Ocular flutter and truncal ataxia may be associated with enterovirus infection.

We report on three patients who presented a rare, uniform clinical syndrome consisting of ocular flutter and truncal ataxia. In all patients the symptoms followed an upper respiratory infection and resolved without sequelae within a few weeks. Previous reports have emphasized the apparent relationship of this entity to infectious disease, but the infectious agent remained uncertain. In one patient we could find a significant rise in antibody titres to enterovirus. We are not aware of any other similar documented case.

Stable xenon CT in patients with chronic cerebrovascular disease.

Xenon-enhanced CT (XeCT) is a method of measuring regional cerebral blood flow (rCBF). Although it is used for acute and perioperative assessment the ability of XeCT to show hypoperfused areas in vivo, as compared with morphological imaging modalities other than noncontrast CT, is not defined. Correlation with MRI helps to define the smallest detectable hypoperfused area. We examined 17 patients 6 weeks after acute cerebral infarcts with CT, XeCT and MRI and the findings were compared. All examinations were performed with the same slice angulation and thickness and the sizes of the abnormal areas were measured. XeCT showed a high correlation with MRI, but less so with conventional CT. Lesions appeared significantly smaller on CT than on XeCT or MRI. No significant difference between MRI and XeCT changes was found. The minimal lesion on MRI correlating with a hypoperfused area on XeCT was 0.8 cm2, whereas lesions measuring less than 0.5 cm2 on MRI could not be detected on XeCT. The good correlation between XeCT and MRI underlines the reliability of rCBF examinations with XeCT. A significant difference between CT and XeCT findings shows the difficulty of interpreting hypoperfused regions only by correlation with CT. Combined XeCT and MRI gives a better estimate of the vascular state of the brain.

Quantification of motor deficit in Parkinson's disease with a motor performance test series.

It was the purpose of the present study to quantify the expected motor deficit in parkinsonian patients with the computer assisted Motor Performance Test Series (MPS), version 05.87 by Schuhfried (1987) and to examine which of the motor test variables found correlate at a significance level of p less than 0.01 with items of "motor examination" recorded at neurological examination and "activities of daily living" of the Unified Parkinson's Disease Rating Scale (UPDRS), version 3.0. 38 patients with idiopathic Parkinson's disease (PD) stages I-IV according to Hoehn and Yahr, aged 41 to 73 years were studied. The study design, i.e. initial rating by the physician followed immediately by testing of motor function with MPS was strictly adhered to in each patient. Physician's rating of rigor and the scores of the semiquantitative tests (finger taps, hand movements and alternating movements) as expression of hypokinesia and the "activities of daily living" correlated with the 3 factors of the Motor Performance Test Series at a highly significant level independent of disease stage. Tremor is only partly and never significantly reflected in the motor data measured. Stages I-II and II-IV (Hoehn and Yahr) differ significantly in the representative data of the Motor Performance Test Series. The results of the study support the assumption that MPS is a valid instrument for quantitative measurement of the motor deficit in parkinsonian patients, but that only some subtests are "pathognomonic".

Impairment of motor planning in patients with Parkinson's disease: evidence from ideomotor apraxia testing.

Compared with a group of age matched controls, patients with Parkinson's disease scored significantly lower in testing for ideomotor apraxia. Imitation of movement sequences was affected more severely than performance of single movements. The degree of impairment was not related to severity of motor disability, but correlated strongly with the results of tests that measured visuospatial and visuoperceptive abilities. It is suggested that defective encoding and central processing of visuospatial information impairs memory for movement which is necessary for correct imitation of movements. Enhanced vulnerability to interference between successively presented items may cause further deterioration of performance in the copying of movement sequences.

[Depression and Parkinson syndrome]. / Depression und Parkinson-Syndrom.

Depressive mood is frequently associated with Parkinson's syndrome, but it may also occur as a precursor of this disease. As regards the subtypes of Parkinson's disease, the frequency of depressive states is significantly higher in the type dominated by akinesia and rigidity than in the type dominated by tremor. On the basis of biochemical changes, certain aspects of the depression can be successfully treated by substitution therapy: L-dopa medication may increase the reduced dopamine values in the striatum, thereby improving drive. Substitution with L-tryptophan raises the lowered serotonin values in the reticular formation, which may influence sleep disturbances. The changes of basic mood, however, which are characteristic of depression, such as cheerlessness and apathy, are the dopamine of antidepressive medication; only these drugs can re-establish the biochemical balance to a large extent.

