Childhood occipital visual epilepsy …

Jules C Beal MD

Epilepsy & Seizures

Updated 12.13.2023
Released 10.18.1993
Expires For CME 12.13.2026

Childhood occipital visual epilepsy

Author: Jules C Beal MD

See Contributor Disclosures

Editor: Solomon L Moshé MD

Childhood occipital visual epilepsy

In This Article

Quick Link

Introduction

Overview

Childhood occipital visual epilepsy is an epilepsy syndrome that manifests with frequent and brief visual seizures consisting of elementary visual hallucinations, blindness, or both. Postictal headache is common. Interictal EEG shows occipital spikes that may be activated by the elimination of fixation and central vision (fixation-off sensitivity); EEG may be normal. The syndrome is sometimes misdiagnosed as migraine with visual aura. Conversely, structural (symptomatic) epilepsies may imitate this syndrome. Prognosis is usually good, with remission of seizures and normalization of the EEG by the late teenage years. In this update, the author details developments in childhood occipital visual epilepsy and provides clues for its proper differential diagnosis in relation to migraine and other occipital epilepsies.

Key points

	• Onset is usually in late childhood or in the early teenage years.
	• It manifests mainly with typical visual seizures of elementary visual hallucinations or blindness that are usually of brief duration.
	• Seizures may be very frequent.
	• Postictal headache is common, and it is often indistinguishable from migraine headache.
	• EEG often shows occipital spikes or occipital paroxysms that may demonstrate fixation-off sensitivity. However, some patients have consistently normal interictal EEG.
	• Treatment with appropriate antiseizure medication is often needed.

Historical note and terminology

Gibbs and Gibbs documented that on EEG, seizure foci in one or both occipital lobes are most commonly found in young children. Occipital foci tend to disappear in adult life, and the subsidence of the electroencephalographic abnormality is usually accompanied by a cessation of seizures” (28). Peak age at first discovery of occipital foci was 4 to 5 years, and they could occur in children without seizures. Gibbs and Gibbs also described an 8.5-year-old boy with “onset of attacks 2 months before the recording, consisting of blindness, followed by holding both hands to eyes, rolling up of eyes, and loss of consciousness. . . severe headache after attack. . . no convulsions at any time” (28). EEG showed independent bilateral occipital spikes.

Subsequently, Gastaut retrospectively identified 36 patients with occipital paroxysms and proposed them as “a new type of epilepsy: benign partial epilepsy of childhood with occipital spike-waves” (26; 27). The 1989 International League Against Epilepsy (ILAE) classification proposed this syndrome be called “childhood epilepsy with occipital paroxysms” (15). This was renamed as “late-onset childhood occipital epilepsy (Gastaut type)” in subsequent ILAE proposals (18; 06). The ILAE 2017 operational classification of seizure types included this syndrome under “self-limited focal epilepsies,” meaning that it is an epilepsy syndrome that is likely to resolve over time (23). In the 2022 position paper from the ILAE Task Force on Nosology and Definitions, this syndrome remains classified as a self-limited focal epilepsy and is renamed “childhood occipital visual epilepsy” (46).

Clinical manifestations

Presentation and course

	• The typical visual seizures consist of simple visual hallucinations or ictal blindness.
	• Motor seizures and hemiconvulsions can occur.
	• Postictal headache is common and, in many cases, difficult to distinguish from migraine.
	• Prognosis is variable. Seizure remission will occur in many patients but is not universal. Patients will often have associated deficits in attention, memory, and visuospatial skills.

Visual seizures are the cardinal symptoms of childhood occipital visual epilepsy. They are usually frequent and diurnal, and they usually last from seconds to 1 to 3 minutes. Ictal blindness may last longer than this. The seizures may progress and coexist with other occipital symptoms such as sensory illusions of ocular movements and ocular pain, tonic deviation of the eyes, eyelid fluttering, or repetitive eye closures. Complex visual hallucinations, visual illusions, and other symptoms from more anterior ictal spreading rarely occur. Consciousness is intact during the visual symptoms but may be disturbed or lost if the seizures progress. Postictal headache often occurs.

Visual seizures

Simple visual hallucinations. Visual seizures are the most typical and usually the first ictal symptom (26; 27; 10; 09; 50). In a study of 129 patients with childhood occipital visual epilepsy, visual hallucinations were the first symptom in 62% and the only manifestation in 39% of patients (50).

