4):504-9. PMID 30271041
22: Dale RC, Brilot F, Banwell B. Pediatric central nervous system inflammatory demyelination: acute disseminated encephalomyelitis, clinically isolated syndromes, neuromyelitis optica, and multiple sclerosis. Curr Opin Neurol 2009;22(3):233-40. PMID 19434783
23: Dale RC, Cross JH. Ictal hemiparesis. Dev Med Child Neurol 1999;41(5):344-7. PMID 10378762
24: de Lange FP, Toni I, Roelofs K. Altered connectivity between prefrontal and sensorimotor cortex in conversion paralysis. Neuropsychologia 2010;48(6):1782-8. PMID 20206641
25: deVeber G. Arterial ischemic strokes in infants and children: an overview of current approaches. Semin Thromb Hemost 2003;29(6):567-73. PMID 14719173
26: deVeber GA, Kirton A, Booth FA, et al. Epidemiology and outcomes of arterial ischemic stroke in children: The Canadian Pediatric Ischemic Stroke Registry. Pediatr Neurol 2017;69:58-70. PMID 28254555
27: Dlamini N, Billinghurst L, Kirkham FJ. Cerebral venous sinus (sinovenous) thrombosis in children. Neurosurg Clin N Am 2010;21(3):511-27. PMID 20561500
28: Domi T, deVeber G, Shroff M, Kouzmitcheva E, MacGregor DL, Kirton A. Corticospinal tract pre-wallerian degeneration: a novel outcome predictor for pediatric stroke on acute MRI. Stroke 2009;40(3):780-7. PMID 19131656
29: Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol 2012;11(10):906-17. PMID 22995694
30: Dusser A, Goutieres F, Aicardi J. Ischemic strokes in children. J Child Neurol 1986;1(2):131-6. PMID 3598117
31: Eyong K, Torty C, Asindi A, Ekanem E. Acute hemiplegia: aetiology and outcome in Nigerian children. Pan Afr Med J 2020;36:155. PMID 32874419
32: Ferriero DM, Fullerton HJ, Bernard TJ, et al. Management of stroke in neonates and children: a scientific statement from the American Heart Association/American Stroke Association. Stroke 2019;50(3):e51-e96. PMID 30686119
33: Feske SK. Stroke in pregnancy. Semin Neurol 2007;27(5):442-52. PMID 17940923
34: Fisher MJ, Khademian ZP, Simon EM, Zimmerman RA, Bilaniuk LT. Diffusion-weighted MR imaging of early methotrexate-related neurotoxicity in children. AJNR Am J Neuroradiol 2005;26(7):1686-9. PMID 16091514
35: Fischer M, Schumutzhard E. Posterior reversible encephalopathy syndrome.  J Neurol 2017;264(8):1432-59. PMID 28054130
36: Fountas KN, Kapsalaki EZ, Robinson JS. Cervical epidural hematoma in children: a rare clinical entity. Case report and review of the literature. Neurosurg Focus 2006;20(2):E6. PMID 16512657
37: Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol 2015;14(9):914-25. PMID 26184985
38: Gallmetzer P, Leutmezer F, Serles W, Assem-Hilger E, Spatt J, Baumgartner C. Postictal paresis in focal epilepsies--incidence, duration, and causes: a video-EEG monitoring study. Neurology 2004;62(12):2160-4. PMID 15210875
39: Ganesan V, Cox TC, Gunny R. Abnormalities of cervical arteries in children with arterial ischemic stroke. Neurology 2011;72(2):166-71. PMID 21220720
40: Gardner-Thorpe C, Pearn J. Erasmus Darwin (1731-1802): neurologist. Neurology 2006;66(12):1913-6. PMID 16801659
41: Gelfand AA, Fullerton HJ, Goadsby PJ. Child neurology: Migraine with aura in children. Neurology 2010;75(5):e16-9. PMID 20679627
42: Gordon AM, Chinnan A, Gill S, Petra E, Hung YC, Charles J. Both constraint-induced movement therapy and bimanual training lead to improved performance of upper extremity function in children with hemiplegia. Dev Med Child Neurol 2008;50(12):957-8. PMID 19160464
43: Hartman AL, Lunney KM, Serena JE. Pediatric stroke: do clinical factors predict delays in presentation. J Pediatr 2009;154(5):727-32. PMID 19111319
44: Hetts SW, Narvid J, Sanai N, et al. Intracranial aneurysms in childhood: 27-year single-institution experience. AJNR Am J Neuroradiol 2009;30(7):1315-24. PMID 19357386
45: Higier H. A paralytic equivalent of genuine epilepsy. Deutsche A Nervenheilk 1916;55:95-100.
46: Hilal SK, Solomon GE, Gold AP, Carter S. Primary cerebral arterial occlusive disease in children. I. Acute acquired hemiplegia. Radiology 1971;99(1):71-86. PMID 4396176
47: John S, Singhal AB, Calabrese L, et al. Long-term outcomes after reversible cerebral vasoconstriction syndrome. Cephalalgia 2016;36(4):387-94. PMID 26088331
