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Stuttering or reflex seizure? A case report.

Stuttering is characterized by involuntary syllabic repetitions and interruption in the smooth flow of speech. The exact cause of primary stuttering remains a matter of debate but a frontal dysfunction has been evoked. On the other hand, acquired stuttering is uncommon. We report a case of reflex epilepsy in which seizures were triggered by reading aloud or stressful conversation. Each paroxysmal event in left frontal region was associated clinically with a language disorder mimicking stuttering. Our observation suggests that reflex frontal focal epilepsy could be a putative etiology for acquired stuttering.

Aged↗

Fold prediction and comparative modeling of Bdm1: a probable alpha/beta hydrolase associated with hot water epilepsy.

Hot water epilepsy (HWE) is a benign and rare form of reflex epilepsy that occurs most commonly in humans. Bdm1 is one of the proteins whose mRNA transcript is overexpressed during HWE in a rat model. We show, by sequence analysis and fold recognition methods, that Bdm1 has strong structural similarities to alpha/beta hydrolases like the thioesterases. A three-dimensional model derived by comparative modeling methods allowed the search for catalytic residues using a flexible functional template characteristic of these enzymes. We predict that Bdm1 might be regulated by homocysteine levels by means of direct participation in degradation pathways.

Amino Acid Sequence↗

Gamma-band phase clustering and photosensitivity: is there an underlying mechanism common to photosensitive epilepsy and visual perception?

Photosensitive epilepsy (PSE) is the most common form of human reflex epilepsy, appearing in up to 10% of epileptic children. It also offers a highly reproducible model to investigate whether changes in neuronal activity preceding the transition to an epileptic photoparoxysmal response (PPR) may be detected. We studied 10 patients with idiopathic PSE (eight female, mean age 26 years, range 9-51 years) using magnetoencephalography. In addition, we also studied the responses of five normal controls (mean age 24 years, age range 9-35 years) and three non-photosensitive epileptic patients (mean age 10 years, range 8-11 years). Spectral analysis of the MEG signals recorded during intermittent photic stimulation revealed relevant information in the phase spectrum. To quantify this effect, we introduced a second order response feature of the stimulus-triggered visual response preceding the PPR: the phase clustering index, which measures how close the phases of successive periods are grouped for each frequency component for all periods of the stimuli applied. We recorded a total of 86 PPRs, including several absence seizures, in nine of the 10 patients. We found that an enhancement of phase synchrony in the gamma-band (30-120 Hz), harmonically related to the frequency of stimulation, preceded the stimulation trials that evolved into PPRs, and differed significantly from that encountered in trials not followed by PPR or in control subjects. This novel finding leads us to postulate that a pathological deviation of normally occurring synchronization of gamma oscillations, underlying perceptional processes, mediates the epileptic transition in PSE.

Adolescent↗

Bathing epilepsy.

A patient who had complex partial seizures provoked by bathing is reported. All the attacks occurred during or immediately after bathing, irrespective of water temperature. The semiology was suggestive of a seizure focus in the mesial temporal lobe. Though there are some similarities with hot-water epilepsy, this case appears to be a distinct type of reflex epilepsy.

Adult↗

Pattern-sensitive epilepsy: electroclinical characteristics, natural history, and delineation of the epileptic syndrome.