Elementary visual hallucinations are typically brief. They develop quickly, lasting for 5 to 15 seconds, and seldom exceeding 1 to 2 minutes if they occur alone without other occipital or extra-occipital spreading. Rarely, they may last 15 to 20 minutes or longer. They are often multicolored and circular, appearing either in the periphery of a hemifield or centrally; they may multiply in number or size, or both; they may move horizontally to the other side; and they may be flashing or static.

They may be the only ictal manifestations, or progress to other seizure symptoms. They typically have a stereotyped appearance and progression. Patients may know at what stage during their visual hallucinations progression to secondary generalization is likely to occur (38; 10; 35; 39). Consciousness is not affected.

Blindness or partial visual loss. Blindness is probably as common an ictal symptom as elementary visual hallucinations. It can occur alone and can be the only ictal event in patients who may, in other times, have visual hallucinations without blindness (38; 39; 26; 27). Blindness is sudden and total, often lasting several minutes, with preserved consciousness (09; 04). Postictal blindness, hemianopia, and other partial visual loss are well established after visual seizures, with or without secondarily generalization.

Complex visual hallucinations and visual illusions. Complex visual hallucinations are much rarer, probably occurring in less than 10% of the patients and usually emerging from elementary visual hallucinations (27). These patients usually see the form of a face or figures, which may have the same location and movement sequence as that of the elementary visual hallucinations. They do not have the same emotional or other characteristics as those associated with temporal lobe semiology.

Ictal visual illusions such as micropsia, metamorphopsia, and palinopsia are most likely generated from the nondominant parietal regions (27). They are extremely rare and may be associated more with symptomatic occipital seizures (38).

Sensory hallucinations of ocular movements and pain. A sensation of ocular movement in the absence of detectable motion is a rare occurrence and mainly occurs as a progression of elementary visual hallucinations (38; 39).

Nonvisual ictal symptoms

Deviation of the eyes and oculoclonic seizures. Deviation of the eyes, usually following elementary visual hallucinations and often associated with ipsilateral turning of the head, is the most common (around 70%) nonvisual symptom (26; 27; 38; 39; 20; 10). This focal motor seizure usually starts after visual hallucinations but may also occur while the hallucinations still persist. It may progress to hemiconvulsions and generalized tonic clonic seizures (GTCS) (38; 09; 39).

Ictal clinical symptoms from occipital seizure propagation. Elementary visual hallucinations or other ictal symptoms may progress to hemiconvulsions or generalized convulsions. According to Gastaut and Zifkin, visual seizures are often followed by other nonvisual seizure symptoms such as hemiconvulsions (43%), complex focal seizures (14%), dysphasia, dysesthesia, aversive convulsions (25%), and generalized tonic-clonic seizures (13%) (27). Focal clonic seizures have been reported (36). Forced eyelid closure and eyelid blinking occur in approximately 10% of patients, usually at a stage in which consciousness is impaired. They signal impending secondarily generalized convulsions (26; 27; 38; 39).

Headache. Postictal headache is a consistent symptom in about half of patients (27; 09). This occurs immediately, or 5 to 10 minutes after the end of the visual hallucinations. The duration and severity of the headache appears to be proportional to the duration and severity of the preceding seizures. The headache may be diffuse and of mild to moderate intensity, but in some patients, it is strong and pulsating and may be associated with nausea, vomiting, photophobia, and phonophobia, which may make it indistinguishable from migraine (27; 04). Ictal headache, mainly orbital, is a rare occurrence.

Circadian distribution. Visual seizures are predominantly diurnal and occur at any time of the day. Longer seizures, with or without secondarily hemiconvulsions or generalized convulsions tend to occur either during sleep, causing the patient to wake, or after awakening.

Frequency of seizures. In untreated patients, brief visual seizures are frequent, occurring at a frequency of several per day or weekly. However, propagation to other seizure manifestations, such as focal or more generalized convulsions is much less frequent, occurring monthly, yearly, or exceptionally (10; 39).

Prognosis and complications

Prognosis is unclear, though available data may indicate that remission occurs in more than 60% of patients (28; 27; 10; 50). Patients whose seizures continue into adulthood may be unlikely to have long-term remission at all (03).

This syndrome is often misdiagnosed as migraine, resulting in the delay of appropriate antiseizure medication, sometimes for years, which may affect prognosis (38; 20; 39). In a study of 19 patients with childhood occipital visual epilepsy, only four patients had remission of seizures before the age of 17 years; seven patients also developed other types of seizures (47). Prognosis was not associated with a specific EEG pattern whether abnormal or normal.

