Hot water epilepsy …

Epilepsy & Seizures

Updated 08.28.2023
Released 01.03.2012
Expires For CME 08.28.2026

Hot water epilepsy

Authors: K P Vinayan MD DM, Solomon L Moshé MD

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Editor: Jerome Engel Jr MD PhD

Hot water epilepsy

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Introduction

Overview

Hot water epilepsy is one of the reflex epilepsies induced by hot water being poured over the head, face, neck, or trunk during bathing. Two types of hot water epilepsies exist: the classical one seen in older children and adults, which is usually triggered by head baths, and a variant seen in infants precipitated by immersion of the lower trunk in hot water. In this article, the authors review the clinical, pathophysiological, and epidemiologic data related to hot water epilepsies. Relevant management strategies are also discussed. Even though initially considered as a geographically restricted epilepsy syndrome, hot water epilepsy is increasingly being reported all around the world. Hot water epilepsy in infancy may have an association with febrile seizures and Dravet syndrome. Management strategies include avoidance of hot water baths, antiepileptic drugs, and psychobehavioral approaches.

Key points

	• Hot water epilepsy is one of the reflex epilepsies induced by hot water pouring over the head, face, neck, or trunk during bathing.
	• Two types of hot water epilepsies exist; a classical one seen in older children and adults usually triggered by head baths, and a variant seen in infants precipitated by immersion of the lower trunk in hot water.
	• The exact pathogenesis is unclear.
	• Avoidance of hot water bath is currently considered to be the best preventive strategy
	• The majority of patients have a good outcome.

Historical note and terminology

According to the most recent position paper from ILAE, a reflex seizure is defined as a seizure that is consistently or nearly consistently elicited by a specific stimulus, which may be sensory, sensory-motor, or cognitive. The stimulus may be "elementary" (eg, light, touch), "complex" (eg, tooth-brushing, eating), or cognitive (eg, reading, calculating, thinking). Such a stimulus will have a high likelihood of eliciting a seizure, in contrast to a stimulus that may facilitate epileptiform abnormality (such as photoparoxysmal responses on EEG) or evoke a seizure, but not consistently (40; 26).

Hot water epilepsy is one of the reflex epilepsies induced by hot water pouring over the head, face, neck, or trunk during bathing (42). This type of reflex epilepsy was first reported from New Zealand in 1945 (02). Even though cases have been subsequently reported from different countries around the world and across all races, there was a clustering of cases from South India (13; 14). Satishchandra and colleagues reported a large clinical series of 279 patients with hot water epilepsy seen over a 4 year period (32). A prospective clinical series of 70 patients over a period of 2 years was added from the same center in 2012 (17). Another study reported 68 children with hot water epilepsy from another center in the same city (06). There have been several clinical series of hot water epilepsy reported from Turkey (19; 42).

Apart from classical hot water epilepsy, another type of reflex epilepsy triggered by hot water baths in infants has also been reported in literature (23). This condition is also called bathing epilepsy or water immersion epilepsy, as the temperature of the water has not always been hot and the trigger includes immersion of only the lower part of the body in hot water, as in the Japanese style bath (20).

Clinical manifestations

Presentation and course

Hot water epilepsy is predominantly seen in children and young adults with a male preponderance of approximately 2 to 3:1 (05). Among the cohort of 70 patients reported in a prospective clinical series, 55 were men (17). The exact cause for the clustering of cases in men is poorly understood. The majority of the reported cases may be classified as epilepsies of unknown cause with a clinical course similar to presumed genetic epilepsies of adolescence and adult onset (also known as idiopathic generalized epilepsies). A significant proportion of these patients have spontaneous seizures apart from those triggered by the hot water bath. In a series, 25 out of 70 patients had spontaneous seizures. Spontaneous seizures were seen more in patients who had an earlier onset of hot water epilepsy. The onset of spontaneous seizures had a variable interval after the onset of reflex seizures (17). Hot water epilepsy syndrome seen in infancy has been shown to have a strong association with Dravet syndrome (08). In a study from Japan, 5 of 7 children with Dravet syndrome showed an exacerbation in the frequency of seizures after hot water immersion. However, there was no difference in the rate of elevation in rectal temperature in these patients compared to control group (04).

