The few existing neuropathological, neurochemical, and neuropharacological studies have shed little light on the pathophysiology of spasmodic torticollis (ST). The relevance of experimental ST in animals and drug-induced ST in man to idiopathic ST is unclear.

Most pharmacotherapeutic endeavors have focused on drugs affecting basal ganglia function. Unfortunately, problems of sample size, clinical heterogeneity of patient population, research design, objective evaluation of response, documentation of key data, and adequacy of duration of follow-up make interpretation of published results difficult. Because of the heterogeneity of ST, investigations aimed at establishing a neurotransmitter profile for each patient by observing the acute response to a test dose of drugs affecting cholinergic, dopaminergic, serotonergic, and gammaaminobutyric acid systems may provide a more rational basis to the selection of treatment.

This retrospective study concerning 102 patients was undertaken to study the therapeutic decisions in these patients when they presented with symptoms of cerebral vascular insufficiency, mostly from the territory of the carotid artery, either transient or accompanied by a deficit. The current literature on the subject is also mentioned. The clinical outcome with regard to the quality of life, seems to have been better in patients who were subjected to carotid endar terectomies than in those who received medical treatment.

Study of 100 children with grand mal convulsive disorders and 100 medically healthy children of matching age showed significantly lower plasma levels of vitamin E in the former (means 632.2 ± 17.3 and 822.5 ± 2\.Zßg/dl respectively; p < 0.001). This finding accords with the ability to prevent seizures in rodents by giving a-tocopherol before exposing them to a convulsion-inducing environment.

Baclofen was used in a double-blind crossover placebo-controlled trial to treat spasticity in patients with multiple sclerosis (MS). While on Baclofen, patients obtained a significant (p<0.001) reduction in spasticity compared to controls. The drug was particularly effective in alleviating flexor and extensors spasms, as well as their associated pain. Side effects were common in this study, but were usually well tolerated by the patients. The commonest side effects were sedation, nausea and vomiting. There were no changes in hepatic, renal, or hematological function in any patients. Increased weakness due to loss of spasticity for support was also a fairly common complaint. The drug seems best indicated in patients in whom spasticity is not required for support or other activities of daily living. Careful monitoring of the patient is essential for effective use of this drug.

In reinnervatedskin transferred from the foot to the hand, sensory thresholds approach normal values — an observation not predicted by the theoretical relationship between innervation density and tactile acuity. Therefore, we suggest that innervation density is not the major factor determining tactile acuity. Rather, the hand region of the central somesthelic map may be specialized to provide a calibre of function unavailable to other regions.

Polysomes extracted from cultured fibroblast cells isolated from patients with Duchenne muscular dystrophy (DMD), carriers of the disease, and normal controls were used for in vitro measurement of protein synthesis in a wheat germ extract system. It was observed that polysomes from patients and carriers (seven of each aged 17 years or older) exhibited a 3-fold and a 1.5-fold decrease in the rate of protein synthesis, respectively, as compared with controls. These results are discussed with a view to developing a sensitive and easily available assay for the detection of DMD carriers.

This case report describes a melanin-containing neurofibroma involving a spinal nerve root. Electron microscopy of the tumor shows that neoplastic Schwann cells are capable of melanogene-sis. Although this capability is suggested in the literature, few reports provide ultra-structural confirmation.

The likely identity between reported “cellular blue nevi of spinal nerve roots” and nerve sheath tumors is discussed. It is possible that pigmented nerve sheath tumors behave more aggressively than nonpigmented ones, although it is debatable.

The results are reported of single muscle fiber uptake of tritiated leucine in muscle biopsy material from Duchenne dystrophy and Charcot-Marie Tooth disease. The uptake in the two conditions is compared and suggests that the previously reported increase in synthesis of cytoplasmic proteins in muscular dystrophy are probably related to regenerative efforts by the muscle fibers.

The history of a patient with painful tonic seizures in association with multiple sclerosis is related. These seizures have been thought by most authors to be spinal in origin. This case is reported because of the localization of a supratentorial lesion, clinically and on CT scan.

The aim of medical management of a patient with a recently ruptured intracranial aneurysm is to preserve residual brain function and prevent systemic complications. Surgery should be performed as soon as the patient is in good neurological condition. Most fatalities result from the destructive effects of the initial hemorrhage, but delayed ischemic neurologic deficit can result from vasospasm and rebleeding. Systemic complications of the brain damaged state result in a smaller proportion of deaths.

Common medical problems are reviewed as well as their medical management. In particular, some special problems related to subarachnoid hemorrhage such as cerebral edema and herniations, rebleeding, and vasospasm are also considered. Major recent advances have been the introduction of antifibrinolytic therapy, the realization of the importance of maintaining blood volume and pressure, as well as general advances in respiratory care.

HLA-A, B, and C typing was carried out on five families with classical Friedreich's Ataxia (F.A.). Segregation patterns in a total of 16 offspring. 10 with the disease and 6 without, indicated no association between F.A. and HLA.

Using radioimmunoassay, the levels of plasma cortisol of 23 patients with multiple sclerosis and 10 control subjects were determined at three or more times over a one-year period. The status of the disease was ascertained by neurological examination at the same time. Twelve of the patients were considered stable because they showed no changes in their disease status during the year. The remaining 11 patients were designated active since each had one or more relapses. The levels of cortisol of the control and stable groups remained relatively constant during the one-year period, but the levels of the active group fluctuated significantly more than either of the other two groups. Furthermore the active patients who had more than one episode had the greatest fluctuation in their cortisol levels. The fluctuations could indicate some endocrinologie al aberration in patients with active multiple sclerosis or may be secondary to the influence of the disease.

This is a report of a giant cystic craniopharyngioma which escaped diagnosis for nine years in spite of seemingly thorough neuroradiological investigation, prior to the advent of the CT scan.

A study of 17 parents (obligate hétérozygotes) of children with Friedreich's ataxia was carried out. In addition to medical histories and physical examinations, a standard 12 lead ECG tracing was obtained. In the age group below 50, there was no significant evidence of ischaemic or primary cardiomyopathy. Older subjects had more frequent risk factors for arteriosclerotic heart disease.

In vitro experiments using the equilibrium dialysis technique were performed to determine the binding of valproic acid to plasma components in the absence and presence of therapeutic concentrations of phenytoin. The free fraction of valproic acid was found to be dependent on the total valproic acid concentration. Phenytoin did not influence valproic acid protein binding.

The evolution of 15patients initially evaluated during Phase One of the Quebec Cooperative Study of Friedreich's ataxia has been studied approximately three years later. It is concluded that the deterioration of cardio-pulmonary function in Friedreich's ataxia is multi-factorial. The neuromyopathy (or the underlying metabolic or cellular defect) appears to be the main contributing factor to the deterioration of cardio-pulmonary function, which is exacerbated by the scoliosis and varying severity of the cardiomyopathy.

A case of carnitine palmityl transferase deficiency in skeletal muscle is described. The usual symptoms associated with this disease (recurrent muscle cramps or pain and pigmenturia) were not observed but sudden exposure to cold precipitate rhabdomyolysis in this patient.

Clinical and pathological findings in two cases of degenerative progressive myoclonic epilepsy (PME) are described. The clinically difficult task of differentiating a “cerebellar” tremor from an action myoclonus is emphasized. Simultaneous elect roencephalography and electrokymography was done, using capacity to ground transients for recording hand movements. This method was found useful in corroborating the cerebellar nature of the remaining disorder, after successful treatment of the myoclonic element with anticonvulsants.