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.

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.

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.

[Cerebral circulation (measured by isotopes) and acid-base equilibrium of the cerebrospinal fluid during migraine]. / Circolazione cerebrale (misurazioni isotopiche) ed equilibrio acidobasico del liquido cefalo-rachidiano durante l'emicrania

In migraine patients a considerable metabolic disturbance characterised by a decrease in bicarbonate and an increase in lactate was demonstrated during the migrainous state. Measurements of the cerebral circulation were taken by means of radioactive isotope and showed a distinct decrease in blood supply to the entire brain during the migraine attack. Following forced hyperventilation during the attack-free interval a pathological reactivity with decreased blood flow was found in the hemisphere which had been indicated as the usual site of the migrainous pain.

[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.

[Acute intermittent porphyria. A case report and family study]. / Akut intermittierende Porphyrie. Kasuistik und Familienuntersuchung

The course of acute intermittent porphyria is described in a patient who died during an acute exacerbation of the disease. An analysis of the urinary porphyrin precursors (delta-aminolaevulinic acid and porphobilinogen), the determination of toal porphyrin excretion and the separation of haem precursors in the urine according to the number of carboxylic groups demonstrate different degrees of biochemical severity in the individual consanuinious members of this patient's family. The detection of latent carriers is of particular importance since the avoidance of porphyrogenic substances is the most important prophylactic measure to be undertaken in all latent clinical cases. According to our experience, however, prophylactic measures must also be extended to young consanguineous family members with negative excretion patterns in view of the difference in age at which the disease manifests itself.

[Organic dementia as a first symptom of infantile Huntington's chorea (author's transl)]. / Organische Demenz als erstes Symptom einer kindlichen Chorea Huntington

A loss of intellectual capacity at the age of 10 was the first symptom in a case of infantile Huntington's chorea. Subsequently a rigid hypokinetic syndrome occurred only at the age of 20. The dementia syndrome was confirmed in psychological tests. Auxiliary findings such as a diffuse abnornal EEG, as well as a dilatation of the lateral ventricles and an extension of the subarachnoid spaces in the PEG corresponded to a positive diagnosis of Huntington's chorea.

[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.

[Acid-base balance in the cerebrospinal fluid of normal subjects. Comparison of cisternal and lumbar CSF (author's transl)]. / Das Säure-Basengleichgewicht im normalen Liquor Cerebrospinalis

1. An investigation was carried out on 40 normal subjects of the acid-base balance in arterialized capillary blood and in the CSF (17 cisternal and 23 lumbar samples). The pH value was 7.324 in the cisternal CSF and 7.317 in the lumbar CSF, giving a difference of 0.007. The respective pCO2 values were 45.0 mm Hg and 45.9 mm Hg. The HCO3-minus value was 21.8 mMol/l, both in the cisternal and the lumbar CSF. 2. A comparison of the cisternal and lumbar CSF samples by means of the Wilcoxon-Mann-Whitney Test did not show any significant differences. 3. Neither of the groups showed a statistically significant dependence on age or sex. 4. Comparative studies by several authors of the acid-base balance in the cisternal and lumbar CSF are mentioned and discussed with reference to the present results.

[The acid-base balance in the CSF of normal subjects regulatory mechanisms (author's transl)]. / Das Säure-Basengleichgewicht im normalen Liquor cerebrospinalis

1. The acid-base balance (pH, pCO2 and HCO-3) of 23 normal subjects was determined both in arterialized capillary blood and in the CSF. 2. Statistically significant correlations (determined by means of Spearman's rank correlation) were found between: pCO2 in arterialized blood and CSF pH (Rs=--0.372, p is less than 0.05), pCO2 in the CSF and CSF pH (Rs=--0.421, p is less than 0.05), HCO-3 in the CSF and in arterialized blood (Rs=0.623, p is less than 0.05), blood pH and CSF pH (Rs=0.485, p is less than 0.025), pCO2 in the CSF and HCO-3 in the CSF (Rs=0.559, p is less than 0.005). 3. The regulatory mechanisms of the CSF acid-base balance in normal subjects and also in patients with extra-neural or CNS disturbances are discussed.

[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.

[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.

[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.