Using a method of a gradual increase of bromocriptine with a concomitant reduction of Sinemet® (levodopa 250 mg + carhidopa 25 mg), 19 patients with advanced Parkinson’s disease have been treated for periods of up to 22 months and 16 of them have shown improvements of varying degrees. Eighteen patients were able to tolerate bromocriptine addition, with early transient adverse effects occurring in seven cases. In contrast to several previously reported studies, it was found necessary to withdraw bromocriptine in only one case.

With the drugs currently available, bromocriptine has a role in the management of patients with advanced Parkinson’s disease. The method described here may allow a greater number of patients to be given a trial with this drug.

Four cases are described illustrating the clinical features of positional vertigo and nystagmus due to posterior fossa tumors and a case of obstructive hydrocephalus. In these cases positional vertigo was the first and only presenting symptom of central nervous system disease. One case of subependymoma of the fourth ventricle and one with hydrocephalus had characteristic symptoms of benign positional vertigo; each showed positional nystagmus of the benign paroxysmal type.

A questionnaire survey has been made of the life effects of narcolepsy in 180 patients, 60 each from North American, Asian and European populations, with 180 similarly distributed age and sex matched controls. Life-effects were attributed by the patients to the primary symptoms of excessive daytime drowsiness, sleep attacks, cataplexy, vivid hypnagogic hallucinations and sleep paralysis, and also to other frequent symptoms such as visual problems (blurring, diplopia) and memory impairment. Occupational problems were prevalent (over 75%) and included statistically significant deleterious effects upon performance, promotion, earning capacity, fear of or actual job loss and increased disability insurance. Driving was greatly affected and patients fell asleep at the wheel more frequently (66%), had near or actual accidents from drowsiness or falling asleep at the wheel (67%), and could experience cataplexy (29%) or sleep paralysis (12%) while driving. Work or home accidents attributed to sleepiness or sleep (49%) or related to smoking (49%) were much more common in patients. There were also deleterious effects on education, recreation and personality related to the disease. Narcolepsy can produce a variety of life-effects probably more serious and pervasive than, for instance, those of epilepsy, therefore emphasizing the importance of early diagnosis and treatment.

Regional cerebral blood flow (rCBF) during internal carotid artery (ICA) occlusion for endarterectomy can be measured without inconvenience using the probe holder illustrated.

When mean ipsilateral hemispheric CBF exceeds 20 ml/100 gm/min, an intraluminal bypass is not necessary (63% of patients), except in patients with extensive cerebrovascular disease in whom rCBF should also exceed 20 ml/100 gm/min in all areas. ICA “stump” pressure is falsely high in about 20% of patients, and is therefore not a dependable criterion for selecting patients who need shunting.

While intraoperative shunting is capable of restoring pre-occlusion CBF levels, it does not eliminate the risk of intraoperative ischemic neurological deficit of probable embolic origin.

The effect of L-tryptophan, a precursor of serotonin, and placebo were studied in eight patients with spasmodic torticollis. L-Tryptophan (5gpo as a single dose) which increased free plasma tryptophan 20-53 fold improved only one out of six patients. Two out of three patients, including the subject who improved following an oral load of tryptophan, improved with L-tryptophan combined with nicotinamide, a tryptophan pyrrolase inhibitor, when administered for 1-3 weeks. However, the magnitude of clinical improvement was not impressive. Our findings suggest that impairment of serotonergic function is not a general finding in spasmodic torticollis though it may play a minor role in the manifestation of this movement disorder in some patients. The present study emphasizes some of the difficulties in evaluating therapeutic response, namely, the intrinsic variability of the disorder, the response to placébo in some subjects and the limitations of methods for measuring change.

Visual function is usually considered to be normal in papilledema. We report previously undescribed abnormalities in the electroretinograms (ERG) and visual evoked potentials (VEP) of two patients with chronic papilledema. The steady-state latencies of the ERG and VEP were calculated using time-difference analysis. The ERG was abnormal under both scotopic (slow) and photopic (fast) flicker conditions, but the VEP was abnormal only to fast flicker photopic stimulation. This may indicate a relative vulnerability of a high frequency signal system under conditions of raised intracranial pressure. The abnormalities resolved rapidly once the intracranial pressure had been normalised. The cause of the V EP and particularly the ERG abnormalities remains unknown.

One hundred multiple sclerosis (MS) patients and hospital controls were compared for coexisting illnesses, and other illnesses among their family members. The patients and controls were compared for allergy, migraine, and cancer among themselves and for a family history of these illnesses. The findings were negative. However a relationship was found between multiple sclerosis and diabetes mellitus. More MS patients than controls were either diabetic or reported a blood relative with diabetes. MS patients who reported another blood relative with MS were also more likely to report a family history of diabetes mellitus than MS patients who had no other relative with multiple sclerosis.

Renal function studies were performed in seventeen patients, under metabolic ward conditions, before the initiation of therapy with levodopa. These studies were repeated during the first two to three weeks of treatment and, again, after one to two years of chronic therapy. There were no significant differences between the results of pre- and post-therapy studies, except that the blood urea nitrogen was slightly, but significantly, elevated in the nine patients who had been on the drug for one to two years. During the early weeks of treatment, there was an insignificant trend towards hypotension and increased excretion of sodium. This did not persist in those patients followed for one to two years after the initiation of treatment. Glomerular filtration rate, as measured by an endogenous method, was unchanged by chronic therapy with levodopa. These results are in contrast to the acutely increased glomerular filtration rate, as measured by an exogenous method, and the increased sodium excretion following a single dose of levodopa or dopamine.