There have been reported cases of childhood occipital visual epilepsy that developed continuous spike-waves during slow-wave sleep with cognitive deterioration, which may be reversible with appropriate medication (37; 21).

Various studies have demonstrated intellectual and neuropsychological abnormalities in children with childhood occipital epilepsy. Gulgonen and colleagues tested intellectual functioning, attention, memory, academic achievement, visual-motor functioning, and executive functioning in 21 patients with childhood occipital epilepsy compared to controls (30). The case patients' performance scores were lower in attention and memory, as well as in intellectual functioning.

Language impairments, and in particular difficulties with semantic function, have also been demonstrated in children with childhood occipital visual epilepsy (45). In neuropsychological assessment with specific visuoperceptual testing of six patients, a deficit in facial discrimination was found in four patients and was associated with a line orientation deficit in three (13). Significant abnormalities have also been found in the domains of visuomotor coordination, memory, and attention (42).

Children with childhood occipital visual epilepsy have been demonstrated to have impairments in visuospatial processing and those who experienced a higher number of seizures or in whom interictal EEG discharges had been present for a longer time demonstrated a higher degree of impairment (07).

Comparative neuropsychological testing between patients with childhood occipital visual epilepsy and Panayiotopoulos syndrome showed impairments in psychomotor speed, writing and arithmetic skills, and verbal memory in both groups whereas children with Panayiotopoulos syndrome showed worse performance in terms of visual memory and reading problems (02).

Clinical vignette

An otherwise normal 8-year-old boy had onset of weekly episodes of elementary visual hallucinations. These were brief, lasting 5 seconds to 15 seconds, and consisted of three to four concentric spherical rings of red and yellow moving from the left to the right visual field, and repeating the same course again after their disappearance on the right. On other occasions, only one colored ball was moving continuously from left to right. The colors were faint at onset, becoming more intense as the seizure progressed. There was no impairment of consciousness, convulsion, or headache. An initial diagnosis of acephalgic migraine was made.

Beginning at 10 years of age, on four occasions, the same concentric rings were bigger (double in size), followed the same course from left to right, but the duration of the seizure was longer, lasting 1 minute. On three more occasions, visual seizures were longer, lasting 2 to 3 minutes, followed by left sided deviation of the head, and left hemiconvulsions. At this stage, the diagnosis of occipital lobe seizures was suspected. An initial routine EEG was normal in the awake state.

Carbamazepine 600 mg daily was initiated at 11 years of age. The head deviation and hemiconvulsions ceased but he continued to have infrequent, brief, elementary visual hallucinations like those mentioned above, which were more likely to occur after watching television or playing video games for long periods. These were occasionally followed by diffuse headache of moderate intensity for 10 to 15 minutes, and once he felt sick. A follow-up EEG was again normal in the awake state but showed occasional occipital spikes during sleep. A high-resolution MRI was normal.

At 17 years of age, he had three visual seizures lasting for nearly an hour each (focal visual status epilepticus). They started with left sided blurring of vision, “whitish like a fog,” together with “small clouds of colors; mainly red,” and mixed with small colored circles. Symptoms did not progress, but when the blurring of vision and the visual hallucinations cleared, he had left sided throbbing headache, which started 5 to 7 minutes after the cessation of the seizures and lasted for half an hour. His carbamazepine dose was increased, and he has had no further seizures of any type.

Biological basis

Etiology and pathogenesis

	• The exact etiology is not clear, but a genetic predisposition is suspected.
	• There may be a relationship with other self-limited focal childhood epilepsies.

The etiology of childhood occipital visual epilepsy is unclear but genetic mechanisms are postulated. There is an increased family history of epilepsies (21% to 37%) or migraine (9% to 16%) (27; 09), but familial childhood occipital visual epilepsy appears to be rare (11). Genetic analysis of a series of patients with self-limited focal epilepsies of childhood found one patient with an atypical occipital epilepsy and a mutation involving EPHB6; no specific gene abnormalities were associated with classic childhood occipital visual epilepsy (44). A mother and son have been reported with symptoms suggesting an atypical childhood occipital visual epilepsy, both with a heterozygous deletion encompassing GABR1 and GABRG2 (24).

