The recent improvements in transsphenoidal microsurgery have once again placed the emphasis on the basophilic adenoma of the pituitary as the etiology of the disease originally described by Harvey Cushing fifty years ago.

In 75 operated cases, tumors were found in 60 cases: 52 were microadenomas, 4 were larger adenomas and 4 were invasive. In 15 cases, no tumor was found at surgery; a selective partial central hypophysectomy was performed in 11 cases and a total hypophysectomy in 4. Among the latter, 3 additional microadenomas were found at pathology. The majority of the microadenomas were located in the center of the gland most often near or within the neural lobe.

In 59 patients with a non invasive tumor, initial cure was obtained in 52 (88%); only 1 of the 4 patients with an invasive tumor was cured.

In the 12 cases without surgical or pathological tumor, partial or total hypophysectomy resulted in 10 cures (83%).

In human entrapment neuropathies the characteristic abnormalities in conduction are frequently limited to a short segment of the nerve. Recognition and precise localization of these discrete conduction abnormalities may require measurement of conduction over shorter lengths of the nerves than those lengths commonly employed in the clinical laboratory. Techniques are described for the more precise location of the primary conduction abnormalities in median, ulnar and peroneal nerve entrapments. Distinctive or atypical locations of the major conduction abnormalities may point towards different mechanisms in the pathogenesis of these localized neuropathies.

Cerebral cysticercosis was studied in 89 Mexican children. These patients were divided into two groups; those with proven diagnosis and those in whom the diagnosis was strongly suspected. The frequency of childhood cysticercosis at autopsy is significantly less than reported in adults from the same environment. Symptoms of initial cerebral invasion by the parasite include headache, fever, vomiting, and seizures. Cerebral edema is observed more frequently in children than in adults, and is the most common cause of intracranial hypertension. The cerebral edema generally is associated with an encephalitic syndrome, either a single bout or a chronic course with multiple relapses. Arterial hypertension, not previously described, was found in two probable cases. The variability of clinical symptomatology thus is great. Symptoms in childhood cerebral cysticercosis may be totally absent, moderate, or severe and ultimately fatal. Computed tomography of the brain is the most useful diagnostic aid, and determines the dynamic neuroradiologic sequence of this disease, since different images appear at each stage to permit its diagnosis. Uncalcified cysticerci may be demonstrated by contrast enhancement if associated with focal cerebral edema; images thus may disappear and reappear in the clinical course of the disease.

In this series of intracerebral hematomas from aneurysmal rupture, gathered from several neurosurgical services, certain morphological features were studied in detail. Patients with very large hematomas tended to have poor neurological grades on admission to hospital and their immediate discharge outlook was correspondingly poor. Ruptured middle cerebral and pericallosal artery aneurysms were relatively common causes of intracerebral hematomas. Patients with temporal lobe hematoma did relatively well; those with parietal hematoma did poorly. The larger the hematoma the less chance there was of developing cerebral vasospasm but the more likely was pre-operative brain herniation. The survival was more closely linked to size and location of the hematoma than to the location of aneurysm or the degree of midline shift.

One hundred multiple sclerosis (MS) patients were compared to healthy controls to determine the prevalence of diabetes mellitus in their families. Significantly, more MS patients than controls were diabetic or reported at least one first degree relative (parent, sibling, child) with diabetes. The relationship between MS and diabetes persisted when second degree relatives (grandparents, aunts and uncles) were taken into consideration.

A greater percentage of MS patients with another MS relative were diabetic or reported a first degree relative with diabetes mellitus than MS patients without an MS relative. However the difference was not statistically significant. Nor was there a significant difference when percentages reporting either a first or a second degree relative with diabetes were compared.

