A case is reported in Montreal of human cysticercosis in a 44-year-old man who emigrated from Italy in 1956. Numerous subcutaneous nodules were found throughout his body. X-ray of his thighs and chest sho wed oblong calcific densities measuring 1 x 0.5 cm in size. Examination of an excised nodule from the right biceps revealed a cysticercus larva morphologically similar to Cysticercus cellulosae although the scolex lacked hooks. On the basis of the intensity of infection, focal and generalized epileptic seizures, changes in the CSF, and well demarcated lucencies observed in brain scan, cerebral cysticercosis was considered the most likely diagnosis.

A new introducer system for subcutaneous placement of shunt tubings is described. The advantages of this system over previous introducers are discussed.

Because of the high concentrations of ɣ-aminobutyric acid (GABA) in the cerebellar cortex and nuclei, an attempt was made to enhance GABAergic transmission in patients with cerebellar disease. Maximum tolerated doses of sodium valproate, a drug which inhibits the degradation of GABA, failed to influence cerebellar deficits in a double blind crossover study on six patients.

Friedreich's Ataxia (F.A.) is a degenerative disease which commonly leads to premature death of cardio-respiratory origin. To explain the early death of these patients, previous investigations have established the existence of I) a cardiomyopathy in nearly 100% of cases, 2) a restrictive pulmonary syndrome of scoliotic origin and 3) a mild hypoxemia associated with slight respiratory alkalosis and a normal oxyhemoglobin dissociation curve. To further assess the cause of early death in patients with such neuromyo-pathy, we evaluated, in eleven FA. patients, the sensitivity of the respiratory centers to hypercapnia, hypoxia, and hyperoxia.

Ventilatory (VE, VT, F, VT/Ti) and occlusion pressure (P0.1) responses were taken as indices of the respiratory centers output during progressive hypercapnia (Read's method) and isocarhic hypoxia (Weil's method). We studied lì Friedreich's Ataxia patients and II age, sex, and arm-span matched controls. The responses of patients to hypercapnia were significantly greater than controls hut their responses to hypoxia were similar to controls.

Our study establishes that the respiratory centers are functioning adequately in early Friedreich's Ataxia and do not contribute to cardio-respiratory insufficiency in such neuromyopathy.

All those related to the medical profession are familiar with the term “resuscitation” which usually means restoration of normal cardio-respira-tory activity in someone who has suffered from the effects of severely impaired functions of these two organs.

The concept of “brain resuscitation” has emerged during the past few years and to the best of our knowledge the term was probably first coined by SAFAR (1977). This term is used more freely by anesthetists. Neuro-surgeons, when they relieve otherwise fatal herniations of the brain, are not in the habit of calling the effort resuscitation. Since, in our opinion, the term brain resuscitation focuses attention on a set of measures in an acute catastrophic situation, we would like to support its use.

A clinical neuro-opthalmo-logical and electro-oculographic study was made on fourteen patients with Friedreich's ataxia. None had evidence of optic nerve dysfunction. No patient complained of oscillopsia although all had ocular motor deficits of varying degrees, which appeared to be related to the severity of the general manifestations of the disease. The defects comprised square wave jerks, jerky pursuit with inability to maintain eccentric gaze resulting in gaze paretic nystagmus and rebound nystagmus. There was failure to suppress by fixation the vestíbulo-ocular reflex. The slow phase velocity of caloric nystagmus was always of reduced velocity. There was inability to augment the slow phase velocity of optokinetic nystagmus with increasing stimulus velocity. Abnormalities of the saccadic system were manifest particularly as hypermetria. These signs in combination are suggestive of disease involving the cere be I lar flocculus and vermis or their brain stem connections. No abnormalities were found in 17 parents or siblings.

An estimate is given of the frequency of pathological changes (assessed by cell populations and from myelin staining) in the brains of 15 cases of Friedreich's ataxia. Mention is made of the special problem of distinguishing primary degenerative lesions from those due to circulatory disturbances arising from the patients cardiac disease.

Electronystagmographic recordings were made of oculomotor and vestibular function in II patients with autósomat recessive spastic ataxia of Charlevoix-Saguenay. All had horizontal gaze nystagmus, marked impairment of smooth ocular pursuit and optokinetic nystagmus, and defective fixation suppression of caloric nystagmus. Many had saccadic dysmetria. but saccade velocity was probably unaffected. Abnormallies pointing to brainstem disturbance were sparse. The findings are thought to indicate mainly diffuse cerebellar disease, with particular involvement of vermis and vestibulo-cerebellum.

Twenty four ataxie patients were investigated with electromyography and nerve conduction studies. They were divided in two groups according to the area they came from, the evolution of the disease, and the clinical signs. Group I patients from the Rimouski area displayed all the clinical and electrophysiological signs of Friedreich's ataxia. Group II comprised patients who presented with a new syndrome known as the autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Although the clinical evolution was better in the latter, there were more electromyographic signs of denervation and the motor conduction velocities were slower. Both groups showed identical and important abnormalities in sensory nerve conduction.

The results of electrophysiological studies in spastic ataxia have not been reported to our knowledge. They underline the place of spastic ataxia as distinct f rom Friedreich's ataxia, spastic paraplegia, and the known familial neuropathies.