The clinical and EEG profiles of 13 cases of eating epilepsy are described. Temporal lobe epilepsy (TLE) formed the seizure type in 12 cases. In all but one case, the seizures were precipitated only by eating a heavy meal. In ten cases EEG showed interictal seizure discharges. EEG recordings were obtained from 12 patients during and after eating. In two patients clinical attacks (TLE) could be induced by eating in the laboratory. There was a good response to conventional anticonvulsants in only 3 cases. Alteration of eating habits helped in controlling reflex seizures in 2 cases. Various mechanisms of reflex precipitation of seizures by eating are discussed.

No abstract

Rippling muscle disease (RMD) has previously been reported as a skeletal myopathy that was attributed to a defect in the sarcomere. Here we report a new form of RMD that is more severe, characterized by fatal arrhythmic cardiomyopathy and delayed bone age. Mortality has previously not been associated with RMD. With this report we hope to raise awareness that a subset of patients with this clinical entity are predisposed to severe cardiac disease.

No abstract

Between 1990 and 1992, 14 children were seen in whom a clinical diagnosis of olivopontocerebellar atrophy (OPCA) had been made. The majority of patients presented with cerebellar ataxia and hypotonia. Five children had a family history of a similar illness in first-degree relatives. All cases had undergone clinical and neurologic examinations, routine laboratory tests and cranial CT. CT features were graded to quantitate the degree of atrophy in each cerebellar hemisphere, vermis and brain stem. All patients had varying degrees of atrophic changes of cerebellum, brain stem and cerebrum. These CT features appear to be distinctive enough to enable the diagnosis of OPCA to be made.

Background: Pathological laughter can be a sign of epilepsy, psychiatric illness, effect of toxins or psychotropic drugs or of structural brain disease. Method: Case Studies. Result: Two patients developed inappropriate uncontrollable laughter following intravenous sodium valproate for treatment of epilepsy. Conclusion: Pathological laughter can be a side effect of intravenous sodium valproate. Can. J. Neurol. Sci. 1998; 25: 252-253 Sodium valproate (SVA) is a first line antiepileptic drug for absence, myoclonic and tonic-clonic seizures. During the past decade an intravenous formulation of SVA has become commercially available and its safety established. RESUME1 Pathological laughter (PL) is laughter that is inappropriate, unrestrained, uncontrollable or dissociated from any stimulus. 2 We report two cases of PL in patients following administration of intravenous SVA. CASE REPORT Patient 1A 20-year-old male Omani (Arab) was being followed up by us for myoclonic jerks and generalized tonic-clonic seizures since 1990. CT scan brain, blood urea, electrolytes, liver function tests and plasma lactic acid were normal. EEGs done during his previous outpatient visits had shown bifrontal sharp wave and generalised spike discharges. A diagnosis of juvenile myoclonic epilepsy was made. He was being treated with SVA 1600 mg/day and clonazepam 6 mg/day. He was admitted in January 1997 with recent exacerbation of his epilepsy with 3-4 generalized tonic-clonic seizures/day for the previous two days. There was no history of exposure to other neurotoxins. At admission, he was in a post ictal, drowsy state. There were no other neurological deficits. At admission, his free SVA level in the serum was 23 |imol/L (N 15-105). He was treated with an intravenous infusion of SVA 800 mg (16/kg body weight) over one hour's time. One hour later, he started laughing uncontrollably. He was conscious but drowsy and could obey simple commands, but could not speak. The laughter was intermittent, spontaneous, unprovoked and could not be suppressed. He had no myoclonus or feelings of mirth during the laughter. SVA free level done at the time of his laughter had risen to 84 u.mol/L. EEG done at that time showed a background of continuous excessive beta fast activity. There were no epileptiform discharges. A decision was made to omit the subsequent oral dose of SVA and the laughter ceased after 6 hours. Subsequent to this, he was reintroduced on his usual oral dose of SVA and Clonazepam. He did not have any further episodes of abnormal laughter. Patient 2A 17-year-old male Omani patient was being treated for myoclonic and generalised tonic-clonic seizures with 600 mg carbamazepine and 600 mg SVA daily, for 4 years. His mentation and neurological examination were normal. CT scan brain and blood biochemical profile were normal. EEG showed brief generalised bursts of polyspike and sharp discharges lasting less than one second and he was diagnosed to have juvenile myoclonic epilepsy. He was admitted in August 1997 with a cluster of seizure...

Following a box jelly fish sting, a 52 year old Chinese fisherman developed acute abdominal distension, inability to pass urine and failure of erection. Examination revealed gaseous abdominal distension and a distended urinary bladder. Absence of lachrimation and absence of changes in the R-R interval in the ECG during breathing and carotid sinus massage gave further evidence of parasympathetic dysautonomia. The patient made a complete recovery. The case highlights the occurrence of reversible parasympathetic dysautonomia following box jelly fish sting.

No abstract