Topographical variations in the endogenous content of dopamine were studied in the rat caudate-putamen. For this purpose, a high performance liquid chromatography with electrochemical detection procedure for the determination of norepinephrine, epinephrine and dopamine in nervous tissue samples is described. Tissue preparation required only homogenization in acidic solution and centrifugation prior to application to the chromatograph. Detection limits in the low picogram range were found for the catecholamines separated. This assay was used in combination with a micropunch dissection technique. The performance of this analytical technique is illustrated by the detailed mapping of dopamine content in 35 subregions of the rat caudate-putamen. The distribution of dopamine varies along the major axis of the nucleus.

In an attempt to clarify issues of brainstem dysfunction and hearing thresholds in autistic children, we studied the Auditory Brainstem Responses (ABRs) in 32 children who clearly fit within the criteria of autism established by the National Society for Autistic Children (1977). ABRs were recorded between Cz and ipsilateral ear in response to click stimuli. Interwave latencies and auditory threshold in each ear were determined. Of the 32 children, 11 had moderate hearing loss (8 bilaterally) and 3 had severe to profound hearing loss, all bilaterally. 8 of the 14 with hearing loss also had associated features (e.g., perinatal encephalopathy). The I-III andI-Vinterwave latencies were significantly longer in the autistic children compared to normal control children; the increased conduction times were found mainly in the early portion of the auditory brainstem pathway. These data confirm some earlier reports of ABR abnormalities in autistic children and are concordant with some theories of the etiological basis of autism. The high incidence of hearing loss in these children is significant and routine ABR testing is recommended.

Although several cases of acute hepatic failure associated with the administration of valproic acid (VPA) have been reported, the pathogenesis of this problem remains unclear. We report the case of a 40 month old male with a chronic seizure disorder treated with VPA and phenytoin for two years, who developed sudden progressive neurological deterioration with evidence of increased intracranial pressure and hepatic failure. The clinical features closely resembled those of Reye’s syndrome suggesting the possibility of a common pathogenesis.

We report the case of a man who died at age 40 after a 9 year illness resembling a hereditary spinocerebellar degeneration, but accompanied also by brain stem signs and dementia. Autopsy disclosed lesions of combined spinocerebellar and ponto-cerebellar degeneration, but also damage to many brain stem nuclei. Prominent amyloid plaques disseminated through the brain indicated a relationship to the Kuru-Creutzfeldt-Jakob group of diseases. Transmission experiments are underway at the National Institutes of Health.

A 6-year-old child with meningitis had a respiratory arrest 20 minutes after a lumbar puncture. Thereafter she required maintenance on a ventilator, had a flaccid quadriplegia, and died 12 days later. Necropsy showed infarction of the central portion of the cord at the level of the decussation of the pyramids. The suggested mechanism of damage is compression of the arterial supply to the cord at the level of the foramen magnum by herniated cerebellar tonsils; concomitant hypotension may have contributed to production of the damage. Four similar cases, who survived with residual deficit, have also been reported. Other separate mechanisms by which the cord can be damaged in meningitis are vasculitis, thrombosis and arachnoiditis.

Adrenoleukodystrophy (ALD) is an X-linked degenerative disease characterized by progressive demyelination and adrenal insufficiency. Several phenotypes are described. In post-mortem tissues there is an accumulation of saturated or mono-unsaturated very long chain fattyacids (VLCFA) in the cholesterol ester fraction of adrenal cortex and cerebral white matter. The accumulated fatty acids are unbranched with carbon chain length between 23 and 32 with most containing 25 or 26 carbons. Determination of VLCFA in readily accessible tissues such as skin fibroblasts and plasma allows for reliable detection of patients and carriers.

Charles Dodgson, better known as Lewis Carroll, author of the famous Alice stories, developed migraine and associated visual symptoms late in life. There has been considerable speculation that the bizarre phenomena and weird visual imaginery in Alice stories was directly related to the author’s migraine.

This paper reviews several aspects of the character and health of Lewis Carroll including his shy, introspective personality, his stuttering and his attraction to young girls. It is concluded that there is no connection between the visual symptoms of migraine and the phenomena described in the Alice stories which were written over 25 years before the author developed migraine in his mid-fifties.