PURPOSE: To elucidate the electroclinical features and long-term outcome of patients with pattern-sensitive epilepsy. METHODS: We reviewed the clinical and electroencephalographic (EEG) findings of 73 (43 female and 30 male) patients in whom pattern-sensitive epilepsy was diagnosed at Mayo Clinic (Rochester, Minnesota, U.S.A.) from 1950 through 1999. We contacted patients and their relatives by letter or telephone to obtain the latest seizure and quality-of-life outcomes. RESULTS: The median age at onset of seizures was 12.8 years (range, 0.6-32.9 years). Most patients had absence, myoclonic, or generalized tonic-clonic seizures. Interictal epileptiform discharges in the EEG were detected in 61 (83.6%) patients and were generalized in 54 (74%). Paroxysmal epileptiform discharges in the EEG elicited with standard patterns were all generalized in two thirds of patients but were restricted to the posterior head region in one-third. Eight (11%) patients did not exhibit photosensitivity. Television was the most common precipitant [30 patients (41%)]. Twenty-nine patients gave a clear history of one or more seizures precipitated while viewing environmental patterns such as window screens, garments, tablecloths, and ceiling tiles; the rest of the patients admitted that they preferred to avoid looking at patterned objects because these objects made them uncomfortable. The electroclinical features suggested juvenile myoclonic epilepsy in 14 patients, progressive myoclonus epilepsy in three, progressive familial cerebellar ataxia with myoclonus in two, and severe myoclonic epilepsy of infancy in one. During a median follow-up period of 15.7 years, 25 (45.5%) of 55 patients who were followed up for > or =5 years achieved complete seizure remission. The median age at remission was 24.4 years. The absence of progressive neurologic disease was correlated significantly with remission; a family history of seizures showed a trend in favor of remission. More than two thirds of the patients did not consider the seizures an impediment to their family life or to educational and occupational achievements. CONCLUSIONS: Although pattern sensitivity as a trait occurs in various epileptic syndromes, pattern-sensitive epilepsy is a readily distinguishable subtype of the visually provoked reflex epilepsies. In our opinion, the location and extent of the excitable region or regions within the visual cortex concerned with different attributes of visual function dictate susceptibility to a specific trigger (intermittent light, pattern, or color) or closely related multiple triggers and the resultant electroclinical phenomenon.

Adolescent↗

Hot water epilepsy occurring at temperature below the core temperature.

A 6-year-old girl had water reflex epilepsy occurring at lower temperature than the core temperature. Seizures episodes consisted of a loss of consciousness absence followed by left predominant hypotonia with right fronto-temporal high voltage slow waves on the ictal-EEG. Seizures were only observed when the water was poured on scalp or face. Neuropsychological evaluation showed frontal dysfunction (Rey's figure). MRI study was normal. Oxcarbazepine permitted the disappearance of seizures and an improvement of executive disorders. In this case, the pathophysiological mechanism cannot be a hyperthermic related event. The temperature control as treatment of hot-water epilepsy could be used after the exploration of its implication in seizure induction.

Anticonvulsants↗

Eating epilepsy associated with a deep forebrain glioma.

Seizures associated with deglutition are rare, and previously reported cases of so-called eating epilepsy have not identified specific electrophysiological or anatomical foci. We evaluated a 14-year-old with a deep hemispheric astrocytoma in whom focal seizures were consistently triggered by eating. Electroencephalograms were consistent with a deeply located seizure focus. Eating epilepsy should be grouped with reflex epilepsies that may originate with the diencephalon.

Adolescent↗

Musicogenic epilepsy.

The problem of musicogenic epilepsy is reconsidered by the author in the light of a case of temporal lobe seizures with secondary generalization precipitated by any type of music. The inclusion of musicogenic epilepsy in the chapter of reflex epilepsies is discussed and critizised by the author considering that music sets in motion too many structures and cannot be reduced to crude perception of rhythm and melodies. It is considered that three levels of integration are involved in music: a sensory level (the lowest one) an emotional and waking level (the second one) and finally a highest level, allowing aesthetic appreciation. It is the opinion of the author that musicogenic epilepsy has to do mostly with the limbic brain, that is with the second level of integration. Musicogenic epilepsy therefore should be better included in the dismissed chapter of affective epilepsy of Bratz.

Acoustic Stimulation↗

Eating epilepsy.

Eating epilepsy is a rare type of reflex epilepsy. A 24 years-old male with eating reflex complex partial seizures was submitted to clinical, neurological, neuroradiological and EEG studies. Neurologic and CT examinations were normal. EEG recordings including video-EEG monitoring during meals disclosed focal abnormalities related to both temporal lobes prevailing at the left side and secondary bilateral synchrony mainly in more anterior regions. Ictal findings were similar to the interictal secondary bilateral synchrony except for its longer duration. PB, VPA and DPH monotherapies were ineffective. High dose CBZ monotherapy yielded good but incomplete seizure control. Since a big number of precipitants could be involved, no specific physiopathological basis could be established.

Adult↗

[Hot water epilepsy: a benign and underestimated form].

