[Atypical absence status in adolescence and adulthood].
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Biomedical subjects
Publications and source records attributed to F Aichner.
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We report on two brothers with focal epileptic seizures and agenesia of the corpus callosum. The diagnosis of agenesia of the corpus callosum in both was made by CT, both brothers were retarded and both showed focal phenomena which can be interpreted as a consequence of the lacking connection between the two hemispheres. One of the patients showed a remarkable focal miniature spike and wave periodicity in the EEG. Neither of the parents had matching abnormalities.
A case of primary leptomeningeal melanoma is presented in which the diagnosis was made by ultrastructural demonstration of melanoma cells from the cerebrospinal fluid (CSF) at a time when cranial computed tomography (CT) still gave negative results. Later CT examinations documented the emergence of a tumor mass of the left temporoparietal lobe. This case clearly illustrates the complementary role of these investigational procedures for the diagnosis of cerebrospinal melanoma: leptomeningeal involvement, characterized by two-dimensional diffuse spread of melanoma tissue ("leptomeningeal melanomatosis"), is invisible with CT, but easily recognisable by CSF cytology; in contrast, nodular parenchymal tumor deposits can be readily detected by CT. Identification of pigmented cells recovered from the CSF requires ultrastructural confirmation.
The echoflowscan findings of 121 patients with acute cerebro-vascular diseases were compared with the angiographic features. The results of the degree of carotid stenosis correlated well in 86%. In 14% the ultrasonic findings were false. To be more effective in non-invasive carotid evaluation, a selected battery of tests is required.
This case report presents two types of primitive motor patterns of the oral sense, the rooting reflex in an apallic patient due to encephalitis and a cobra-bite-like motor pattern in a patient with a hypoxic apallic syndrome. In the apallic syndrome the disintegration of cerebral functions to a mesodiencephalic level is a characteristic feature. The reappearance of primitive motor patterns of the oral sense in the apallic syndrome thus gives further evidence for the integration of primitive motor patterns (e.g. rooting reflex) in the brainstem reticular formation. The cobra-bite-like motor pattern combined with aggressive actions might be due to the connection between brainstem functions and limbic structures.
23 cases of supratentorial extracerebral cysts of the Sylvian fissure in children and adult patients are reported. There were 21 male and 2 female patients and the medial age was 15 years (range 2--60 years). The cysts were located in the left middle cranial fossa in 18 cases, in two cases there were bilateral cysts and three patients only had right-sided lesions. In 14 patients the presence of the cyst was uncomplicated. In two of these patients the cyst was detected only incidentally. Four patients had subdural haematomas in addition to the cyst. Two patients had subdural haematomas and were hydrocephalic as well. Three patients had large cysts and hydrocephalus. We did not encounter intracystic bleeding. Operation on the cysts by uncapping them was done in four cases. In all four instances histological examination of the cyst wall revealed that it was composed of arachnoid. Uncapping of the cyst was not followed by its diminution in any of the four patients. It seems that uncapping of such cysts is not a helpful treatment and that surgical treatment in our cases should have been restricted to complications such as subdural haematomas and hydrocephalus. A search for better operative methods for the treatment of large extracerebral supratentorial cysts of the middle cranial fossa should be undertaken.
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Activities in the alpha band are to be encountered in unresponsive patients. A review is given over the different conditions. Patients suffering from pontine lesions mostly are not comatose but locked-in. Alpha activities with intoxications and with comatose states due to transtentorial herniation can be distinguished by the accompanying neurological signs. In severe metabolic coma alpha activities are to be observed as an epileptic phenomenon. Monorhythmical alpha activities with hypoxic coma are not accompanied by slow waves, succeed a burst suppression pattern and are an ominous prognostic sign.
Plasma exchange has been used for therapy in eight patients with the Guillain-Barré syndrome. All patients were severely ill. They became tetraplegic and showed cranial nerve involvement. Five patients received assisted respiration, but the others were also at risk of ventilatory insufficiency. Recovery was abrupt in all cases after the first plasma exchanges. Improvement was more marked when plasmapheresis was done on three successive days with plasma exchanges of 2.0-3.01 each in the initial progressive stage of the disease. A considerable advantage of this therapy is the avoidance of continued artificial respiration and nutrition, which both carry the risk of further complications.
Periodic lateralized discharges (PLDs) were studied in 68 patients. Seventy nine percent had seizures, mostly of the partial motor type, and 62 percent showed signs of acute seizure activity. In the majority of cases, localizing signs were present at neurological examination or in structural tests. Among the etiological causes, vascular accidents, chronic seizure disorders, space occupying lesions, CNS infections and several other disorders could be identified. None of the EEG characteristics were linked to any clinical variables except the mode of appearance of PLDs. Episodically occuring PLDs correlated to chronic seizure disorders. For pathophysiological explanations, a relationship to the clonic phase of a convulsive seizure was stressed.
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Aspergillosis of the central nervous system (CNS) is an uncommon infection, mainly occurring in immunocompromised patients. Beside cerebral involvement spinal cord lesions are extremely rare. To our knowledge, aspergillosis initially presenting with acute paraplegia due to mycotic thrombosis of the anterior spinal artery in a formerly healthy patient has, so far, not been reported. Neither a primary focus nor an underlying disease had been detected.
One hundred and sixty-five EEGs were analysed from 140 patients in postraumatic coma with secondary traumatic midbrain and bulbar syndrome. The EEG-pattern was related to the stage of midbrain of bulbar syndrome caused by supratentorial brain shift. There was a close relation between EEG-pattern and the grade of rostro-caudal impairment. A decrease in the number of different EEG-patterns was associated with increasing intra-cranial pressure. An unfavourable state of coma was indicated by the disappearance of sleep or sleep-like activities, alternating pattern and loss of reactivity. In deep stages of coma the neurological examination reveals no lateralizing signs at all. The EEG gives the only hint to a local cerebral lesion. EEG-abnormalities due to the herniation itself interferred with the EEG-changes due to secondary circulatory, respiratory and metabolic encephalopathies. In such cases the above mentioned regularities were blurred.
Computed tomography and magnetic resonance (MR) studies were done in one case of Behcet disease and showed a focal lesion in the upper brain stem. Early institution of therapy was followed by a complete clinical remission and disappearance of the CT and MR changes. Computed tomography and MR are important modalities for the early detection of Behcet disease when reversibility of the pathological changes is still possible.
The only randomized data on heparin treatment in acute cerebral sinus venous thrombosis (CSVT) are derived from a small number of patients. The rate of intracranial hemorrhages as a complication of high-dose heparin treatment is still unknown. This retrospective study evaluates the clinical features, neuroimaging monitoring and outcome of 42 patients with proven CSVT. Diagnosis was established by DSA, CT, MR tomography and MR angiography. All patients received heparin intravenously guided by doubling the aPTT value for 3 weeks, followed by oral anticoagulation. Partial or complete recanalization was found in 36 cases. 40 patients improved clinically, in 26 of them complete recovery was observed. One patient deteriorated and developed an apallic syndrome, one further patient died of septic multiorgan failure. Only in one patient was hemorrhagic transformation of infarcted brain tissue observed but without clinical deterioration.
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