48: Kaube H, Herzog J, Käufer T, Dichgans M, Diener HC. Aura in some patients with familial hemiplegic migraine can be stopped by intranasal ketamine. Neurology 2000;55(1):139-41. PMID 10891926
49: Kenet G, Sadetzki S, Murad H, et al. Factor V Leiden and antiphospholipid antibodies are significant risk factors for ischemic stroke in children. Stroke 2000;31(6):1283-8. PMID 10835445
50: Kirton A, Deveber G, Gunraj C, Chen R. Neurocardiogenic syncope complicating pediatric transcranial magnetic stimulation. Pediatr Neurol 2008;39(3):196-7. PMID 18725065
51: Klapper J, Mathew N, Nett R. Triptans in the treatment of basilar migraine and migraine with prolonged aura. Headache 2001;41(10):981-4. PMID 11903526
52: Kurlemann G, Ziegler R, Grüneberg M, Bomelburg T, Ullrich K, Palm DG. Disturbance of GABA metabolism in pyridoxine-dependent seizures. Neuropediatrics 1992;23(5):257-9. PMID 1454145
53: Lai R, Abrey LE, Rosenblum MK, DeAngelis LM. Treatment-induced leukoencephalopathy in primary CNS lymphoma: a clinical and autopsy study. Neurology 2004;62(3):451-6. PMID 14872029
54: Lebre A, Rio M, Faivre d’Arcier L, et al. A common pattern of brain MRI imaging in mitochondrial diseases with complex I deficiency. J Med Genet 2011;48(1):16-23. PMID 20972245
55: Liepert J, Hassa T, Tüscher O, Schmidt R. Abnormal motor excitability in patients with psychogenic paresis. A TMS study. J Neurol 2009;256(1):121-6. PMID 19172217
56: Liedholm LJ, Eeg-Olofsson O, Ekenberg BE, Nicolaysen RB, Torbergsen T. Acute postasthmatic amyotrophy (Hopkins’ syndrome). Muscle Nerve 1994;17(7):769-72. PMID 8008004
57: Lo W, Zamel K, Ponnappa K, et al. The cost of pediatric stroke care and rehabilitation. Stroke 2008;39(1):161-5. PMID 18032740
58: Lynch JK, Han CJ. Pediatric stroke: what do we know and what do we need to know. Semin Neurol 2005;25(4):410-23. PMID 16341997
59: Mackay MT, Wiznitzer M, Benedict SL, et al. Arterial ischemic stroke risk factors: the International Pediatric Stroke Study. Ann Neurol 2011;69(1):130-40. PMID 21280083
60: Mancini J, Girard N, Chabrol B, et al. Ischemic cerebrovascular disease in children: retrospective study of 35 patients. J Child Neurol 1997;12(3):193-9. PMID 9130094
61: Marchant WA, Walker I. Anaesthetic management of the child with sickle cell disease. Paediatr Anaesth 2003;13(6):473-89. PMID 12846703
62: Masoud M, Prange L, Wuchich J, Hunanyan A, Mikati MA. Diagnosis and treatment of alternating hemiplegia of childhood. Curr Treat Options Neurol 2017;19(2):8. PMID 28337648
63: Mikati M, Kramer U, Zupanc M, Shanahan RJ. Alternating hemiplegia of childhood: clinical manifestations and long-term outcome. Pediatr Neurol 2000;23(2):134-41. PMID 11020638
64: Miller TR, Shivashankar R, Mossa-Basha M, Gandhi D. Reversible cerebral vasoconstriction syndrome, part 2: diagnostic work-up, imaging evaluation, and differential diagnosis. AJNR Am J Neuroradiol 2015;36(9):1580-8. PMID 25614476
65: Moharir MD, Shroff M, Stephens D, et al. Anticoagulants in pediatric cerebral sinovenous thrombosis: a safety and outcome study. Ann Neurol 2010;67(5):590-9. PMID 20437556
66: Monagle P, Chan A, Massicotte P, Chalmers E, Michelson AD. Antithrombotic therapy in children: the Seventh ACCP conference on antithrombotic and thrombolytic therapy. Chest 2004;126(3 Suppl):645S-87S. PMID 15383489
67: Monreal FJ. Acute hemiparesis in juvenile insulin-dependent diabetes mellitus. Neurology 1983;33(6):812-13. PMID 6682532
68: Munot P, Crow YJ, Ganesan V. Paediatric stroke: genetic insights into disease mechanisms and treatment targets. Lancet Neurol 2011;10:264-74. PMID 21349441
69: Nakajima M, Sugano H, Iimura Y, et al. Sturge-Weber syndrome with spontaneous intracerebral hemorrhage in childhood. J Neurosurg Pediatr 2014;13(1):90-3. PMID 24160667
70: Ness D. Physical therapy management for conversion disorder: case series. J Neurol Phys Ther 2007;31(1):30-9. PMID 17419887
71: O'Donovan CA, Chan A, Maxwell S. Focal akinetic seizures as the only clinical manifestation of partial epilepsy. Seizure 2007;16(6):542-4. PMID 17507254
72: Oestreich LJ, Berg MJ, Bachmann DL, Burchfiel J, Erba G. Ictal contralateral paresis in complex partial seizures. Epilepsia 1995;36(7):671-5. PMID 7555983
73: Pearce JM. Acute childhood hemiplegia. J Neurol Neurosurg Psychiatry 2003;74(9):1244. PMID 12933927