[Contribution of cerebral angiography to the prognosis of juvenile stroke]. / Der Beitrag des zerebralen Angiogramms zur Prognose beim juvenilen Insult.

46 patients (aged 13 to 40 years) suffering from ischaemic cerebrovascular disease were investigated by means of complete 4-vessel angiography. 40 of these patients were re-examined after a mean follow-up period of 57 months. The aim of the study was to investigate possible connections between the angiographic data and the clinical condition some years later. In the 37 patients who were not subjected to surgical treatment prognosis was not significantly different in cases with, and those without stenoses/occlusions in the craniocervical vessels. However, patients suffering from severe stenoses/occlusions had a significantly worse prognosis than patients with stenoses of a mild degree. The clinical course in patients with stenoses/occlusions localized in the vertebro-basilar system was significantly more favourable than the course of the disease in patients with similar changes in the carotid system. There was a trend towards a worse clinical picture at the end of the follow-up period in cases with degenerative alterations in the small intracranial arteries. The difference in prognostic value of cerebral 4-vessel angiography in cases of stroke in the young, as opposed to the findings in older patients is discussed.

[Long-term prognosis of patients with juvenile infarct. Catamnestic results]. / Langzeit-Prognose von Patienten mit juvenilem Insult. Katamnestische Ergebnisse.

Seventy patients suffering from ischemic cerebrovascular disease had had their first attack before the age of 40 years. Of these patients 57 were re-examined 84 months (mean) after the initial event. Within 48 months, 22 of 51 patients (43.1%) without surgical treatment had recurrent ischemic events (78.6% patients with transient ischemic attacks, 33.3% patients with protracted reversible deficits, 28.6% patients with completed stroke). Of all patients 31.5% had their first recurrent attack during the first year, most of them during the first few months after the initial event. In spite of the considerably higher recurrence rate, patients with initial transient ischemic attacks or protracted reversible deficits had a lower degree of social disability at the end of the follow-up period than patients with an initial completed stroke. In 72.2% of the patients with recurrent events the ischemic focus was found in a vascular area different from the one originally concerned. Patients with arterial hypertension during the follow-up period had a somewhat less-favorable long-term prognosis, but nicotine abuse or relative body weight had no influence on the degree of social disability at the end of the follow-up period. In general, long-term prognosis was rather favorable, 78.4% of the patients being fully capable of work at the end of the follow-up period. Nevertheless, results point to the necessity of complete diagnostic evaluation without delay and introduction of individual therapy for patients suffering from "juvenile stroke."

[Cerebral metastases as the first clinical manifestation of cancer]. / Zerebrale Metastasen als klinische Erstmanifestation eines Karzinoms.

A review of the case histories over the 10-year period 1969 to 1978 revealed 80 patients with cerebral metastases. Group 1 comprised 41 patients (43.9% carcinoma of the breast, 12.2% malignant melanoma, 9.8% hypernephroma, 9.8% carcinoma of the colon) in whom the primary site had been established before the manifestation of neurological deficit. Group 2 comprised 39 patients (56.4% bronchogenic carcinoma, 10.1% hypernephroma, 20.5% primary malignancy not identifiable) in whom the neurological symptoms due to metastasis had been first manifestation of malignancy. The sex distribution, age distribution, site of metastasis, cerebral symptoms and the time-lag before the diagnosis were analysed and compared with the literature. The patients in group 1 had an average survival time of 9 months, as compared with 7 months in group 2, from the time of the initial symptoms of cerebral metastasis. The survival time was not essentially different whether surgical or radiotherapeutic measures were undertaken.

Spinal cavernous haemangioma (intradural-extramedullary) underlying repeated subarachnoid haemorrhage.

Within a period of 13 months a 24-year-old male student experienced four attacks of subarachnoid haemorrhage. An intradural-extramedullary haemangioma at the level of the second thoracic segment was found to be the cause of the repeated bleeding.

[The Steel Richardson Olszewski-syndrome. A report on 3 further cases (author's transl)]. / Progressive supranukleäre Blicklähmung. Klinischer Verlauf von drei weiteren Fällen.

In this report we are describing 3 further cases of progressive supranuclear palsy, all displaying the typical clinical features (first described by Steele, Richardson and Olszewski, 1964): Ophthalmoplegia (affecting chiefly vertical gaze), pseudobulbar palsy, dysarthria, dystonic rigidity of the neck and upper trunk and dementia. Clinical symptoms started between 49 and 51 years of age with slow progression during 2 to 4 years. One patient died 2 years after the first clinical symptoms began. The purpose of this paper is, to describe a further group of 3 cases of progressive supranuclear palsy and to point out in detail the clinical symptoms, that all correspond to supranuclear localisation of this disease and to report about some differences in the development of the disease and the fully developed disorder. The use of treatment with Adamantin and Akineton was not (very) satisfying.

