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The authors report the case of a 45-years-old female patient, with progressive hemiatrophy of the left face and tongue. The laboratorial and image studies were normal, including routine blood exams, cerebral-spinal fluid, hepatic function tests, renal and rheumatic; besides X ray, computerized tomography and magnetic resonance of skull. The electroneuromyography showed peripheral neurogenic alterations of motor trigeminal and hypoglossal nerves, markedly on the left side. Those findings are compatible with Romberg's facial hemiatrophy diagnosis, motivating discussion of several aspects of this rare disease according to the account and literature.

A prospective study was designed to follow patients with syndromes of brain stem during 12 months (in 1991-1992). The aim was to correlate clinical and radiologic findings and comparing them with the classical descriptions of brain stem syndromes. Twenty one consecutive patients were admitted at the Hospital de Base do Distrito Federal. The diagnosis and the follow up were carried out by neurological examination and neuroradiologic images: CT (100%), angiography (24%, 5) and MRI (5%, 1). Hypertension and old age were the most important risk factors and hemorrhage at pons with extension to midbrain was the most frequent finding. Coma at admission was associated to a poor outcome. The outcome was defined by the Glasgow Outcome Scale: 28% rated 5, 24% rated 4, 5% rated 3, 28% rated 2 and 14% rated 1. Our results show that the clinical presentation of ischemic strokes and hemorrhages of the brain stem very frequently follow a mixed pattern, which do not conciliate with the classical of these syndromes.

The authors report a case of symptomatic basilar impression and Arnold-Chiari malformation being presented as the first symptom of hemifacial spasm. The surgical treatment of the malformation resulted in improvement of the clinical manifestation with reduction of the hemifacial spasm. The need for the aetiological therapy for the hemifacial spasm is emphasized, before symptomatic treatment with botulinum toxin is tried.

The authors report a case of Raeder's paratrigeminal syndrome and make some comments about the diagnosis: a dissecant aneurysm of internal carotid artery.

The case of a 17-year-old boy who had been well until the age of four when progressive stiffness of lower limbs developed is reported. Soon walking became difficult and on on his thighs, buttocks and calves one could notice the appearance of myokymia. Painful and intermittent spasms were often present specially after physical strain. This was followed by profuse sweating. Strength was normal but muscle relaxing was slow mainly in the quadriceps. The familiar history for neuromuscular pathology was negative. Muscle enzymes, thyroid function tests were within normal limits and muscular biopsy showed no abnormalities. The EMG disclosed a permanent spontaneous activity, more evident in the quadriceps, which was normal in shape, amplitude and duration. In association to this activity multiplets discharges and low motor units were also observed; in interferencial pattern appeared at maximum contraction but the multiplets and low motor units potentials kept on showing even though voluntary muscular activity ceased. The therapeutic essay with carbamazepin had a dramatic effect upon the course of the patient's conditions. He showed a great improvement with this drug and today leads a normal life being able even to play soccer and ride a bicycle. An interesting remark which should be done is the fact that the flexion of the neck inhibits the quadriceps myokymia whereas the extension makes them to appear and even worsen. Unfortunately is was not possible to carry out a more accurate investigation concerning electropharmacology and hystopathology as the patient was uncooperative and refused to undergo such examinations. However the authors believe that this clinical picture can be considered as a case of "continuous muscle-fibre activity" due to the great similarity it bears to the cases related by Isaacs in 1961. An extensive review concerning the pertinent literature has been done.

The study of the ciliospinal reflex in twenty-three patients is reported. The patients were divided into two groups: the first with spinal cord lesions and the second one with brain stem lesions. The authors discuss the pathways and the mechanisms of the ciliospinal reflex in man and emphasize their value on the diagnosis of brain stem lesions.

The authors report a case of bilateral ptosis with miosis, paralysis of upward gaze and of smooth pursuit movements to the right, as well as a slowly regressive state of lassitude. This syndrome followed a haemorrhagic brain stem infarction. After a review of the pathophysiology of ptosis and of vertical eye movements, together with analysis of data provided by computerized tomography studies--since the case was not fatal--explanations are offered for each clinical sign and it is proposed that the lesion, a paramedian, slightly deviated to the right haemorrhagic focus in the midbrain, with a diameter of 15 mm, might have involved several structures, namely the rostral interstitial nucleus of the medial longitudinal fasciculus, the mesencephalic reticular formation, as well as cortico-nuclear and sympathetic pathways.