74: Peer Mohamed B, Goadsby PJ, Prabhakar P. Safety and efficacy of flunarizine in childhood migraine: 11 years’ experience, with emphasis on its effect in hemiplegic migraine. Dev Med Child Neurol 2012;54(3):274-7. PMID 22268377
75: Peter P, George A. Posterior reversible encephalopathy syndrome and the pediatric population. J Pediatr Neurosci 2012;7(2):136-8. PMID 23248696
76: Pisciotta L, Gherzi M, Stagnaro M, et al. Alternating hemiplegia of childhood: pharmacological treatment of 30 Italian patients. Brain Dev 2017;39(6):521-28. PMID 28249736
77: Roach ES, Golomb MR, Adams R, et al. Management of stroke in infants and children: a scientific statement from a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young. Stroke 2008;39(9):2644-91. PMID 18635845
78: Rollins N, Winick N, Bash R, Booth T. Acute methotrexate neurotoxicity: findings on diffusion-weighted imaging and correlation with clinical outcome. AJNR Am J Neuroradiol 2004;25(10):1688-95. PMID 15569732
79: Russell MB, Ducros A. Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurol 2011;10(5):457-70. PMID 21458376
80: Saitoh M, Ishii A, Ihara Y, et al. Missense mutations in sodium channel SCN1A and SCN2A predispose children to encephalopathy with severe febrile seizures. Epilepsy Res 2015;117:1-6. PMID 26311622
81: Schweitzer AD, Parikh NS, Askin G, et al. Imaging characteristics associated with clinical outcomes in posterior reversible encephalopathy syndrome. Neuroradiology 2017;59(4):379-86. PMID 28289809
82: Shellhaas RA, Smith SE, O'Tool E, Licht DJ, Ichord RN. Mimics of childhood stroke: characteristics of a prospective cohort. Pediatrics 2006;118(2):704-9. PMID 16882826
83: Singh RK , Zecavati N, Singh J, et al. Seizures in acute childhood stroke. J Pediatr 2012;160(2):291-6. PMID 21911225
84: Solomon GE, Hilal SK, Gold AP, Carter S. Natural history of acute hemiplegia of childhood. Brain 1970;93(1):107-20. PMID 5418395
85: Smith SE, Millman G, Ichord R, Telfeian A, Thompson D, Kirkham F. Good outcome following decompressive craniectomy for malignant middle cerebral artery infarction in children. Stroke 2004;35(1):335.
86: Steenbergen B, Crajé C, Nilsen DM, Gordon AM. Motor imagery training in hemiplegic cerebral palsy: a potentially useful therapeutic tool for rehabilitation. Dev Med Child Neurol 2009;51(9):690-6. PMID 19709140
87: Tatli B, Aydinli N, Caliskan M, et al. Clinical features of nine patients with alternating hemiplegia of childhood. J Paediatr Child Health 2011;47(10):734-6. PMID 21449903
88: Tenney JR, Schapiro MB. Child neurology: alternating hemiplegia of childhood. Neurology 2010;74(14):e57-9. PMID 20368625
89: Testai FD, Gorelick PB. Inherited metabolic disorders and stroke part 1: Fabry disease and mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes. Arch Neurol 2010a;67(1):19-24. PMID 20065125
90: Thomsen LL, Ostergaard E, Olesen J, Russell MB. Evidence for a separate type of migraine with aura: sporadic hemiplegic migraine. Neurology 2003;60(4):595-601. PMID 12601098
91: Todd RB. Clinical lectures on paralysis, certain diseases of the brain, and other affections of the nervous system. 2nd edition. Philadelphia: Lindsay and Blakiston, 1865.
92: Toldo I, Cecchin D, Sartori S, et al. Multimodal neuroimaging in a child with sporadic hemiplegic migraine: a contribution to understanding pathogenesis. Cephalagia 2011;31(6):751-6. PMID 21172953
93: Topcuoglu MA, Chan ST, Silva GS, Smith EE, Kwong KK, Singhal AB. Cerebral vasomotor reactivity in reversible cerebral vasoconstriction syndrome. Cephalalgia 2017;37(6):541-7. PMID 27206962
94: Villani F, D'Amico D, Pincherle A, Tullo V, Chiapparini L, Bussone G. Prolonged focal negative motor seizures: a video-EEG study. Epilepsia 2006;47(11):1949-52. PMID 17116038
95: Voon V, Brezing C, Gallea C, et al. Emotional stimuli and motor conversion disorder. Brain 2010;133(Pt 5):1526-36. PMID 20371508
96: Wheeler SD. Phenotype-driven preventive strategies for migraine and other headaches. Neurologist. 2009; 15(2):59-70. PMID 19276783
97: Zimmer JA, Garg BP, Williams LS, Golomb MR. Age-related variation in presenting signs of childhood arterial ischemic stroke. Pediatr Neurol 2007;37(3):171-5. PMID 17765804