The clinical features of the hot water epilepsy reported from south India have been well characterized (29). Older children are more frequently affected, although it has also been reported in adults (31). Age of onset varies from 10 to 50 years (17). In a pediatric series, the age of onset of seizures was between 1 to 5 years in the majority (06). Temperature of the hot water used for bathing ranges between 40°C and 50°C. Usually water is collected in a bucket, and is poured over the body or head by using a bowl. The frequency of the seizures depends on the frequency of the head baths. A small proportion of people had seizures only on body bath without head bath. The episodes may be characterized as focal seizures with impairment of consciousness. Seizures usually start with intense fear, behavior arrest, irrelevant speech, and complex automatisms. Some of the patients showed a tendency for self-induction of seizures by continuously pouring water over the head until they lost consciousness. Occasionally, secondary generalization occurs, resulting in generalized tonic clonic seizures. Spontaneous nonreflex epilepsy has been reported to occur a few years later in 16% to 38% of patients. A history of epilepsy among the family members has been reported in 7% to 22.6% of cases (14; 31).

In hot water epilepsy of infancy, the temperature of the water is usually close to body temperature (around 37.5°C). The seizures are predominantly characterized by autonomic features. Affected infants have often been described as becoming quiet with staring, apnea, pallor, blue lips, and limpness (20).

Neurologic examination, interictal EEG, and imaging are usually normal. However, focal epilepsies with structural cortical malformations have been reported, presenting initially with reflex seizures triggered by hot water bath (10; 27). A case of macrocephaly-capillary malformation syndrome presenting with hot water epilepsy has also been reported (41). In view of the technical considerations, ictal EEG recordings are difficult in hot water epilepsies induced by head bath (34). In the infantile form of hot water epilepsy induced by immersion of the inferior part of the body, ictal EEG showed a focal onset in the temporal areas (23). Multimodal imaging studies with SPECT and EEG-fMRI were also reported in the literature (22; 28).

Prognosis and complications

The natural course for hot water epilepsy cases reported in older children and adults was usually favorable. A majority of patients outgrow their epilepsy. Five percent to 10% of patients develop seizures in hot water body baths alone at a later stage of their course (34). Spontaneous nonreflex seizures have been seen in around 16% to 38% of patients after few years of onset of hot water epilepsy. Bharathi and colleagues reported nonreflex seizures in around 35.3% of their pediatric cohort, which included both fever-triggered seizures (16.2 %) and unprovoked seizures (19.1%) (06).

The natural course for hot water epilepsy in infancy is also usually benign. All of the 5 patients reported by Plouin and Vigevano were seizure-free at 3 years follow-up (23). However, hot water epilepsy in infancy was found to be one of the strong clinical predictors for future development of Dravet syndrome (11). In cases of hot water epilepsy with associated history of severe early onset febrile seizures, a genetic testing for SCN1A mutation may be useful for prognostication.

Clinical vignette

A 2-year-old girl presented with reported recurrent events during bathing. She was born of nonconsanguineous parents with a normal birth history. Birth weight was 3.5 Kg. Neonatal period was uneventful. She had the first episode of generalized tonic clonic seizures after DPT vaccination on day 42. EEG and MRI brain were normal. Subsequently, she developed recurrent myoclonic jerks and generalized tonic clonic seizures. By the age of 6 months, she developed frequent seizures during febrile illnesses. She was on multiple antiepileptic drugs without much response. Her mother also noted frequent episodes during bathing of the body with hot water during which child became pale initially, then became unresponsive with tonic posturing of the limbs and up rolling of the eyes. Examination showed no dysmorphic features. She had significant global developmental delay and symmetric hypotonia. Video-EEG telemetry demonstrated 2 habitual clinical events on immersion of the body in hot water. The EEG showed a sudden burst of high amplitude diffuse asymmetrical delta slow waves that were maximum over both temporal regions. No events were noted spontaneously or on immersion in cold water. The possibility of Dravet syndrome with symptomatic hot water epilepsy in infancy was considered clinically. The family was advised to avoid hot water baths. SCN1A mutation study was not carried out because of the lack of availability.

