In a retrospective study of 4,294 consecutive postmortem cases, there were 15 with histologically proved sarcoidosis, including 4 with CNS involvement. Whereas in previously reported autopsy series the prevalence of neurosarcoidosis was 15%, it is 27% in this review. Five cases with incidental, clinically inapparent sarcoidosis died at a mean age of 54.6 years; those six with systemic sarcoidosis, not involving the CNS, died at a mean age of 47.0 years; while those four with a variety of neurologic manifestations in addition to systemic sarcoidosis died at a mean age of 38.2 years. Thus, it appears that at one end of the spectrum of morbidity, sarcoidosis may cause no or only trivial symptoms and permit prolonged survival. At the other extreme, sarcoidosis may affect a younger population more severely. CNS involvement occurs relatively early in the course and is rather rapidly progressive, accounting for the poor prognosis, despite appropriate medical and neurosurgical management. In its turn, neurosarcoidosis may present with a variety of signs and symptoms, depending on the site of involvement along the craniospinal axis. This feature is illustrated by the manifestations of compression myelopathy, hydrocephalus with dementia, hydrocephalus with seizures and ataxia, and anosmia, blindness, seizures, and diabetes insipidus. In addition, one patient developed a Nocardia brain abscess as a complication of the altered immune system in sarcoidosis.

A unique case of Huntington’s disease is reported because of the extremely early onset and death, and the atypical mode of presentation including severe behavioural problems and a negative family history. Although rare, Huntington’s disease must be considered along with the established degenerative disorders of white and gray matter peculiar to the pediatric population when one examines an infant or child with progressive motor deterioration, rigidity, mental retardation and behavioural abnormalities. Computed tomography is a reliable and non-invasive method of establishing the diagnosis during life.

We report multimodal evoked response studies in eleven children with ataxia telangiectasia. The ABRs were normal in all but one patient despite the severity of the disorder. The SERs were abnormal in all of the patients, the waveforms being either absent or of delayed latency, the abnormalities were more marked in the older patients. Abnormal motor and sensory nerve conduction studies were also found in all but the younger few patients. Most notably, the VERs were abnormal in all but 2, the older patients had delayed VERs and most had small amplitude responses. The electrophysiological profile of ataxia telangiectasia patients on the multimodal evoked responses differs from that found in certain other degenerative ataxias studied and may have some diagnostic utility.

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.

Two paragangliomas resected from the cauda equina had similar morphology, both containing cells having dense core secretory granules with no associated sustentacular or supporting cells. The cells were arranged in characteristic lobules that were surrounded by basement membrane and separated by a fibrovascular stroma. This tumor, although rarely found elsewhere in the central nervous system, does occur in the region of the filum terminale, and causes a clinical syndrome typical of tumors in that area.

A 33 year old man developed bilateral leg weakness two years following radiotherapy to the pelvis and lower abdomen for the treatment of a seminoma. Clinical and electrophysiological examinations and nerve and muscle biopsies suggest that this is an example of either post-irradiation myelopathy selectively affecting the anterior horn cells in the lower spinal cord or a purely motor lumbo-sacral radiculopathy.

A 79 year old male acutely developed a Brown-Sequard syndrome two weeks following resection of renal cell carcinoma. The subsequent clinical course was marked by subacute progression of spinal cord signs. Pathologic studies showed extensive multifocal spinal cord necrosis that could not be attributed to vascular occlusion or to vasculitis. This case appears to be a rare example of paraneoplastic subacute necrotic myelopathy.

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.

A case of tuberous sclerosis diagnosed at autopsy in a neonate at 28 weeks gestation is described. This is believed to be the earliest case yet reported. The CNS lesions consist of cortical tuberosities, heterotopic white matter nodules and subependymal nodules. These nodules consist predominantly of giant astrocytes but a small undifferentiated cell component is present as well. Multiple cardiac rhabdomyomas are also present. An explanation for neuronal hypocellularity within the cortical tuberosities is suggested.

Seventeen years before the Scharrers demonstrated neurosecretory activity of neurons within the brains of vertebrates and invertebrates and convinced the scientific world of the existence of a neuroendocrine system, Carl Caskey Speidel (1917) had identified glandular neurons in the spinal cord of the skate, postulated a neurosecretory function, and performed experiments to prove his hypothesis. The correct conclusions that he formulated from morphologic observations were not believed by biologists until ‘proved’ by the Scharrers, who acknowledged his pioneering contributions. The Scharrers studied many species and even demonstrated neurosecretion in nemertine worms, now believed to be closely related to the ancestors of all vertebrates. Evolutionary theorists had speculated on neuroglandular function as early as 1900, and the contributions of comparative neuroanatomists to this field have resulted in a major medical advance.

A patient with long standing sustained unilateral headache (“background vascular”) and occasional multiple jabs received prompt and lasting relief from indomethacin. The effectiveness of the drug was tested in a placebo controlled double-blind trial. Indomethacin may be of value in some types of sustained headache.

Two patients with acute hemorrhagic leukoencephalopathy, one of which was pathologically proven, were serially studied with CT scanning. Both patients showed marked distinctive low density white matter changes throughout both hemispheres, which correlated with clinically involved areas. One patient recovered from the disease, perhaps due to steroid treatment, and showed slow but complete resolution of CT scan changes. We feel that CT scan findings significantly help in the diagnosis of this disease, which may be amenable to early treatment with steroids.