The serious, late complication of superficial cerebral hemosiderosis, which appears after several years in 1/4–1/3 of patients who have undergone hemispherectomy, has resulted in recent years in a considerable reluctance to carry out this operation despite the fact it has proved to be highly effective in patients with medically refractory seizures associated with hemiplegia. Preservation of a small portion of the hemisphere, usually the frontal or occipital pole, has proved to be effective in preventing this late complication, but at the cost of a significant reduction in the effectiveness of the operation in reducing the patients’ seizure tendency. Preserving the frontal and occipital poles but disconnecting them from the rest of the brain, resulting in a functional complete but anatomical subtotal hemispherectomy, retains the therapeutic effectiveness of a complete hemispherectomy while still protecting adequately against the serious late postoperative complication of superficial cerebral hemosiderosis and its associated neurologic deterioration, hydrocephalus and sometimes death.

Periventricular/intraventricular hemorrhage occurs commonly in the premature newborn. Recent studies indicate an incidence of 35–45%. Following PVH/IVH, the likelihood of developing hydrocephalus is related to the severity of the hemorrhagic lesion. Ventricular dilation may be due to an obliterative arachnoiditis affecting principally the posterior fossa or, less commonly, due to obstruction of flow of cerebrospinal fluid within the ventricular system by clots or other debris. With moderate to severe hemorrhagic lesions, ventricular dilation may occur at the time of PVH/IVH. More commonly, progressive dilation begins one to three weeks following PVH/IVH. The classical clinical signs of hydrocephalus, ie. bulging of anterior fontanelle and inappropriate increase in head circumference, may not appear for days to weeks following onset of ventricular dilation. The precise significance of such normal-pressure hydrocephalus in the genesis of brain injury in the newborn is unknown. Following diagnosis of PVH/IVH, close surveillance of ventricular size with ultrasound scans is indicated. When there is slowly progressive ventricular dilation with normal intracranial pressure, the choice of therapy is made difficult because of frequent spontaneous arrest in such cases. Several modes of therapy have been reported including drugs to decrease the formation of cerebrospinal fluid and the use of serial lumbar punctures. When ventricular dilation is rapid with intracranial hypertension, ventricular drainage is indicated.

Thirty-seven patients with advanced Parkinson’s disease who initially tolerated, and responded to bromocriptine therapy were followed for 12 to 50 (mean 28) months. Using a method of gradual increase of bromocriptine, with concomitant levodopa reduction, the peak effect of the drug was apparent by three months, at which time the mean daily dose of bromocriptine was 23.9 mg and Sinemet (levodopa + carbidopa) had been reduced by 34 percent.

Eight patients had sustained improvement without further drug changes for an average of 29 (range 14–50) months. After periods of improvement varying between 3 and 30 months, 29 patients had a fall-off from peak effect. Peak effect was regained in 21 of these 29 patients for an average of 16 additional months by initially increasing bromocriptine or Sinemet, or by eventually increasing both drugs. The main adverse effect was a confusional state which necessitated late withdrawal of bromocriptine in four patients. The best results were in younger patients with end-of-dose deterioration and levodopa induced dyskinesias.

With cautious introduction, and intermittent dosage adjustment, bromocriptine can be of long-term benefit to patients with advanced Parkinson’s disease. The majority of patients have a gradual late fall-off in effect which can frequently be reversed with dosage adjustment.

Twenty-seven patients with epilepsy as the only manifestation of a cerebral AVM were seen at the MNI/MNH from 1973 to 1981. The nine females and eighteen males between the ages of fourteen and fifty-four years (Mean 25.2 years) had epilepsy for an average of 3.5 years prior to the diagnosis of AVM.

Seven patients had primarily generalized seizures, ten patients had partial seizures with complex symptoms, fourteen patients had partial seizures with elementary symptoms, and thirteen patients had secondarily generalized seizures. Although there was no history of intracranial hemorrhage, seven patients had negative hemispheric signs (hemiatrophy, hemianopia, dysphasia, hemiparesis), two had impaired mentation, and two were ataxic. The EEG, normal in ten cases, showed non-epileptiform activity in six cases, and focal epileptiform activity in eleven cases. Angiographic and/or histological examination revealed eight small (less than 2 cm in diameter) and nineteen large lesions. Fifteen AVMs involved the frontal lobe, ten the temporal lobe, five the parietal lobe, and one the occipital lobe. Angiography failed to demonstrate three frontal and three temporal AVMs.

Plain CT scanning demonstrated a high or low density lesion without a mass effect in sixteen cases, enlargement of the ipsilateral ventricle or Sylvian fissure in seven cases, and diffuse ventricular enlargement in three cases. CT scanning was performed after the intravenous injection of contrast material in nineteen cases and demonstrated vascular enhancement in fifteen cases and an abnormal blood vessel in six cases. Two angiographically occult AVMs demonstrated vascular enhancement with infusion CT scanning, thereby demonstrating the vascular nature of the lesion where angiography had failed to do so.