This case was presented as an interesting example of a global arteriovenous malformation (AVM) involving muscle, bone, extradural and intradural spaces of the cervical region.

A twenty-eight year old male in October, 1976, fell at work sustaining a blow to his lower back. The following day he complained of cervical pain. Physical examination was negative and he had a full range of movement of his cervical spine.

Plain films and tomograms of the cervical spine showed a lytic honeycomblesion of C2 and C3, and a 4 mm. subluxation of C3 on C4 (Fig. 1). The bone scan showed increased flow and decreased static activity in the upper cervical region. The radiological diagnosis was vertebral hemangioma; tumor, infection and histiocytosis were unlikely but important differential considerations.

An experiment was conducted to evaluate the effect of 1-Hz or direct current brain stimulation on kindling behavior induced by 60-Hz sine wave stimulation. The effective threshold intensity to elicit a convulsion was determined on four separate occasions with 5 days of daily trials between determinations. On each day one group of experimental rats was stimulated with 1-Hz sine wave current before and after stimulation with 60-Hz sine wave current (1-60-1 group). Another group received direct current stimulation and 60-Hz current (D-60-D group). A third group received only 60-Hz stimulation. Suppression of kindling behavior usually induced by the 60-Hz stimulation occurred with 1-Hz stimulation; the mean threshold value increased on each successive determination. Suppression was most pronounced for the direct current group; it appeared after a single trial and persisted for 32 days after the last threshold determination. In contrast, most of the rats in the 1-60-1 group had recovered from the suppression after the 32 day period of nonstimulation. A second phase of the experiment indicated that the increase in threshold values for the D-60-D group occurred after a single DC stimulation. These results are consistent with the hypothesis generated by previous research that suppression following 1-Hz stimulation is not due to tissue damage.

An 8 year old boy developed cerebral abscess and purulent meningitis due to Clostridium perfringens and Clostridium paraputrificum after trauma to the left orbit. The patient made a satisfactory recovery with surgical treatment and antibiotic therapy. A review of the literature revealed that meningitis due to Clostridia is rare and usually related to penetrating trauma, and that penetrating trauma to the orbit is associated with significant central nervous system morbidity.

Antibodies directed against glial cells may be involved in autoimmunity in multiple sclerosis (MS). Using a tissue culture system, the presence of glial cell antibodies in MS-patient serum was detected through immunofluorescent technique. Thirty one of 73 MS-sera were strongly positive for anti-glial cells, 13 were equivocal and 29 were negative. The antibody staining was either cytoplasmic or associated with cell surface membrane, and involved IgG type of antibody.

The natural course of the pathologic features in striated muscle was studied in a full-term infant with myotubular myopathy. At 5 days of age a muscle biopsy revealed that more than 90 percent of muscle fibers fulfilled histologic, histochemical and electron microscopic criteria of fetal myotubes. The infant died unexpectedly at 9 months of age from spontaneous rupture of a multifocal cavernous hemangioma of the liver. Postmortem examination revealed that progressive maturation of the fetal muscle had not occurred postnatally, and more than 90 percent of myofibers were still apparent myotubes. This maturational arrest was generalized to all striated muscles. The only changes detected since the neonatal period were hypertrophy of the small population of large fibers, but with minor cytoarchitectural alterations, and loss of the incomplete histochemical differentiation with ATPase stains or dedifferentiation not attributed to postmortem diffusion. Involvement of the gubernaculum testis accounted for the undescended testicles. The brain and spinal cord appeared normal. Evidence of degenerating and regenerating axons in the sciatic nerve suggested that the etiology of this maturational arrest of fetal muscle may be neurogenic.

A 52 year old female with polymyositis presenting with distal and asymmetrical weakness is presented. Similar reported cases are reviewed. These patients respond favorably to steroids. Increased awareness of this unusual distal polymyositis should avoid delay in diagnosis.

A case of Fisher’s variant of Guillain Barré Syndrome is presented. Treatment with exchange transfusion coincided with clinical improvement. This is the first case of Fisher’s variant treated in this manner.

Three cases of suhacutely progressive spinal paraparesis had myélographie evidence of spinal cord swelling suggesling the diagnosis of intramedullary tumour. The diagnosis of multiple sclerosis (MS) was made in each case with the aid of CSF protein electrophoresis, evoked responses and CT scan. Because acute MS may produce cord swelling simulating tumor or syrinx, these additional investigations may he necessary to make a correct diagnosis.

A patient presenting with palatal myoclonus was diagnosed as suffering from possible multiple sclerosis. A reduction of cerebrospinal 5-hydroxy-indole-acetic acid suggested a decreased central serotonergic turnover. A therapeutic trial with clonazepam, a serotonergic agonist, improved the myoclonus symptomatically. The possible involvement of serotonergic inhibitory systems in the expression of palatal myoclonus is discussed.

Giant-axonal neuropathy (GAN), a chronic peripheral neuropathy with associated Central Nervous System dysfunction and tight curly hair, is described in a 17-year-old girl. Biopsies of this girl’s muscle and nerve are characteristic of this condition. Her clinical course over a 12 year period characterizes a disease of a slowly progressive nature.

The anticonvulsant activity of the ethyl esters of the major valproic acid metabolites was assessed against minimal pentylenetetrazol seizures in adult male ICR mice. The ethyl ester 3-hydroxy-propylpentanoic acid was found to possess significant anticonvulsant activity.