Regional quantitative noninvasive assessment of cerebral perfusion and function with N-isopropyl-[123I]p-iodoamphetamine.

Although several reports on the clinical usefulness of N-isopropyl-[123]p-iodoamphetamine (IMP) in the diagnosis of cerebral disease have appeared in the literature, quantitative, noninvasive measurements of regional cerebral blood flow with this method pose difficulties because cerebral IMP uptake not only depends on cerebral perfusion but also on cerebral function. Rather than trying to develop a method to measure cerebral perfusion with IMP, we have chosen to test a method to quantitatively evaluate planar and emission computed tomographic (ECT) studies by comparing the data obtained in patients with established pathology (n = 51, number of scans = 54) with the data obtained in a group of normal individuals (n = 10, number of scans = 11). Using this method, absolute cerebral IMP uptake (counts/pixel/mCi/min) and planar anterior right-left ratios were obtained. Also measured were right-left ratios obtained from 12 paired regions in three ECT slices. In the control group, we found an IMP uptake of 35.6 +/- 4.3 cts/pixel/mCi/min and right-left ratios around 1.00 (s.d. less than 2%). The evaluation of the patients cerebral IMP uptake asymmetries relative to the normal standard values is a useful adjunct to qualitative image analysis in assessing the presence and severity of disease, as qualitative analysis is prone to false-positive and negative results. Cerebral IMP uptake as measured in cts/pixel/mCi/min is abnormal only in severe cerebral disease and therefore generally a less helpful parameter.

Locked-in state in Borrelia burgdorferi meningitis.

The case is reported of a 28-year-old woman with persistent tetraplegia following acute meningitis due to Borrelia burgdorferi infection. The patient developed erythema chronicum migrans before radicular pain occurred in the upper extremities. The poor clinical outcome was suggestive of pontine infarction due to vasculitis of branches of the basilar artery.

Polymyositis, myasthenic syndrome and thymoma in a patient with defective cell-mediated immunity.

We studied a 57-year old woman with severe myasthenic syndrome predominantly proximal. There was no therapeutic effect using cholinesterase inhibitors. Clinical findings, electromyography, whole body scanning and biopsy revealed polymyositis. Thirteen years before the patient was operated of a benign thymoma. The history of the patient showed numerous life threatening episodes of viral and fungal infections. Autoimmune anemia was diagnosed. Investigations of the immune system in vivo and in vitro revealed severe qualitative and quantitative defects in the lymphocyte population spontaneously forming rosettes with sheep red blood cells (SRBC). Thymoma, autoimmune disorder, such as polymyositis and myasthenia gravis, unspecifically elevated antibody titers, multiple severe viral and fungal infections and the defect of the cell-mediated immunity suggest a T-lymphocyte effector- and regulatory dysfunction in this patient.

[Rehabilitation of hemiplegic patients]. / Rehabilitation des Hemiplegikers.

The multifactorial aspects of rehabilitation of hemiplegic patients are reported. Medical and neuropsychological aspects as well as the significance of therapies, such as occupational and language training, physiotherapy and recreation therapy, are discussed. The importance of the role of the family, the architectonic aspects, institutional placement, an early start to rehabilitation and the limits are emphasized.

[Epilepsies from the neurologic viewpoint]. / Epilepsien aus neurologischer Sicht.

A retrospective view of the last 25 years of clinical epileptology in this 100th anniversary year of the Clinic for Epileptics Bethesda, Tschugg, leads to the following conclusions: A generally accepted classification of epilepsies has still not been reached. Several new pathophysiological aspects of epilepsy have emerged. Of special importance are the elucidation of PDS, the application of the Kindling-phenomenon, the Ca-ion influx theory and the analysis of intracerebral metabolic processes in epileptogenesis, the spreading and limitation of convulsions. Seven new diagnostic methods have been introduced over the last 25 years. Five new antiepileptic drugs have become available over the past quarter of a century. During this period 30 other drugs were withdrawn from clinical use. The most significant advance in the treatment of epilepsy in recent years has been the determination of anticonvulsant concentrations in body liquids. A deeper understanding of the pharmacokinetics of anticonvulsants has led to increased competence in recognizing drug interaction, in treating pregnant epileptics and in the concomitant use of other medications. Monotherapy is generally preferred to combination regimes. Slow-release preparations and calcium-influx blockers have reached the phase of clinical trials. Further advances have been attained in the pre-operative preparation and the operative therapy of drug-resistant epilepsies.

