Computed tomography in Wilson's disease.

In a case of Wilson's disease with flapping tremor, computed tomography demonstrated bilateral low-density areas in the thalamocapsular regions. Computed tomographic findings in Wilson's disease are discussed.

Papilledema in Behçet's syndrome.

Six patients with Behçet's syndrome (five male, one female) had intracranial hypertension diagnosed by elevated CSF pressure in the presence of normal or small ventricles. All six patients had headaches and papilledema. Histories included oral ulcers in 5 patients, genital ulcers in 3, uveitis in 1, arthralgia in 4, and thrombophlebitis in 3. One patient had obstruction of the superior vena cava. A filling defect in the superior sagittal sinus was shown in two patients by carotid angiography, and cerebral circulation time was prolonged in one patient. Papilledema and intracranial hypertension as a result of cerebral vein thrombosis can be initial manifestations of Behçet's syndrome. This has not been a well-recognized feature of the syndrome.

Acute idiopathic demyelinating polyneuropathy: passive transfer to mice by immunoglobulin.

Systemic administration of acute idiopathic demyelinating polyneuropathy (AIDP) immunoglobulins to mice for two weeks resulted in reduced sural nerve action potential amplitudes and reduced (rotarod) motor performance. Electron microscopic examination of the sciatic nerves of the AIDP-immunoglobulin-treated animals revealed loosening of myelin lamellae with widening of interperiod lines and multivesicular disruption of myelin. Vacuolar degeneration was detected in half of the nerves examined by light microscopy. Injection of AIDP-immunoglobulins for three days led to only minor changes, and mice receiving healthy human immunoglobulins showed no abnormalities. These data show that some features of AIDP can be transferred to mice by systemic administration of immunoglobulins and suggest that humoral factors have a pathogenic role in AIDP in addition to cellular factors.

Electrophysiological evidence for activation of NMDA receptors and its antagonism by MK-801 in cerebral ischemia.

We studied the effects of iontophoretically administered MK-801 (50-150 nA) on ischemic changes on the CA1 hippocampal field potential. Twenty rats under urethane anesthesia, of which the hippocampal field response was depressed or lost upon ligation of the carotid arteries, were used. MK-801 applications starting before carotid ligation, decreased the depression of the field response in 8 of 11 trials. MK-801 was applied after the appearance of ischemic changes and partly restored the deteriorated hippocampal field potential in 16 of 34 penetrations. MK-801 was ineffective in preventing or restoring the severely depressed or lost evoked activity. During ischemia a DC potential shift of -32.6 +/- 3.7 mV (n = 10) was recorded. MK-801 reduced the amplitude of the DC potential shift by 50% when applied before (n = 6) or after (n = 4) the initiation of ischemia. Activation of N-methyl-D-aspartate (NMDA) receptors by glutamate or N-methyl-DL-aspartate (NMDLA) induces a slow negative wave on the field response. During ischemia a similar negative wave spontaneously appeared in 9 trials and was also induced with low currents (5-10 nA) of NMDLA which were insufficient to evoke the NMDA-mediated wave before ischemia. These data provide electrophysiological evidence that NMDA receptors are activated during ischemia and MK-801 reduces ischemia neuronal dysfunction.

Whistling epilepsy: a case report.

Whistling, as an ictal symptom, has been previously reported in frontal lobe epilepsy. For the first time in the literature, we present a patient with complex partial seizures of the temporal region associated with this peculiar symptom.

Hot water epilepsy.

The clinical and EEG features of 10 patients with hot water epilepsy were presented. Eight of the 10 cases were male. The mean age of onset was 4.7 years. In four patients partial and in six cases generalized seizures were seen. Three patients also had non-hot water precipitated seizures. Interictal EEGs showed generalized abnormalities in eight and focal abnormalities in two cases. CT was performed and found normal in two patients. Anticonvulsant therapy was instituted for all patients. In six cases with adequate follow-up, complete remission was obtained in four and the frequency of the seizures was decreased in two, with anticonvulsant therapy.

Severe ptosis without ophthalmoplegia due to porphyric neuropathy.

In this case report, we present a patient with severe ptosis without ophthalmoplegia due to porphyric neuropathy. This could be explained only by selective involvement of oculomotor nuclei.

EEG and the linear skull fractures.

The EEGs of 111 children under the age of 16, and 21 adults after a head injury with a linear skull fracture were reviewed. From our results it can be concluded that linear skull fractures do not add any significant abnormality or any variation to what is stated for EEGs of minimal to mild concussions.

The prognosis of subacute sclerosing panencephalitis in adults. Report of 8 cases and review of the literature.

In this article, 8 cases of subacute sclerosing panencephalitis (SSPE) are presented with a review of the literature. The symptoms developed during pregnancy in 2 cases. All of the patients died within 1 year duration except one. According to the reviewed literature, the prognosis in the adult cases is worse than in children; the two sexes are equally affected in adult SSPE.

Thrombosis of intracranial venous sinuses: aetiology, clinical findings and prognosis of 56 patients.

