1/241. Acute hemorrhagic encephalitis (Hurst disease) associated with neuroaxonal dystrophy. Two cases that fulfil the clinical and neuropathological criteria of acute hemorrhagic encephalitis are described. Histological examination revealed additionally focal changes in the white matter characteristic for neuroaxonal dystrophy. The differences in the clinical course and morphological picture observed in both cases are discussed. ( info) |
2/241. Epileptic falling spells after epidural haematoma in adult Down's syndrome. A 35-year-old man with Down's syndrome showed epileptic falling spells. He had suffered from a traumatic right-sided epidural haematoma 3 years before. It had been neurosurgically treated, but MRI taken 5 days later had revealed a small contracoup contusion at the left temporal lobe. His falling spell was a brief tonic seizure without disturbance of consciousness. Background activities of EEG consisted of slow alpha waves interspersed with sporadic theta waves and the amplitude at the left temporal area was lower than the opposite one. Interictal EEG showed sharp waves or sharp and slow wave complexes predominantly at the right temporo-centro-parietal area as well as diffuse, though predominantly at frontal areas, bursts of slow waves with high amplitude. The EEG suggested focal epileptic activities evolving into secondary generalization. SPECT of the brain showed the hypoperfusion at the left temporal area and at the right posterotemporo-parietal area, where the hypoperfusion was somewhat reduced after the improvement of seizures. seizures were well controlled with phenytoin combined with phenobarbital. The incidence of epilepsy in the Down's syndrome has been reported to increase after the middle age in association with the development of Alzheimer's neuropathology. When those people would sustain head injuries, it was necessary to follow carefully using SPECT and EEG. ( info) |
3/241. Deficient platelet-activating factor and related enzymes in hemimegalencephaly. Platelet-activating factor (PAF) may influence neuronal migration, and gyral anomaly in hemimegalencephaly is believed to result from a neuronal disorder. A 7-month-old girl with hemimegalencephaly presented with intractable seizures, for which graded hemispherectomy was performed. In the resected specimen, we could not detect PAF, and related enzyme activities were low. These results suggest a role of PAF in neuronal migration. ( info) |
| PURPOSE: Gas embolism during hysteroscopy is rare but sometimes fatal. A fatal case of gas embolism during diagnostic hysteroscopy using carbon dioxide (CO2) is presented. CLINICAL FEATURES: A 68 yr old woman was admitted for treatment of myoma and cancer of the uterus. hysteroscopy using CO2 was performed without monitoring or anesthesia on the ward. At the end of the examination, just after the hysteroscope was removed, she developed tonic convulsions, lost consciousness, and her pulse was impalpable. Cardiac massage was started, anesthesiologists were called and the trachea was intubated. She was transferred to the intensive care unit with continuous cardiac massage. Cardiac resuscitation was successful. A central venous line was inserted into the right ventricle under echocardiography in an attempt to aspirate gas with the patient in the Trendelenberg position, but the aspiration failed. Positive end expiratory pressure and heparin for emboli, midazolam for brain protection, and catecholamines were administered. Fifteen hours after resuscitation, the pupils were enlarged and she died 25 hr after resuscitation. CONCLUSION: Gas embolism is a rare complication of hysteroscopy. The procedure should be performed with monitoring of blood pressure, heart rate, oxygen saturation and end-tidal CO2 concentration. ( info) |
5/241. Long-term extracorporeal bilirubin elimination: A case report on cascade resin plasmaperfusion. Acute hepatic failure develops as a disease entity of rather diverse origin. With disease progression, toxic bilirubin levels may cause severe complications which include AV-nodal blockage, cardiac arrhythmia, impaired consciousness, generalized seizures, and status epilepticus. Treatment choices to prevent clinical deterioration comprise of costly and limited available orthotopic liver transplantation, utilization of extracorporeal bioartificial liver support devices and haemoperfusion/plasmaperfusion treatment with activated charcoal/anion exchange filters. Here, we present a patient with acute drug-induced cholestatic hepatitis. Excessively elevated bilirubin levels were accompanied by cardiac and cerebral complications. Extracorporeal resin perfusion treatment (Plasorba, BR-350) was successfully performed over a 50-day period without activation of the coagulation system or side effects. bilirubin levels were lowered to a minimum of 225 micromol/l, with concurrent clinical improvement. In conclusion, extracorporeal anion exchange plasmaperfusion may be a viable long-term treatment for hyperbilirubinaemic side effects in overt cholestatic hepatitis. ( info) |
6/241. Solitary olfactory groove schwannoma: case report with review of the literature. A 45-year-old man presented with headaches of 6 months' duration and generalized seizures in a cluster 2 days prior to admission. Examination revealed bilateral papilloedema and no focal deficits. CT scan showed an isodense enhancing mass lesion in the right frontobasal region with perilesional edema. Bifrontal craniotomy (with total excision of the tumor) revealed a tumor in the region of the right olfactory groove attached to the cribriform plate. Histopathological examination disclosed it to be a hyalinised schwannoma. Solitary schwannomas arising from the olfactory groove are extremely rare. Only six other cases have been reported so far. The presentation. CT scan findings and histogenesis of the tumor are discussed along with a review of the literature. ( info) |
7/241. Unusually prolonged survival and childhood-onset epilepsy in a case of alobar holoprosencephaly. Alobar holoprosencephaly is one of the most severe congenital malformations of the central nervous system. Most affected infants are stillborn or have a very short life-span. The survivors can present with neonatal seizures and/or infantile spasms. We report on an unusually long-lived patient with alobar holoprosencephaly and minor facial dysmorphism, who developed generalized epilepsy during childhood. ( info) |
8/241. Primary ciliary dyskinesia syndrome and primary generalised epilepsy. A child with primary ciliary dyskinesia syndrome (PCDS) developed primary generalised epilepsy (PGE). The PGE had characteristics of both childhood absence epilepsy (CAE) and juvenile myoclonic epilepsy (JME). The association of PCDS and PGE has not previously been recorded. ( info) |
9/241. Tiagabine-induced absence status in idiopathic generalized epilepsy. Several medications such as baclofen, amitriptyline and even antiepileptic drugs such as carbamazepine or vigabatrin are known to induce absence status epilepticus in patients with generalized epilepsies. Tiagabine (TGB) is effective in patients with focal epilepsies. However, TGB has also been reported to induce non-convulsive status epilepticus in several patients with focal epilepsies and in one patient with juvenile myoclonic epilepsy. In animal models of generalized epilepsy, TGB induces absence status with 3-5 Hz spike-wave complexes. We describe a 32-year-old patient with absence epilepsy and primary generalized tonic-clonic seizures since 11 years of age, who developed her first absence status epilepticus while treated with 45 mg of TGB daily. Administration of lorazepam and immediate reduction in TGB dosage was followed by complete clinical and electroencephalographic remission. This case demonstrates that TGB can induce typical absence status epilepticus in a patient with primary generalized epilepsy. ( info) |
10/241. Painful tonic/dystonic spasms in sjogren's syndrome. Three patients with sjogren's syndrome are presented in whom frequent tonic/dystonic spasms of the limbs developed during the course of the illness. These patients' clinical findings suggested spinal cord involvement, a localization that was confirmed by magnetic resonance imaging in two patients. In one patient the painful movements responded to treatment with phenytoin and in one other to baclofen. sjogren's syndrome should be considered in the differential diagnosis of conditions that produce tonic/dystonic limb spasms. ( info) |