BACKGROUND: Hot water epilepsy belongs to the group of reflex epilepsies. Seizures are provoked by hot water, due to the association of both cutaneous and heat stimuli. Described mainly in India and Japan, it seems to be rare in Europe where it occurs in young children. CASE REPORTS: Five infants aged between 6 months to 2 years had seizures during bathing with activity arrest, hypotonia and vasoactive modification. Sometimes clonic movements could be observed. The diagnosis was confirmed by EEG recorded during bath in the fives cases, with video for two of them. The course of the seizures and of the psychomotor development were favorable. CONCLUSION: Hot water epilepsy is a benign epilepsy. Its incidence could be underestimated because seizures can be confused with febrile convulsions or vagal fits.

Baths↗

Hot water epilepsy.

A case of hot water epilepsy is presented and the literature on the subject is reviewed. The data show that hot water epilepsy is a benign form of reflex epilepsy occurring mainly in children. Males are affected more than females. The triggering stimulus is immersion in hot water (over 37 degrees C). The seizure is usually psychomotor, although generalized convulsions may occur. The EEG findings indicate that the abnormality lies in the temporal lobe.

Child, Preschool↗

[Primary reading epilepsy: therapeutic efficacy of clonazepam in one case].

Primary reading epilepsy is a rare form of reflex epilepsy, in which reading is the specific stimulus evoking attacks. The authors report a case of an 18-year-old man who since the age of 17 presented myoclonic jaw jerking provoked exclusively by reading. In one episode, in which reading was not interrupted, jerks were followed by a generalized convulsive seizure. EEG with routine activating procedures was normal, while EEG recorded during reading showed bilaterally synchronous paroxysmal small-voltage spikes, more prominent in frontocentral regions, coinciding with jaw myoclonus. Complete clinical seizures control and EEG normalization were achieved with clonazepam 2 mg daily in a 24-month follow-up.

Adolescent↗

[Epilepsy and accidents: what is the risk in children?].

BACKGROUND: Constant distress and overprotection of parents result from the apprehension and misinformation received, regarding epilepsy of their children and the risk of accidents occurring due to a seizure. Large retrospective studies show that the epileptic children are not more often concerned than the general population. CASE REPORTS: One hundred and ninety-eight epileptic children without permanent motor or mental handicap were followed between 1971 and 1981, covering 1056 years of child's life. They were compared to 3822 injured children admitted to the surgical pediatric emergency unit between 1983 and 1992. RESULTS: Only six accidents occurred because of a seizure in the first group of epileptic children. There were neither deaths nor sequelae. Four of these six accidents revealed an unknown epilepsy. Among the 3,822 injured children, 19 were epileptic but only four of these 19 accidents were caused by a seizure. There were no sequelae. CONCLUSION: The majority of epileptic children without associated handicap are able to have all the normal activities of healthy children. Only severe epilepsies with frequent refractory seizures, some reflex epilepsies and some cases before the seizures are under control require constant supervision.

Accidents↗

[Reflex benign myoclonic epilepsy of childhood. Apropos of a new case].

INTRODUCTION: Recently a series of cases has been reported characterized by myoclonic crises similar to those occurring in benign myoclonic epilepsy of childhood. However, these crises only occurred after unexpected tactile or auditory stimuli. These clinical conditions represent a new epileptic syndrome, which is age-dependent and has been called benign myoclonic epilepsy of childhood. CLINICAL CASE: We present the case of a 12 month old girl with myoclonic crises which occurred only after auditory or tactile stimuli. The myoclonia could be set off whilst awake or asleep. No other types of crises or neurological changes were seen. A brother of the patient had had febrile convulsions. The EEG recorded during the crises showed generalized brief spike-and-wave discharges at 3 cycles/second. The intercritical EEG was normal whilst awake, but during sleep showed brief generalized discharges. After treatment with valproate was started the crises became less frequent. CONCLUSIONS: The case we describe is similar to those described by Ricci et al in 1995. We, therefore, consider it to fit the concept of reflex myoclonic epilepsy of childhood of benign character. We consider that this condition should be differentiated from other reflex epilepsies and epileptic syndromes with a predominance of myoclonia, including benign myoclonic epilepsy of childhood.