Childhood occipital visual epilepsy and other syndromes of self-limited childhood focal seizures may be linked together based on a common, genetically determined, functional derangement of the brain cortical maturational process, which Panayiotopoulos proposed to call “benign childhood seizure susceptibility syndrome” (39). EEG and seizure manifestations often follow an age-related localization and prevalence, and the location of spikes may shift over time (17). That occipital paroxysms may be bilateral and synchronous can be explained by cortical occipital hyperexcitability, which is driven simultaneously by fixation-off sensitivity (39). Conversely, Gastaut and Zifkin hypothesized that the occipital paroxysms are due to a subcortical or thalamocortical mechanism “in order to explain the rhythmic spike and wave complexes seen with eye closure or intermittent photic stimulation over an intact occipital lobe" (27).

An association between childhood occipital visual epilepsy and typical absence seizures has been reported. Typical absences may appear at the same time as or after the onset of visual seizures (49).

The mechanisms of postictal headache, common even after minor visual seizures with or without a predisposition to migraine, are unknown. It is likely that the occipital seizure discharge triggers a genuine migraine headache through trigeminovascular or brain stem mechanisms (38; 39).

Differential diagnosis

Confusing conditions

The differential diagnosis of childhood occipital visual epilepsy is mainly from other occipital epilepsies and migraine syndromes. Occipital epilepsies include structural occipital epilepsy, self-limited epilepsy with autonomic seizures (Panayiotopoulos syndrome), and photosensitive occipital lobe epilepsy. Migraine syndromes that may mimic occipital visual epilepsy include migraine with aura, basilar, or acephalgic migraine (38; 39; 01).

Occipital epilepsies. Symptomatic occipital epilepsy often imitates childhood occipital visual epilepsy; neuro-ophthalmological examination and initial brain imaging may be normal, requiring high-resolution MRI for detection of subtle lesions. Occipital seizures of hyperglycemia, mitochondrial disorders (12), Lafora disease, and celiac disease should be considered (39). In a study from Turkey, 15.7% (six out of 38) children with childhood epilepsy with occipital paroxysms had biopsy-proven celiac disease (31).

Self-limited epilepsy with autonomic seizures often has similar interictal EEG recordings to childhood occipital visual epilepsy. However, onset and clinical progression of the seizures distinguishes the two. In self-limited epilepsy with autonomic seizures, the seizures start with autonomic manifestations (emesis in 70%), and the whole seizure may be entirely autonomic, but commonly other more conventional symptoms occur in the progress of the seizures. These primarily include deviation of the eyes and impairment of consciousness; visual hallucinations and illusions may occur but not at onset. In childhood occipital visual epilepsy, seizures are exclusively occipital, and onset predominantly consists of elementary visual hallucinations. Deviation of the eyes is common; ictal vomiting may occur during the progression of seizures (20; 39).

Photosensitive occipital lobe epilepsy has been recognized as a syndrome of reflex epilepsy with age-related onset (18; 08). Photosensitive occipital seizures may start in adulthood (34), develop later in children with Rolandic seizures (38), or occur incidentally during intermittent photic stimulation of normal or migraine subjects (39). Occipital seizures may be induced by television, video games, and intermittent photic stimulation and can manifest with multicolored circular visual hallucinations that are often associated with blindness (29; 38; 20; 39; 41). Tonic deviation of the eyes, epigastric discomfort and vomiting, headache, and generalized convulsions may follow. Duration varies from 2 to 5 minutes or up to 2 hours. Prognosis is uncertain. Some children may have only one or two seizures, but others may not remit. Interictal EEG shows spontaneous and photically induced occipital spikes. Centrotemporal spikes may coexist. Ictal EEGs documented the occipital origin and the spreading of the discharges to the temporal regions (29; 41). Visual evoked potentials in this condition show higher amplitudes compared to both normal controls and patients with occipital visual epilepsy (08).

Migraine. Visual seizures are often confused with migraine with aura and basilar and acephalgic migraine, but their epileptic nature becomes apparent with careful evaluation (38; 39; 40; 14).

Paroxysmal tonic upgaze deviation, an uncommon neuro-ophthalmological syndrome characterized by episodes of sustained upward eye deviation, may be associated with occipital spikes on EEG (53). Of note, the spikes in this case occurred in the intervals between episodes. Not all cases of paroxysmal tonic upgaze deviation are associated with epileptiform discharges.

Associated or underlying disorders

Childhood occipital visual epilepsy is classified among the self-limited focal epilepsies of childhood, along with self-limited epilepsy with centrotemporal spikes and self-limited epilepsy with autonomic seizures. Occasionally a patient may exhibit aspects of more than one of these syndromes. Fortini and colleagues reviewed 7,705 individuals with self-limited focal epilepsy of childhood (25). Thirty-one patients exhibited findings consistent with both childhood occipital visual epilepsy and self-limited epilepsy with autonomic seizures; 22 patients had findings consistent with both childhood occipital visual epilepsy and self-limited epilepsy with centrotemporal spikes.