[Lactate acidosis in the cerebrospinal fluid as a prognostic parameter of malacic cerebral insult (author's transl)]. / Laktatazidose im Liquor cerebrospinalis als prognostischer Parameter bei Insultpatienten.

The concentrations of lactate and pyruvate were determined in 111 CSF and blood samples. The CSF and blood chemistry of 43 patients with a lateralized ischaemic cerebral insult was compared with that of a control group of 18 patients. The first tests were carried out within 24 to 48 hours and 50 follow-up determinations were undertaken in the cerebral insult group. The patients were classified according to their level of consciousness (lucid-somnolent-soporous-comatose). Covariance analysis revealed a distinct relation between the CSF chemistry and the level of consciousness of the insult patients; accordingly, a continuous increase in the lactate level from 1.54 mmol/l to 3.48 mmol/l and in the pyruvate level from 0.11 mmol/l to 0.21 mmol/l was noted. Spearman's correlation analysis also pointed to a statistically significant correlation between decreasing levels of consciousness and the CSF lactate/pyruvate quotient. The increase in the CSF/blood quotient of lactate from 1.17 to 2.14, corresponding to the decrease in the level of consciousness, also indicates that during the early stages of the disease, cerebral tissue hypoxia is reflected in the CSF rather than in the blood. Further subdivision of the groups according to whether the patients survived or died, showed that the critical maximum concentrations from the prognostic point of view are 2.74 +/- 0.19 mmol/l in the case of CSF lactate, 0.186 +/- 0.017 in the case of CSF pyruvate and 1.92 +/- 0.65 for the CSF/blood quotient of lactate. None of the patients with a CSF lactate level of 3.1 mmol/l or more survived the ischaemic cerebral insult. Thus, the above-mentioned parameters are to be regarded as important indicators of the threat to the patient's life.

Inflammatory demyelinating polyradiculitis in a patient with multiple sclerosis.

A case of multiple sclerosis (MS) occurred in which there were recent demyelinated plaques in the CNS, and inflammatory demyelination and remyelination in the peripheral (Schwann cell) portions of the spinal roots. The lesions in the peripheral nervous system (PNS) were characterized by inflammation, primary segmental demyelination, myelin stripping, the occurrence of lipid debris-containing macrophages in the endoneurium, and remyelination. To our knowledge, this is the first description of simultaneous acute inflammatory demyelination in the CNS and PNS in MS.

[The cerebral angiogram in cases of stroke in youth (author's transl)]. / Das cerebrale Angiogramm beim "Juvenilen Insult".

Sixty-seven patients (ages: 11-40 years) suffering from ischemic cerebral circulatory disturbances were investigated clinically and angiographically. In 34 cases, transient ischemic attacks or reversible ischemic neurological defects were diagnosed; 33 patients suffered from completed strokes. In most cases the completed stroke took place without previous transient ischemic attacks. A very good tendency toward recovery was observed in 60 of 67 cases. Stenoses or occlusions were found in only 21.5% of the clinically affected vessel regions. However, 50% of those patients, on whom panangiography was performed, were shown to have stenoses or occlusions. Stenotic vessel wall lesions are also obviously to be expected in clinically nonaffected vessel regions; consequently, in cases of cerebral circulatory disturbances, angiographic investigation of all craniocervical vessels is advisable.

[Arteritis in cerebral inflammatory diseases. Diagnostic value of angiography (author's transl)]. / Arteriitiden bei entzündlichen Erkrankungen des Zentralnervensystems. Diagnostische Wertigkeit des Angiogramms.

Cerebral angiography may sometimes show up inflammatory changes in the brain arteries in cases of tuberculous and acute purulent bacterial meningitis. The arteries at the base of the brain are predominantly affected, in some cases together with peripheral branches. Normal angiograms or non-specific alterations may be expected in patients suffering from non-purulent meningitis and encephalitis. Patients with luetic diseases show circumscribed or diffuse vessel wall lesions which cannot be differentiated from alterations caused by arteriosclerosis. The results are presented of 15 patients suffering from inflammatory diseases of the central nervous system of different aetiology. The morphology and the distribution of the arterial changes in the cerebral angiogram are discussed.