Acoustic Stimulation↗

[Myoclonic epilepsies in infancy].

INTRODUCTION: The presence of myoclonus in a patient has different meanings: there exist myoclonus without encephalopathy or epilepsy (sleep myoclonus), encephalopathies with non-epileptic myoclonus (Kinsbourne's syndrome), encephalopathies with epileptic myoclonus (progressive or not), epileptic encephalopathies with myoclonic seizures (the classic West and Lennox-Gastaut syndromes) and myoclonic epilepsies. DEVELOPMENT: Main types of myoclonic epilepsies (benign childhood myoclonic epilepsy, severe-polymorphic-myoclonic epilepsy, juvenile myoclonic epilepsy, childhood familial myoclonic epilepsy, benign reflex myoclonic epilepsy, as well as progressive myoclonic epilepsies, among which progressive myoclonic epilepsy (PME) type 1 (Unverricht-Lundborg's disease) and PME2 (Lafora type) are prominent, whereas other entities, such as those related to neuronal ceroid lipofuscinosis or mitochondrial cytopathies are discussed more briefly) are reviewed, analyzing clinical, EEG and therapeutic issues, while the most recent contributions in the field of genetics are considered. CONCLUSIONS: Myoclonic epilepsies constitute a very heterogeneous type of epilepsy, both in their origin and in their prognosis, with favorable forms of course along with other progressive and refractory forms with an ill-fated prognosis.

Adult↗

Hot-water epilepsy in an adult: ictal EEG, MRI and SPECT features.

Reflex epilepsy constitutes a rare form of epileptic seizures. We observed a 20-year-old man who presented with seizures induced by immersion in hot water. The trigger stimulus was specific. Contrast CT scan and MRI were all normal, not revealing any structural lesion. Ictal EEG recorded during a hot bath showed focal epileptic discharges in the left temporo-occipital area. Interictal SPECT showed a hypometabolism in the same cerebral region. Neuroimaging studies were rarely performed in this uncommon type of epilepsy. Nevertheless, in our case the result of the SPECT suggests a localized functional disturbance in the emergence of the disorder.

Adult↗

Two visual mechanisms of photosensitivity.

PURPOSE: Photosensitive epilepsy is the most common of the "reflex" epilepsies. Precipitated by television viewing, flickering light, or specific visual patterns, it is the cause of seizures in 10% of young people with epilepsy. Photosensitivity is associated with two types of EEG abnormalities: photoparoxysmal responses (PPRs) and occipital spikes (OSs). It is unclear whether these abnormalities are mediated by different mechanisms, and furthermore, the clinical significance of OS is unknown. METHODS: By using our previously established population of patients with photosensitive epilepsy, all showing EEG abnormalities on intermittent photic stimulation or pattern stimulation, we examined the effects of pattern contrast, spatial and counterphase temporal frequency, and colour on these abnormalities. RESULTS: PPRs and not OSs show linear contrast dependency and are elicited by stationary stimuli and by non-colour-opponent isoluminant stimuli. CONCLUSIONS: PPRs and OSs are generated independently by the parvocellular and magnocellular visual systems, respectively. The results add support to the hypothesis that only PPRs and not OSs are clinically significant.

Cerebral Cortex↗

Incidence of photosensitive epilepsy in unselected Indian epileptic population.

The recognition of photosensitivity depends upon the finding of an abnormal EEG response to intermittent photic stimulation (IPS). Photosensitive epilepsy being the most common form of reflex epilepsy, has been studied most extensively and is reported to account for 5-10% of the epileptic population in general. One thousand unselected epileptics hailing from different parts of Northern India were screened for photosensitivity, both clinically and on a standard protocol of IPS-provoked EEG recordings. Six patients (mean age 14.5 +/- 3.56 yr) were found to be photosensitive: 4 had generalized, 1 had complex partial and 1 mixed seizures; 3 had history of seizures provoked by visual stimuli. The baseline EEG in 4 patients showed generalized and 2 partial, with secondary generalized, epileptiform discharges. On IPS, similar EEG findings were obtained with a wide range of stimulus frequency (6-60 cycles/s). There is a low incidence of photosensitivity in our epileptic population, for which we believe, genetic factors are responsible.

Adolescent↗