[Sneddon syndrome. Report of 3 cases]. / Síndrome de Sneddon. Relato de três casos.

The Sneddon's syndrome consists of neurologic manifestations associated to the presence of livedo reticularis and cyanosis of the extremities. The pathological process is an endothelial obliteration of arterioles, leading to a reticular appearance of the skin, despite the environment temperature. The authors present three new cases, caucasian males with 7, 16 and 54 years of age. The youngest started with hemilateralized motor seizures and showed a porencefalic area in the CT scan. The oldest had livedo reticularis, acrocyanosis and started with hemilateralized motor seizures, and a hemiparesis as sequela; CT scan with parasagittal infarct and occlusion presented of one anterior cerebral artery on angiography. The third patient started with hemifacial seizures, developed a labioglossolaringeal paresis and dysarthria as sequela; CT scan and MRI showed multiple infarcts, with multiple occlusions of cortical branches on angiography. The skin biopsies showed endothelial vascular hyperplasia in all cases. Only one (54 years old) patient had a positive IgG antiphospholipid antibodies. The Sneddon's syndrome seems not to be so rare and have to be considered in the etiological investigation of cerebral infarcts, mainly in young people.

[Temporal lobectomy in cerebral infarction with mass effect]. / Lobectomia temporal no infarto cerebral com efeito de massa.

Cerebral edema secondary to ischemia can threaten life, mainly due to frequent failure of medical management. Imminent herniation of the temporal lobe can be timely detected by clinical signs and CT-scan. Eight patients (4M, 4F; 48-74 years, mean 62) with ischemic stroke and imminent herniation, were surgically decompressed by a standard temporal lobectomy as described by Olivier for temporal lobe epilepsy. Six patients survived, two died and were considered failure of the procedure, probably due to late surgical indication. In conclusion, temporal lobectomy is life-saving for patients with large ischemic cerebral infarction with mass effect and deteriorating signs of brain stem compression.

[Intracranial dissecting aneurysms of the posterior circulation: report of 3 cases and review of the literature]. / Aneurismas dissecantes intracranianos da circulação posterior. Relato de três casos e revisão da literatura.

Dissecting aneurysms of cerebral arteries are unusual causes of stroke. The carotid system is the commonest site of this pathology, the vertebral arteries are less involved and dissection of the basilar artery is rare. The authors report three cases of arterial dissection of the vertebrobasilar system, two of the vertebral arteries and one of the basilar artery. An extensive review of the literature is presented. The clinical picture of dissection of vertebrobasilar system was inespecific but pain was a prominent symptom, though had not occurred in the site of the arteries involved. The pain was suggestive of subarachnoid hemorrhage. Associated or risk factors were mild trauma, migraine and high blood pressure. The angiographic findings were suggestive, however just the "double lumen" has been considered pathognomonic. The prognosis is variable. It was benign in case 3, left sequela in case 2, and case 1 rebleed fatally.

[Periodic lateralized epileptiform discharges in neurocysticercosis]. / Descargas epileptiformes periódicas lateralizadas em neurocisticercose.

A case of a girl (10 years old) with a psycho-organic syndrome plus headache without signs of clear intracranial hypertension is related. The diagnosis of neurocysticercosis was made by CSF abnormalities. PLEDs over the left cerebral hemisphere were recorded at the first day after treatment with praziquantel (50mg/kg/day during 21 days). The first CT scan was normal and just after the treatment showed a prominent left cerebral hemisphere involvement, despite parenchymatous dissemination of cysticerci was bilateral. The treatment was successful and the patient became asymptomatic in the two years follow-up. The neurophysiologic aspects of this EEG abnormality is also discussed.

[Clinical treatment of resistant epilepsy]. / Tratamento clínico da epilepsia resistente.

The authors analysed 34 cases of resistant epilepsy (20 males and 14 females, mean age 23 years), treated clinically between February/1984 and May/1986. The patients underwent neurological, neuropsychological, psychological, psychiatric, cerebrospinal fluid, electroencephalographic, tomographic and/or angiographic examination. Most of the patients had complex partial seizures. The etiology was unknown in 19 patients (55.8%), probable neurocysticercosis in 6, perinatal hypoxia in 5, delivery trauma in 3 and probable sequelae of encephalitis in 2 patients. There was a clear past history of infantile febrile convulsion in 2 patients. Most patients received carbamazepine (mean dose 24.5 mg/kg/day), phenytoin (5 mg/kg and valproic acid (28 mg/kg) as monotherapy or in association. Twenty-two patients (64.7%) had more than 80% decrease of the seizure frequency. Nine resistant epilepsy cases (24.5%) were evaluated as candidates for surgical therapy. The authors concluded that the resistant epilepsy is best managed by a specialised, multidisciplinary team, and pointed out the need of a correct diagnosis of the seizure type, an adequate drug therapy and a good engagement of the patient and his family in the treatment.

[Peripheral polyneuropathy induced by benzonidazole in the treatment of Chagas' disease]. / Polineuropatia periférica induzida por benzonidazol no tratamento da doença de Chagas.

Neurophysiological examination before and after the administration of benzonidazole, has shown peripheral polyneuropathy induced by this drug in most of the patients treated for chronic Chagas' disease. The polyneuropathy was mostly axonal and it was dose dependent being more severe in patients who had denervation of skeletal muscles before receiving the drug.

Haemophilic neuromyopathy.

Twenty haemophilic patients were examined neurologically and found to have varying degrees of muscle atrophy. The pathogenesis of the atrophy was investigated by electromyography, serum muscle enzyme levels, and muscle biopsies. In most cases the EMG showed such abnormalities as reduced numbers of functioning motor units and "myopathic" motor unit potentials. Serum enzyme levels were frequently raised, and type 2 fibre atrophy was found in the three patients who underwent muscle biopsy. These findings suggest the presence of neuromyopathic involvement in haemophilic patients.