Diagnostic workup

	• The diagnosis is made by a combination of clinical history and EEG findings.
	• Interictal occipital spikes are typical; these often demonstrate fixation-off sensitivity.
	• A number of ictal EEG patterns may be present.
	• Brain imaging should be performed to rule out structural causes of seizures.

Neurologic exam is normal. Brain imaging is normal. All patients should have high resolution MRI to exclude static or progressive occipital lesions (01).

The interictal EEG can show occipital spikes or occipital paroxysms, often superimposed on an otherwise normal background (27; 35; 04). The spikes can be unilateral or bilateral and are often high in amplitude. Spike frequency is variable. Some patients may have a normal interictal EEG or may have spikes only during sleep; thus, prolonged EEG monitoring may be indicated when a routine study is unrevealing (09; 17). EEG abnormalities may be disproportional to clinical severity. The spikes may demonstrate fixation-off sensitivity, meaning that spikes can be induced by darkness, closing the eyes, or otherwise eliminating visual fixation. Conversely, spikes can sometimes be suppressed via visual fixation or eye opening. Occipital paroxysms may also be suppressed during various neuropsychological conditions (43).

In addition to the classic occipital spikes, a number of other abnormalities may be present on EEG. Generalized spikes, spike-wave or polyspike-wave discharges, centrotemporal spikes, and other focal spikes have all been reported (27; 47). Of 19 patients with this syndrome in one study, only four had the classical occipital paroxysms. Prognosis was not associated with a specific interictal EEG pattern whether abnormal or normal (47).

It should be emphasized that the characteristic occipital spikes are not by themselves diagnostic of this condition. Occipital spikes can also be seen in the setting of structural lesions, other epilepsy syndromes, patients with visual impairments, and a small number of normal children (09; 04). The presence of occipital spikes with associated frontal-central positivity or tangential dipoles may suggest one of the self-limited occipital epilepsies as opposed to these other etiologies of occipital spikes (16).

Although it is more common in occipital visual epilepsy, it should also be noted that fixation-off sensitivity is not specific and may occur in children and adult patients with or without occipital spikes with generalized and symptomatic focal epilepsies (33; 34; 39; 19; 32).

Simple focal occipital seizures with positive signs are characterized by fast rhythmic activities with intermixed spikes over the occipital region. Complex visual hallucinations, when they occur, can be accompanied by slower discharges. In oculoclonic seizures, slow spikes and spike-wave discharges are typical as the seizure progresses. A localized ictal fast spike rhythm may be observed before eye deviation.

Head deviation may be accompanied by rhythmic theta activity. The EEG during ictal blindness is characterized by pseudoperiodic slow waves and spikes. There are usually no postictal abnormalities or else they resolve quickly (05; 39; 22; 04).