[Cranial polyradiculitis or the so-called Fisher syndrome]. / Polyradiculitis cranialis oder das sogenannte Fisher-Syndrom

In the light of 3 personally observed cases a description is given of polyradiculitis cranialis or socalled Fisher's syndrome, a separate form of Guillain-Barre-Strohl polyradiculitis. It is a combination of acute ataxia, areflexia and ophthalmoplegia. The cause is probably an immunological reaction to a harmless infectious disease. Reversible or limited demyelination is assumed. The demyelination occurs particularly in the peripheral nerves and their spinal roots, but also in the spinal-cerebellar pathways and possibly in the nuclei of the cranial or optic nerves. Diagnosis is based on detection of "albumino-cytologic dissociation" in the cerebrospinal fluid and the finding of a lesion of the peripheral motor neuron in the EMG. Special drug therapy is unnecessary. The prognosis is good: complete remission is usually observed.

[Polyneuropathy and epileptic seizures in a family with acute intermittent porphyria]. / Polyneuropathie und epileptische Anfälle bei einer Sippe mit akuter intermittierender Porphyrie.

Report on clinical and electrophysiological findings in four members of a family with acute intermittent porphyria in the remission period. One patient had suffered from repeated epileptic seizures of the grand-mal type since the age of 24 years. Generalized and multifocal epileptic potentials were found in her EEG. Two other members of the family, a man and a woman, were found electromyographically and neurographically to have a florid neuropathy with damage to the axon and the myelin sheath. Only the female patient showed manifest clinical signs of the polyneuropathy. The 4th member, who years previously had had abdominal colics and suspect biochemical signs of acute intermettent porphyria, was not striking either neurologically or in electromyographic and neurographic polyneuropathy screening at the time of the examination. Epileptic seizures and the symptoms of the polyneuropathy had a close connection with the menstrual cycle (two cases). Before and at the beginning of the menstruation a deterioration of the disease was observed. Problems of antiepileptic therapy in acute intermittent porphyria are discussed.

[Centrally caused achromatopsia]. / Zentral bedingte Achromatopsie.

A 65-year-old patient sustained a transient left-sided sensomotoric monoparesis with homonymous hemianopsia. One day later there was an acute deterioration in the patient's vision and he developed achromatopsia, prosopagnosia, disturbance of spatial orientation and a homonymous upper hemianopsia. Six months later the findings are about the same. The CT shows a bioccipital lesion with left-sided accentuation, extending dorsocaudally. The origin of the cerebral infarct is presumably a vertebrobasilar insufficiency due to congestive cardiomyopathy and atrial fibrillation.

[Etiology and clinic of status epilepticus]. / Zur Atiologie und Klinik des Status epilepticus

In the presented study we examined 110 patients who suffered from status epilepticus. Total 148 status were analysed with regard to etiology, releasing factors, course and treatment. Causes stressed were head injury with brain damage and intracranial space occupying, especially in the frontal region. Head injury with opened dura mater showed more tendency for development of status than closed ones. In a quarter of all cases origin couldn't be found. All these, with exception of one case, developed intercurrently. Isolated or initial always was consequence of brain lesion, the origin of which was or has been detected. No trend with reference to distribution of age in the particular forms of status were found out. In a third part of all a releasing factor could be discovered. Most the question in these cases was failure in medicamental treatment. We couldn't draw one's interferences about pathogenesis from etiology, age of patient, place in course and form of status. About in a quarter of all status we found defective neurological sequels, in 12% also psychical disturbances. 16 patients died during the period of observation, but only 2 within 24 hours, all together 8 in the first 14 days in deap coma. Immediately cause of death were above all complications of the heart, circulation of blood and respiration tract. In the most cases the treatment consisted in a mixture of anticonvulsive medicaments. The predominant rate of status epilepticus could be inhibited within 24 hours, only 20 lasted a longer time.

[Chorea minor under ovulation inhibitors]. / Chorea minor unter Ovulationshemmern

Three weeks after commencing oral contraception for the first time, a 26-year-old woman with a history of rheumatic carditis and chorea minor presented with sudden recurrence of hyperkinesis. Withdrawal of the contraceptive agent was followed by rapid remission of the hyperkinesis, which suggests a casual relationship between the contraceptive and this condition and an analogy between this case and so-called chorea gravidarum. A young non-pregnant woman presenting with chorea minor should be specifically questioned by ingestion of oral contraceptives. If there is a past history of rheumatic fever with or without chorea, oral contraceptives are contraindicated.

PIP: 3 weeks after commencing oral contraception (Ovulen) for the 1st time, a 26-year-old woman with a history of rheumatic carditis and chorea minor presented with sudden recurrence of hyperkinesis. Withdrawal of the contraceptive agent was followed by rapid remission of the hyperkinesis, which suggests a causal relationship between the contraceptive and this condition and an analogy between this case and so-called chorea gravidarum. A young nonpregnant woman presenting with chorea minor should be specifically questioned about ingestion of oral contraceptives. If there is a past history of rheumatic fever with or without chorea, oral contraceptives are contraindicated.