Refractory seizures in a young army cohort.

This survey covered male Singapore citizens born in 1974 who were medically screened at the age of 18 years before enlistment for compulsory military service. Suspected epileptics were referred to government hospitals for further management. Out of 20,542 men, there were 121 epileptics, giving a cumulative incidence of 5 per 1000 by age 18 years. We had information on 106 (87%) of these individuals and were able to interview them and review their hospital records. Seventy-three of the 106 (69%) epileptics had generalised seizures while 14 (13%) had refractory seizures. There was no statistically significant racial bias amongst these epileptics. Unprovoked afebrile seizures occurred early in these patients, half of whom had seizures onset before 7 years of age. Nine refractory epileptics had a history of febrile seizures, 4 of which were complex febrile seizures. Magnetic resonance imaging identified mesial temporal sclerosis in 2 patients and a hypothalamic lesion in 1 patient. Computed tomographic scans revealed focal cortical atrophy in 2 patients. Nine other patients had normal imaging studies. Nine out of 14 (64%) patients with refractory epilepsy had partial seizures; 4 (29%) had generalised seizures and 1 (7%) was unclassified. This is in contrast to the distribution of the entire cohort of epileptics studied. Two out of 9 patients with refractory partial seizures (gelastic epilepsy and mesial temporal sclerosis) had undergone surgery while 6 of the other 7 patients refused to consider surgery.

Ischaemic stroke from cerebral embolism in cephalic fibromuscular dysplasia.

Fibromuscular dysplasia (FMD) of the internal carotid arteries and its relationship with focal cerebral ischaemia is unproven. This vasculopathy is often detected incidentally during a cerebral angiogram for non-ischaemic cerebral events. FMD affects the proximal one-third of the internal carotid artery in almost all cases and is bilateral in 60% to 85%, with middle-aged women affected in 85% of the cases. Ischaemic stroke has been postulated to result from severe stenosis or thrombotic occlusion at the FMD site. Cerebral embolism from FMD has rarely been reported. We report 3 young patients with acute ischaemic stroke who had FMD on cerebral angiography. They presented with a focal hemispheric stroke where the probable pathophysiology is embolism to the distal internal carotid artery from thrombus formed at the proximal FMD site. The patients were all males, with unilateral proximal internal carotid artery FMD lesions and occlusion of the internal carotid artery distally on the same side. All were extensively investigated and no other causes for stroke were found.

Computed tomography in patients with recurrent seizures.

Eighty patients had Computed Tomography (CT) performed for evaluation of epileptic seizures. Abnormal scans were found in 37 of the 80 patients (46.3%). Focal CT abnormalities were seen in 26 of the 80 patients (32.5%). Tumors were present in four and arteriovenous malformation (AVM) in three. Simple partial motor seizures were most strongly correlated with abnormal scans (five, 45.4%). Nineteen out of 21 patients with focal electro-encephalographic (EEG) abnormalities had focal CT abnormality compared to one out of 15 of those with generalised abnormality. 88.9% of patients with hemiplegia had abnormal scans. Whilst focal EEG abnormalities and abnormal neurologic signs pointed to a higher likelihood of CT abnormality, two subjects who were shown to have vascular malformations had normal EEG and neurologic exams. Routine CT scanning for evaluation of patients with recurrent seizures is advocated.

The localizing value of downbeat nystagmus.

Downbeat nystagmus (DBN) is a primary position nystagmus with the fast phase in a downward direction. It is a rare but distinctive disorder of ocular motility and usually localizes the lesion at the posterior fossa. Four patients with DBN were seen in the department. One had a medullary glioma, and another congenital basilar invagination. The other two were initially diagnosed as demyelinating disease. One was subsequently found to have Arnold Chiari Malformation on magnetic resonance imaging (MRI). Review of the literature showed that cerebellar ectopia (Arnold Chiari Malformation) is the commonest cause of DBN. However 1/3 of reported cases have no obvious cause. DBN is of such high localizing value that we recommend MRI of cervicomedullary junction for all patients with DBN to exclude cerebellar ectopia or medullary lesion.

Cryptococcal meningitis.

Cryptococcosis is a systemic fungal disease and meningitis is the most serious complication. The purpose of this study is to define problems related to its diagnosis and treatment. This is a retrospective analysis of 25 patients admitted from January 1978 to December 1981. All patients had cryptococcal neoformans meningitis proven by culture of cerebrospinal fluid. One patient had a predisposing illness, being on immunosuppressant therapy after a renal transplant 2 years ago. A progressively severe headache of recent onset was the most striking presentation. Fever was frequently absent as a symptom. Cranial nerve palsies were commonly seen. Impairment of consciousness and areflexia signified a poor prognosis as all four patients who died early in the course of treatment were comatose and two of them were areflexic on admission. In newly suspected cases at least 3 separate lumbar punctures are recommended as initial smears or cultures may be negative. Cerebral CT scans were abnormal in 12 patients and those with cerebral oedema or hydrocephalus had a poorer prognosis. Combined amphotericin B and 5-fluorocytosine therapy was the treatment of choice. If there is no relapse 3 years after completion of treatment, patients are considered as cured. Positive smears may remain for years after completion of treatment and retreatment is only indicated if the cultures are positive. Twenty patients are alive today and none of them have relapsed. One patient had vasculitis of both anterior cerebral arteries as a result of cryptococcal meningitis.

Early and predominant respiratory muscle involvement in motor neurone disease.

We report a 61 year old man with motor neurone disease who presented with dyspnoea and ventilatory failure when the limb and bulbar muscles were relatively spared. This unusual pattern of predominant respiratory muscle involvement persisted throughout the twenty-one month course of his illness.

Phlebography of the internal spermatic vein (and the ovarian vein).

Phlebography of the internal spermatic vein was performed on 87 patients. In only two cases were slight complications (fever and chill) recorded after the examinations. The patients felt only a little discomfort during the examination. They did not have to be hospitalized before or after the examinations. In a few cases we saw sufficient values distally, at the level of L4-5. We have interpreted these cases as being normal.

Primary diffuse alveolar septal amyloidosis.

The case is reported of a 61-year-old man with primary diffuse alveolar septal pulmonary amyloidosis. Amyloid infiltration of the heart and other organs was also observed. The clinical findings and laboratory investigations reveal features characteristic of defective gas transfer with pulmonary oedema due to left ventricular failure from myocardial involvement.