References

01: Adcock JE, Panayiotopoulos CP. Occipital lobe seizures and epilepsies. J Clin Neurophysiol 2012;29:397-407. PMID 23027097
02: Akca Kalem S, Elmali AD, Demirbilek V, et al. Panayiotopoulos syndrome and Gastaut syndrome are distinct entities in terms of neuropsychological findings. Epilepsy Behav 2019;99:106447. PMID 31398558
03: Angus-Leppan H, Clay TA. Adult occipital lobe epilepsy: 12-years on. J Neurol 2021;268(10):3926-34. PMID 33900448
04: Beal JC, Eisermann M, Misra S, et al. Seizures and epilepsy in preterm and term neonates, infants, children and adolescents. In: Niedermeyer’s electroencephalography, 7th Edition. Schomer DL, Lopes da Silva FH, editors. New York: Oxford University Press, 2018:469-520.
05: Beaumanoir A. Semiology of occipital seizures in infants and children. In: Andermann F, Beaumanoir A, Mira L, Roger J, Tassinari CA, editors. Occipital seizures and epilepsies in children. London: John Libbey, 1993:71-86.
06: Berg AT, Berkovic SF, Brodie MJ, et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia 2010;51(4):676-85. PMID 20196795
07: Brancati C, Barba C, Metitieri T, et al. Impaired object identification in idiopathic childhood occipital epilepsy. Epilepsia 2012;53(4):686-94. PMID 22352401
08: Brinciotti M, Mittica A, Matricardi M. Characteristics of visual evoked potentials related to the electro-clinical expression of reflex seizures in photosensitive patients with idiopathic occipital lobe epilepsy. Epilepsy Res 2020;164:106345. PMID 32388124
09: Caraballo R, Koutroumanidis M, Panayiotopoulos CP, Fejerman N. Idiopathic childhood occipital epilepsy of Gastaut: a review and differentiation from migraine and other epilepsies. J Child Neurol 2009;24(12):1536-42. PMID 19955346
10: Caraballo RH, Cersosimo RO, Fejerman N. Childhood occipital epilepsy of Gastaut: a study of 33 patients. Epilepsia 2008;49(2):288-97. PMID 17888075
11: Carney PW, Harvey AS, Berkovic SF, Jackson GD, Scheffer IE. Siblings with refractory occipital epilepsy showing localized network activity on EEG-fMRI. Epilepsia 2013;54:e28-32. PMID 23294395
12: Cesaroni E, Scarpelli M, Zamponi N, Polonara G, Zeviani M. Mitochondrial encephalomyopathy lactic acidosis and strokelike episodes mimicking occipital idiopathic epilepsy. Pediatr Neurol 2009;41(2):131-4. PMID 19589463
13: Chilosi AM, Brovedani P, Moscatelli M, Bonanni P, Guerrini R. Neuropsychological findings in idiopathic occipital lobe epilepsies. Epilepsia 2006;47(Suppl 2):76-8. PMID 17105468
14: Cianchetti C, Pruna D, Ledda M. Epileptic seizures and headache/migraine: a review on types of association and terminology. Seizure 2013;22(9):679-85. PMID 23831147
15: Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 1989;30(4):389-99. PMID 2502382
16: Datta AN, Wallbank L, Micallef J, Wong PKH. Pediatric occipital spikes at a single center over 26 years and the significance of tangential dipole. J Child Neurol 2021;36(7):530-6. PMID 33406372
17: Emmady PD, Das JM. Benign occipital seizure. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing 2021. PMID 32491402
18: Engel J. Report of the ILAE classification core group. Epilepsia 2006;47(9):1558-68. PMID 16981873
19: Fattouch J, Casciato S, Lapenta L, et al. The spectrum of epileptic syndromes with fixation off sensitivity persisting in adult life. Epilepsia 2013;54 Suppl 7:59-65. PMID 24099056
20: Fejerman N, Caraballo RH. Benign Focal Epilepsies in Infancy, Childhood and Adolescence. London: John Libbey Eurotext, 2007.
21: Fejerman N, Caraballo RH, Flesler S. Atypical evolutions of idiopathic focal epilepsies in childhood. J Pediatr Epilepsy 2016a;5:122-32.
22: Ferrari-Marinho T, Macedo EF, Costa Neves RS, et al. Gastaut type idiopathic childhood occipital epilepsy. Epileptic Disord 2013;15:80-3. PMID 23531515
23: Fisher RS, Cross JH, French JA, et al. Operational classification of seizure types by the International League Against Epilepsy: position paper of the ILAE Commission for Classification and Terminology. Epilepsia 2017;58(4):522-30. PMID 28276060
24: Fonte J, Videira G, Chorão R, et al. Familial occipital lobe epilepsy associated with GABAA receptor variants. Seizure 2023;112:139-42. PMID 37852164
25: Fortini S, Espeche A, Galicchio S, et al. More than one self-limited epilepsy of childhood in the same patient: A multicenter study. Epilepsy Res 2021;177:106768. PMID 34547632
26: Gastaut H. A new type of epilepsy: benign partial epilepsy of childhood with occipital spike-waves. Clin Electroencephalogr 1982;13:13-22. PMID 6802526
27: Gastaut H, Zifkin BG. Benign epilepsy of childhood with occipital spike and wave complexes. In: Andermann F, Lugaresi E, editors. Migraine and epilepsy. Boston: Butterworths, 1987:47-81.
28: Gibbs FA, Gibbs EL. Atlas of electroencephalography. In: Epilepsy. Vol 2. Reading, MA: Addison-Wesley, 1952:214-90.
29: Guerrini R, Dravet C, Genton P, et al. Idiopathic photosensitive occipital lobe epilepsy. Epilepsia 1995;36:883-91. PMID 7649127