Biological basis

Etiology and pathogenesis

The etiology of hot water epilepsy is not known. It is predominantly seen in children and adults with no identified etiologies. A high proportion of the infants with hot water epilepsy also had febrile seizures (37), suggesting a common pathophysiology. Hot water epilepsy in infancy is one of the strong clinical predictors for future development of Dravet syndrome (04; 08; 11).

The exact pathogenesis of hot water epilepsy is unknown. Although it was possible to provoke seizures in the laboratory by pouring hot water over the heads of patients, hot-water towels, sauna, or blowing hot air on the head failed to induce seizures, suggesting that the triggering stimulus is complex and would involve a combination of factors such as contact of scalp with hot water, temperature of water, and specific cortical area of stimulation (35). The warmth receptors are most active in the temperature range between 40°C and 50°C, which is most often associated with precipitation of seizures. Above this range of temperature, pain fibers are activated (noxious heat). None of the reported cases reported pain on pouring of hot water; rather, some of them described a pleasurable sensation before the development of frank seizures. This may suggest that a specific range of temperature is an important trigger (12). A defective inhibitory influence over afferent volleys of somatosensory stimuli, such as warm or hot water over large body surfaces, with an additive effect from the specific temperature range described, might be acting as the final trigger.

A hypothesis based on the hyperthermic kindling model has also been suggested (38). According to this model, an aberrant thermoregulatory system is postulated in patients with hot water epilepsy, along with an increased sensitivity of the brain to rapid surges in body temperature. Satishchandra and colleagues experimentally demonstrated that people with hot water epilepsy had a rapid spurt in body temperature compared to their normal peers (33). This higher body temperature also persisted for prolonged periods compared to the controls. Another study reported impaired autonomic system balance in drug naive patients with hot water epilepsy (16). A subsequent study from the same group showed a different pattern of autonomic dysfunction in patients who had both hot water epilepsy and spontaneous seizures compared to those with hot water epilepsy alone (18). In another clinical series from south India, a significant proportion of patients had features suggestive of nonepileptic psychogenic events during hot water baths, suggesting the possibility of a nonepileptic condition, pseudo hot water epilepsy (39).

A high proportion of the infants with hot water epilepsy also had febrile seizures (09). Eleven percent to 27% of the older children and adults with hot water epilepsy reported from India also had history suggestive of febrile seizures before the development of epilepsy (36; 14). Children with Dravet syndrome with severe febrile convulsions also showed an increased tendency for seizures stimulated by hot water baths. There was no difference in the rate of elevation in rectal temperature between the patients with Dravet syndrome and controls, suggesting that the brain is exquisitely sensitive to elevation in body temperature in children with this disorder (03).

The genetic mechanism underlying the expression of hot water epilepsy in humans is not known. Eighteen percent of Indian patients had at least 1 family member affected by hot water epilepsy. Two genetic loci have been described from India (25; 24). SCN1A mutations, pathognomonic of Dravet syndrome, may possibly contribute to the expression of hot water epilepsy in infancy. A distinct genetic epileptic syndrome called “X-linked focal epilepsy with reflex bathing seizures” has been reported in a French–Canadian family (21). Q555X mutation of synapsin 1 (SYN1) on chromosome Xp11-q21 was documented in this cohort. SYN1 mutations were identified in 12 individuals from 10 different Caucasian families with bathing epilepsy (01). However, none of the patients with pure hot water epilepsy in this cohort had this variation.