Cerebral venous thrombosis may occur as a complication of infectious and noninfectious processes. In this study 56 patients with angiographically proven cerebral venous thrombosis (CVT) affecting dural sinuses are being reported. Sixty-one percent of the patients were female and 60% were below 30 years of age. Sixty-four percent of the patients had lateral sinus thrombosis and in 26.8% of the cases a septic focus has been found. The diagnosis is established by serial angiography and clinical findings. CVT is not a rare disease while the clinical diagnosis may be difficult because of the variable modes of onset. As the CT findings are found to be non-specific, angiography remains as the best diagnostic tool. Early diagnosis, controlled intracranial pressure, and appropriate antibiotic treatment may reduce the mortality and morbidity rates due to CVT.

Hemidystonia due to porencephalic cyst. A case report.

We present a 28-year old patient with left hemidystonia since early childhood after head trauma. This is the first report of a patient who had hemidystonia due to CT proven porencephalic cyst involving the caudate nucleus.

High dose anticholinergic therapy (biperiden) in dystonia.

We studied the effect of biperiden in the treatment of dystonia in six patients aged 15-30 years. Five patients had generalized and one patient had segmental dystonia. Biperiden was started at a dose of 2 mg/day and was gradually increased to 40 mg/day in a few weeks. All patients had clinically significant response in varying degrees after a mean follow up of 1.9 years. Three patients showed considerable or dramatic benefit. The 40 mg/day was generally well tolerated. High dose anticholinergic therapy is effective in the management of torsion dystonia.

Fibromuscular dysplasia of the basilar artery: a case with brain stem stroke.

Fibromuscular dysplasia of the basilar artery is a rare and usually asymptomatic vascular disease with only 5 cases reported in the literature. A thirty-eight-year-old man who developed ischemic neurologic symptoms of the brain stem due to dysplasia of the basilar artery is presented, and the clinical features, radiology, treatment, and prognosis of this disease are discussed in correlation with the current data.

[Idiopathic localized paroxysmal hyperhidrosis. Treatment with clonidine]. / Hyperhidrose paroxystique localisée idiopathique. Traitement par la clonidine.

Paroxysmal localized hyperhidrosis is a rare sign of a disorder of the autonomic nervous system. It has been reported in association with peripheral nerve, spinal cord and hypothalamic lesions and in some cases the etiology is unidentified. We present 12 cases of idiopathic paroxysmal localized hyperhidrosis and discuss the therapeutic effect and possible mechanism of clonidine which is a specific central nervous system alpha 2--adrenergic receptor agonist.

[Complex partial epileptic seizures as the initial symptom of subacute sclerosing panencephalitis]. / Crises épileptiques partielles complexes, premier symptome d'une panencéphalite subaiguë sclérosante.

A 18 year-old boy, without a previous history, presented with psychomotor seizures and, 4 months after, mental deterioration. EEG showed focal epileptic abnormalities characterized by high amplitude spike waves in the left temporal region. Periodic complexes appeared after administration of Diazepam. CT scan showed mild cortical atrophy in the left temporal region. Measles virus HI antibody titers were 1/2048 in the serum and 1/64 in the CSF. The patient died one year after the first complex partial seizures.

[A case of hemiballismus during cryptococcal meningitis]. / Un cas d'hémiballisme au cours d'une méningite à cryptocoque.

We present a case of hemiballismus related to cryptococcal meningitis. A 23 year-old man was hospitalized because of involuntary movements of his left side, confusion, hyperpyrexia, neck stiffness, bilateral papilledema, right hemiparesis and bilateral pyramidal signs. Diagnosis was made by CSF examination demonstrating cryptococci by india ink. CT with contrast showed hyperdense lesions in the head of the right caudate nucleus, in the left internal capsule and in the frontal and occipital lobes. After treatment with amphotericin B, 5-fluorocytosine and haloperidol, he experienced rapid recovery with disappearance of hemiballismus. To our knowledge, this is the first report of hemiballismus caused by cryptococcal meningitis.

[A case of Cryptococcus neoformans meningitis]. / Bir Cryptococcus neoformans menenjiti vakasi.

In this paper, a fatal Cryptococcus meningitis has been reported. This type of meningitis is quite rare in this country.

[Neurologic complications due to a sample type of rabies vaccination]. / Sample tipi kuduz asisina bagli nörolojik komplikasyonlar.

Neuroparalytic accidents due to sample type rabies vaccination are still an important problem in our country. We present seven patients with ascending polyneuritis, due to rabies vaccine, treated between 1982-1986, and discuss the importance of the problem.

[Two treated cases of botulism]. / Tedavi edilmis botulizm vakasi.

Botulism is a severe neuroparalytic disease caused by the neurotoxins of Clostridium botulinum which exert their effects on peripheral nerve junctions. Guillain-Barre syndrome, Myasthenia Gravis, acute Poliomyelitis and diphtheria must be considered in the differential diagnosis. In this study we have discussed two patients who were treated in our clinic, the differential diagnosis and the role of anti-Cholinesterase drugs in the treatment.