30: Gulgonen S, Demirbilek V, Korkmaz B, Dervent A, Townes BD. Neuropsychological functions in idiopathic occipital lobe epilepsy. Epilepsia 2000;41:405-11. PMID 10756405
31: Isikay S, Kocamaz H. Prevalence of celiac disease in children with idiopathic epilepsy in southeast Turkey. Pediatr Neurol 2014;50(5):479-81. PMID 24656466
32: Karkare KD, Menon RN, Radhakrishnan A, Cherian A, Thomas SV. Electroclinical characteristics and syndromic associations of "eye-condition" related visual sensitive epilepsies-A cross-sectional study. Seizure 2018;58:62-71. PMID 29665529
33: Koutroumanidis M, Tsatsou K, Sanders S, et al. Fixation-off sensitivity in epilepsies other than the idiopathic epilepsies of childhood with occipital paroxysms: a 12-year clinical-video EEG study. Epileptic Disord 2009;11(1):20-36. PMID 19258232
34: Koutroumanidis M, Tsirka V, Panayiotopoulos C. Adult-onset photosensitivity: clinical significance and epilepsy syndromes including idiopathic (possibly genetic) photosensitive occipital epilepsy. Epileptic Disord 2015;17(3):275-86. PMID 26293003
35: Michael M, Tsatsou K, Ferrie CD. Panayiotopoulos syndrome: an important childhood autonomic epilepsy to be differentiated from occipital epilepsy and acute non-epileptic disorders. Brain Dev 2010;32(1):4-9. PMID 19386455
36: Mironov MB, Chebanenko NV, Zykov VP, et al. Epileptic syndromes associated with focal clonic seizures. [Article in Russian] Zh Nevrol Psikhiatr Im S S Korsakova 2023;123(3):41-5. PMID 36946395
37: Pal DK, Ferrie C, Addis L, et al. Idiopathic focal epilepsies: the "lost tribe". Epileptic Disord 2016;18:252-88. PMID 27435520
38: Panayiotopoulos CP. Elementary visual hallucinations, blindness, and headache in idiopathic occipital epilepsy: differentiation from migraine. J Neurol Neurosurg Psychiatry 1999;66(4):536-40. PMID 10201433
39: Panayiotopoulos CP. A clinical guide to epileptic syndromes and their treatment. Revised 2nd edition. London: Springer, 2010.
40: Panayiotopoulos CP, Bureau M, Caraballo RH, Dalla Bernardina B, Valeta T. Idiopathic focal epilepsies in childhood. In: Bureau M, Genton P, Dravet C, et al, editors. Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th ed with video. Montrouge, France: John Libbey Eurotext, 2012:217-54.
41: Parmeggiani L, Guerrini R. Idiopathic photosensitive occipital lobe epilepsy. In: Panayiotopoulos CP, editor. Atlas of epilepsies. London: Springer, 2010:1077-80.
42: Polat M, Gokben S, Tosun A, Serdaroglu G, Tekgul H. Neurocognitive evaluation in children with occipital lobe epilepsy. Seizure 2012;21(4):241-4. PMID 22265577
43: Rots ML, De Vos CC, Smeets-Schouten JS, Portier R, van Putten MJ. Suppressors of interictal discharges in idiopathic childhood occipital epilepsy of Gastaut. Epilepsy Behav 2012;25(2):189-91. PMID 23032130
44: Rudolf G, de Bellescize J, de Saint Martin A, et al. Exome sequencing in 57 patients with self-limited focal epilepsies of childhood with typical or atypical presentations suggests novel candidate genes. Eur J Paediatr Neurol 2020;27:104-10. PMID 32600977
45: Savas M, Tunçer AM, Çokar AÖ, Demirbilek AV, Tüzün E. Impact of epilepsy on language and discourse: Two self-limited focal epileptic syndromes of childhood. Epilepsy Behav 2020;102:106671. PMID 31805505
46: Specchio N, Wirrell EC, Scheffer IE, et al. International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions. Epilepsia 2022;63(6):1398-1442. PMID 35503717
47: Tsiptsios D, Kokkinos V, Sanders S, et al. Electroencephalographic manifestations and their prognostic significance in idiopathic childhood occipital epilepsy of Gastaut. Epilepsia 2010;51(Suppl 4):156.
48: Valeta T, Panayiotopoulos CP. Treatment of classic syndromes in idiopathic focal epilepsies in childhood. J Pediatr Epilepsy 2016;5:142-6.
49: Verrotti A, Coppola G, D'Egidio C, Parisi P, Chiarelli F. Gastaut type-idiopathic childhood occipital epilepsy and childhood absence epilepsy: a clinically significant association. Seizure 2010;19(6):368-72. PMID 20471287
50: Verrotti A, Laino D, Rinaldi VE, et al. Clinical dissection of childhood occipital epilepsy of Gastaut and prognostic implication. Eur J Neurol 2016;23(2):241-6. PMID 26498733
51: Verrotti A, Parisi P, Loiacono G, et al. Levetiracetam monotherapy for childhood occipital epilepsy of Gastaut. Acta Neurol Scand 2009;120(5):342-6. PMID 19754870
52: Yan H, Wang X, Zhang X, et al. Deep brain stimulation for patients with refractory epilepsy: nuclei selection and surgical outcome. Front Neurol 2023;14:1169105. PMID 37251216
53: Zhang YF, Wang YZ, Hao XS, Zhang HB, Wang JT, Liang JM. Paroxysmal tonic upgaze accompanied by occipital discharge on electroencephalography: a case report and literature review. J Int Med Res 2021;49(2):300060520984929. PMID 33530807
54: Zhao F, Ren Y, Geng G, et al. Effectiveness and tolerability of perampanel monotherapy in children with newly diagnosed focal epilepsy. Front Neurol 2023;14:1144759. PMID 37292124