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Uluc Yis MD

Dr. Yis of Dokuz Eylül University has no relevant financial relationships to disclose.
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Haluk Topaloglu MD

Dr. Topaloglu of Hacettepe Children's Hospital in Ankara, Turkey, has no relevant financial relationships to disclose.
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Nina F. Schor MD PhD

Dr. Schor of the National Institutes of Health has no relevant financial relationships to disclose.
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David A Griesemer MD (original author), Timothy D Carter MD, Sabrina E Smith MD PhD, Lawrence Brown MD, Belinda Oyinkan Marquis MD, Heather A Lau MD, Ruth Nass MD, Kimon Bekelis MD, and Robert J Singer MD

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Acute hemiplegia in childhood

Differential Diagnosis

External dissection
Focal cerebral arteriopathy dissection type
Focal cerebral arteriopathy inflammatory type
Moyamoya disease
Fibromuscular dysplasia
- ACTA2-related vascular disease
- Thrombophilia
- Sickle cell disease
- Primary angiitis of central nervous system
- Systemic lupus erythematosus
- Polyarteritis nodosa
- Takayasu arteritis
- Adenosine deaminase deficiency
- Wegener disease
- P-ANCA-related vasculitis
- C-ANCA-related vasculitis
- PHACE syndrome
- Fabry disease
- MELAS
- POLG1-related mitochondrial disease
- Ehler-Danlos type IV
- Oleys-Diets syndrome
- Patent foramen ovale
- Arrhythmia
- Congenital cardiac anomaly
- Acquired cardiac anomaly
- Vascular malformations
- Brain tumors
- Sturge-Weber syndrome
- Mitochondrial disorders
- Organic acidemias
- Migraine
- Seizures
- Demyelinating disorders
- Toxication
- Conversion
- Head trauma
- Neck trauma
- Hypertension
- Immunosuppressive drugs
- Antineoplastic drugs

Also Relevant

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Hemiplegic migraine
- Illicit drug use: neurologic complications
- Moyamoya disease
- Porencephaly
- Stroke associated with sickle cell disease
- Stroke in young adults
- Sturge-Weber syndrome

Acute hemiplegia in childhood …

Acute hemiplegia in childhood

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Prognosis and complications

Clinical vignette

Biological basis

Anatomic localization

Pathophysiology

Table 1. Common Causes of Pediatric Stroke

Epidemiology

Differential diagnosis

Table 2. Differential Diagnosis of Acute Hemiplegia in Children

Diagnostic workup

Prevention

Management

Pregnancy

Anesthesia

Outcomes

References

Contributors

Authors

Editor

Former Authors

Patient Profile

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Also in This Category

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Acute hemiplegia in childhood

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Prognosis and complications

Clinical vignette

Biological basis

Anatomic localization

Pathophysiology

Table 1. Common Causes of Pediatric Stroke

Epidemiology

Differential diagnosis

Table 2. Differential Diagnosis of Acute Hemiplegia in Children

Diagnostic workup

Prevention

Management

Pregnancy

Anesthesia

Outcomes

References

Contributors

Authors

Editor

Former Authors

Patient Profile

This is an article preview. Start a Free Account to access the full version.

Questions or Comment?

Also in This Category

NF2-related schwannomatosis

Breath-holding spells

Ischemic stroke

Cerebral embolism

Hormonal contraception and stroke

Acute cerebellar ataxia in children

Antiphospholipid antibody syndrome

Headache in children: overview and treatment approaches

This is an article preview.
Start a Free Account
to access the full version.