Management

Complete avoidance of the precipitating stimuli, the hot water baths, is considered to be the most ideal step in management. Other modifications, like using lukewarm water for the head bath and using hot towels, have also been advocated (14). Phenytoin and carbamazepine have been used in the past to control the seizures. Follow-up of 208 patients treated with conventional antiepileptic drugs for a mean period of 14 ± 12.9 months (range, 6 to 60 months) showed that 60% could be easily controlled, and 18.3% had a 50% reduction in frequency, but others continued to have seizures at their last follow-up (32). Drugs like phenobarbital, sodium valproate, oxcarbazepine, lamotrigine, and levetiracetam were also used in some cases (42). If the patients are taking head baths infrequently, oral administration of 5 to 10 mg of clobazam about 1.5 to 2 hours before head bath has been found to be effective in preventing the seizures (07). If the hot water epilepsy is associated with other nonreflex seizures, regular intake of antiepileptic dugs may be necessary (30).

Venkataramiah and Ghorpade reported a pilot trial of psychotherapeutic intervention that was found to be successful in preventing the recurrence of events in a cohort of patients with presumed hot water epilepsy. After the characterization of the symptoms and the temperature and volume of the hot water used in an assessment trial bath, the patients underwent a series of therapeutic trial baths, based on the principle of desensitization. To begin with, the hot water used was 1°C less than the temperature at which patients previously experienced the symptoms. During the therapeutic baths, the investigator continuously kept patients engaged in conversation, boosting their confidence to take the bath and also diverting attention away from the anticipatory anxiety. This procedure was repeated over a few days, increasing the temperature of water by 2°C each time until the highest tolerable level was achieved. Out of the 8 patients who were followed up for 1 year, 6 were completely symptom-free and were able to take hot water baths without any recurrence of symptoms (39).

Outcomes

References

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Contributors

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

Authors

K P Vinayan MD DM

Dr. Vinayan of the Amrita Institute of Medical Sciences has no relevant financial relationships to disclose.
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Solomon L Moshé MD

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

Jerome Engel Jr MD PhD

Dr. Engel of the David Geffen School of Medicine at the University of California, Los Angeles, has no relevant financial relationships to disclose.
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Patient Profile

Age range of presentation: 1 month to 44 years (mostly in children)
Sex preponderance: Male>female, >2:1
Heredity: Heredity may be a factor. Autosomal dominant inheritance in certain families. Two possible loci have been described from India at 4q24-q28 and 10q21.3-q22.3
Population groups selectively affected: Hot water epilepsy has been reported from all over the world, mostly from India and Turkey. Hot water epilepsy in infancy is commonly reported from Japan.
Occupation groups selectively affected: None preferentially affected

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Hot water epilepsy

Associated Disorders

Aquagenic urticaria
Dravet syndrome
Idiopathic reflex epilepsies
Infantile syncope

Differential Diagnosis

Aquagenic urticaria
Infantile syncope
Paroxysmal extreme pain syndrome
Hyperekplexia
Alternate hemiplegia of childhood
- Gastroesophageal reflux

Hot water epilepsy …

Hot water epilepsy

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Prognosis and complications

Clinical vignette

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Confusing conditions

Associated or underlying disorders

Diagnostic workup

Management

Outcomes

Special considerations

Pregnancy

References

Contributors

Authors

Editor

Patient Profile

ICD & OMIM codes

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Questions or Comment?

Also in This Category

Photosensitive occipital lobe epilepsy

Intermittent explosive disorder

Pyridoxine/P5P-dependent developmental epileptic encephalopathy

Reflex seizures

Anti-LGI1 encephalitis

Self-limited (familial) neonatal epilepsy

Epilepsy with auditory features

Alcohol withdrawal seizures

Hot water epilepsy

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Prognosis and complications

Clinical vignette

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Confusing conditions

Associated or underlying disorders

Diagnostic workup

Management

Outcomes

Special considerations

Pregnancy

References

Contributors

Authors

Editor

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

Photosensitive occipital lobe epilepsy

Intermittent explosive disorder

Pyridoxine/P5P-dependent developmental epileptic encephalopathy

Reflex seizures

Anti-LGI1 encephalitis

Self-limited (familial) neonatal epilepsy

Epilepsy with auditory features

Alcohol withdrawal seizures

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