Contributors

All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.

Author

Jules C Beal MD

Dr. Beal of Weill Cornell Medicine and New York-Presbyterian Queens Hospital has no relevant financial relationships to disclose.
See Profile

Editor

Solomon L Moshé MD

Dr. Moshé of Albert Einstein College of Medicine has no relevant financial relationships to disclose.
See Profile

Former Authors

Jerome Engel Jr MD PhD (original author) and C P Panayiotopoulos MD PhD

Patient Profile

Age range of presentation: 2 to 18 years
Sex preponderance: male=female
Heredity: heredity may be a factor
Population groups selectively affected: none selectively affected
Occupation groups selectively affected: none selectively affected

ICD & OMIM codes

ICD-9: Localization-related (focal) (partial) epileptic syndromes with partial seizures: 345.5
ICD-10: Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with simple partial seizures: G40.1

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

Nearly 3,000 illustrations, including video clips of neurologic disorders.
Every article is reviewed by our esteemed Editorial Board for accuracy and currency.
Full spectrum of neurology in 1,200 comprehensive articles.
Listen to MedLink on the go with Audio versions of each article.

Questions or Comment?

MedLink®, LLC

3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660

Support: service@medlink.com

Editor: editor@medlink.com

ISSN: 2831-9125

Childhood occipital visual epilepsy

Differential Diagnosis

structural (cryptogenic) occipital epilepsy
structural occipital epilepsy
celiac disease
migraine with aura
basilar migraine
- acephalgic migraine
- occipital seizures of hyperglycemia
- mitochondrial disorders
- Lafora disease
- Panayiotopoulos syndrome
- photosensitive occipital lobe epilepsy

Also Relevant

Hemiclonic seizures
Photosensitive occipital lobe epilepsy
Migraine aura without headache
Panayiotopoulos syndrome
Reflex seizures
- Seizures of the posterior neocortex
- Visual-sensitive epilepsies

Clinical Categories

Epilepsy & Seizures

Childhood occipital visual epilepsy …

Childhood occipital visual epilepsy

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Visual seizures

Nonvisual ictal symptoms

Prognosis and complications

Clinical vignette

Biological basis

Etiology and pathogenesis

Epidemiology

Differential diagnosis

Confusing conditions

Associated or underlying disorders

Diagnostic workup

Management

Outcomes

References

Contributors

Author

Editor

Former Authors

Patient Profile

ICD & OMIM codes

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

Questions or Comment?

Also in This Category

Anti-LGI1 encephalitis

Alcohol withdrawal seizures

Epileptic lesions due to malformation of cortical development

Epileptic spasms

Jacksonian seizures

Neuroacanthocytosis

Transient epileptic amnesia

Self-limited epilepsy with autonomic seizures

Childhood occipital visual epilepsy

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Visual seizures

Nonvisual ictal symptoms

Prognosis and complications

Clinical vignette

Biological basis

Etiology and pathogenesis

Epidemiology

Differential diagnosis

Confusing conditions

Associated or underlying disorders

Diagnostic workup

Management

Outcomes

References

Contributors

Author

Editor

Former Authors

Patient Profile

ICD & OMIM codes

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

Questions or Comment?

Also in This Category

Anti-LGI1 encephalitis

Alcohol withdrawal seizures

Epileptic lesions due to malformation of cortical development

Epileptic spasms

Jacksonian seizures

Neuroacanthocytosis

Transient epileptic amnesia

Self-limited epilepsy with autonomic seizures

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