Epileptic seizures can be evoked in chicks homozygous for the epileptic seizure gene (epi, epi) by elevating their body temperature using microwave diathermy. These seizures precede and differ in motor seizure pattern from a second clonic-tonic seizure produced by hyperthermia in both epileptic and carrier (heterozygote, Epi, epi) chicks. Hyperthermia did not evoke seizures in adult epileptic chickens. Phenobarbital delayed the onset of epileptiform seizures whereas phenytoin and valproate had no effect. These data suggest that epileptic chicks may provide a suitable model for studies on febrile convulsions.

A recent questionnaire survey of the life-effects of narcolepsy in 180 patients, 60 each from North American, Asian and European populations, compared to similarly distributed age and sex matched controls, documented multiple and marked effects on work, education, driving, accidents, recreation, personality, memory and other parameters. The data have now been further analysed according to the patients’ geographic (culturo-genetic) origin and to a number of other patient and illness variables. The three different geographic populations showed few significant differences for the some 160 life-effects items in the questionnaire. This strongly indicates that these are an integral part of the disease or of the human reaction to it. Most of the few significant population differences appeared cultural in origin (e.g., concerning driving records, personality changes), although a few may possibly reflect genetic differences (e.g., visual problems). Analysis of the pooled data according to respondees’ age, sex, age at illness onset, duration of illness and treatment led again to relatively few significant findings. It is concluded that, in general, once the disease has been diagnosed, all the major life effects are present and remain so. The results strongly support the contention that most life-effects are not related to the diagnostic ‘tetrad’ symptoms themselves but rather to excessive daytime sleepiness, the symptom most resistant to anti-narcoleptic treatment.

The murine ependymoblastoma is a transplantable tumor of cerebral origin. The growth pattern and survival times of the murine ependymoblastoma implanted peripherally and intracranially in non nude C57B1/6J mice have been found to be predictable and consistent when examined by means of Tumor Cell Dose Assessment (end point solution), Tumor Growth and Survival Assessment. The results suggest that a greater tumor cell dose is required to generate peripheral tumor take than brain tumor take. This difference may result from a greater immunologic response to tumor implanted peripherally than into the immunologically privileged brain.

Cerebral ventricle size was measured in one hundred normal people, age 10 to 50 years. The measurements were obtained from computed tomographic (CT) scan examinations, using the Huckman (1975) method. Normal ventricles were significantly bigger at age 45 than at age 15. These normals were compared to groups of patients with pseudotumor cerebri, water intoxication and cerebral edema, and cerebral hypoxia and edema due to cardio-respiratory arrest.

The relative ventricular size was the same in the normal group and all three disease groups. Measurement of ventricle size from CT scan images is not even a crude index of cerebral edema in these three diseases.

A 36 year old male developed bilateral sensorineural deafness as the chief manifestation of secondary syphilis. Cerebrospinal fluid showed pleocytosis. Treatment with penicillin and prednisone resulted in good recovery of hearing. Initial recovery seemed dependent on corticosteroids. Deafness can complicate acquired syphilis in both early and late stages of the disease and may be its sole manifestation. Early acquired syphilitic deafness is usually the result of a meningitis affecting the eighth nerve and responds well to treatment. These features are contrasted with those of late acquired syphilitic deafness.

During early 1982 one of the authors (RGL) was privileged to spend two months in Nepal as part of an exchange program between the University of Calgary, Faculty of Medicine and the new medical school at Tribhuvan University in Kathmandu. A short visit of this type does not qualify a Canadian neurologist to comment on the neurological-neurosurgical problems of a country such as Nepal with any degree of expertise. Nevertheless, as a result of a number of discussions between the two authors and interviews with many other physicians, both Nepalese and Western, it was possible to form some general impressions and draw some comparisons between neurologic illnesses in Nepal and Canada.

Disorders affecting the nervous system are common in Nepal. Some of the problems which are seen regularly would be considered rare and exotic in North America. However, despite the vast differences in culture, geography, dietary habits, and socio-economic conditions, there are some surprising similarities between the patterns of neurologic illness in Nepal and Canada. Disorders such as migraine, stroke, epilepsy and meningitis are well known to Nepali physicians. To appreciate the magnitude of health care problems in Nepal and to view neurologic illness in its proper perspective, it is helpful to have a few basic facts and figures concerning the geography and population of the country.

Rhabdomyosarcoma of the head and neck region in children frequently presents with otolaryngologic symptoms. But tumours which arise along the cranial base and involve the middle ear, mastoid or nasopharynx may be associated with specific neurological phenomena (Fleischer et al., 1975). The following case illustrates these points. It also records the unusual development of remote, intradural metastatic disease. Rhabdomyosarcoma rarely gains access to the subarachnoid space. When it does, the route of penetration is usually directly from ear through internal auditory meatus to cerebellopontine angle (Russell and Rubinstein, 1970). Our case developed a metastatic intradural spinal lesion without interposed disease.