Pathogenesis of Binswanger chronic progressive subcortical encephalopathy.

We studied the clinicopathologic findings in four hypertensive patients with multiple leukomalacia, demyelinated lesions, and lacunar state. Only one patient had clinical evidence of dementia. The periventricular watershed infarcts were attributed to transient episodes of cardiac failure in brains with a compromised circulation in the territory of the deep perforating branches.

Wernicke's encephalopathy in patients with tumors of the lymphoid-hemopoietic systems.

In three treated patients with a generalized invasion by a tumor of the lymphoid-hemopoietic systems, the neuropathologic findings were consistent with Wernicke's encephalopathy. The clinical picture was atypical, but thiamine deficiency by severe malabsorption was the probable cause of this neurologic complication. It is postulated that the chronic form of Wernicke's encephalopathy must occur more frequently than previously shown in treated and long-standing cases of such kinds of tumors.

Cytophotometric DNA determination in human oligodendroglial tumours.

Cytophotometric DNA determination was performed on 11 surgically resected oligodendroglial tumours and on oligodendroglial cells in two normal brains. The results are compared with the histological findings, the mitotic index and the degree of clinical malignancy. In this type of glioma there is no correlation between the DNA pattern and the histological appearances and the mitotic index.

The target phenomenon in tenotomized rat gastrocnemius muscle. A comparative electrophysiological and morphological study.

In ten female rats electrophysiological recordings and subsequent histological and histochemical examinations were performed at different time intervals and in different parts of the gastrocnemius muscle, following tenotomy and following combined tenotomy and neurotomy. Repetitive motor action potentials were more easily evoked during the period and at the sites of occurrence, of target fibres in tenotomized gastrocnemius muscle with an intact nerve supply. Both phenomena, the electrophysiological as well as the morphological one, were strongly inhibited by the simultaneous neurotomy.

Parkinsonism in patients with cerebral infarcts.

From 220 necropsies of patients with cerebral infarcts, 5 were selected, in which the clinical history revealed typical parkinsonian symptoms with a progressive course of the extrapyramidal disorder. No correlation could be found between the location and the size of the infarcts on the one hand and the signs of parkinsonism on the other hand. In the 5 cases presented, the substantia nigra showed patchy areas of neuronal degeneration without any Lewy bodies nor neurofibrillary changes, around a pronounced status cribrosus in areas irrigated by paramedian branches of the mesencephalic arteries.

Epilepsy in patients with cerebral infarcts.

Out of series of 240 patients, with proved cerebral infarcts at necropsy, 14 with a clinical history of epilepsy were selected. The possible etiology of seizures was analyzed by comparing the clinical and pathological data. It appeared from this series that epileptic spells, preceding for years the occurrence of a stroke, were due to other causes than to cerebrovascular insufficiency.

Dumb-bell sarcoma of the foramen jugulare with syringomyelia. A radio-induced tumour?

The clinicopathological findings of a 50-year-old man, who developed cervicothoracic syringomyelia at the age of 25 are presented. He was given radiation therapy at the age of 33. At the age of 57 he developed a foramen jugulare syndrome on the left, caused by a low grade leiomyosarcoma.

Influence of 2,4 dichlorophenoxyacetate and of dantrolene sodium on the target phenomenon in tenotomized rat gastrocnemius muscle.

The effect of 2,4 dichlorophenoxyacetate and of dantrolene sodium on the occurrence of target fibers in tenotomized rat gastrocnemius muscle was studied. Neither of the drugs influenced the appearance of target fibers or its inhibition by simultaneous neurotomy.

Cytophotometric DNA determination in human astroglial tumours.

The DNA content is determined by the cytophotometric method in 16 astroglial tumours, classified according to the degree of clinical malignancy. The values are compared to those found in non-tumoural astrocytes. This method appears to be more reliable in assessment of the degree of malignancy of the tumour in biopsy material than histological criteria and the mitotic index.

Reye's syndrome in an adult.

Reye's syndrome or encephalopathy with fatty infiltration of the liver occurs mainly in childhood and to a lesser degree in adult age. This report concerns a rare adult case of this syndrome in Europe. The different diagnostic steps are described, with special emphasis on the enzymatic disturbances of the Krebs-Henseleit cycle and on the morphological criteria needed to confirm the diagnosis.

Subarachnoid hematoma in staphylococcal septicemia. A report of two cases.

Two cases of subarachnoid hematoma, due to a Staphylococcus aureus septicemia are described. The clinical as well as the pathological findings suggest a two-step evolution, consisting first in the appearance of a small to moderate stroke, due to a septic embolic occlusion of a leptomeningeal artery and to cerebral infarction, followed by rupture of the artery and the formation of the subarachnoid hematoma, acting as a mass-lesion and leading to death by uncal herniation and brain stem compression. The typical location of the hematoma over the convex surface of a cerebral hemisphere suggests that local factors, such as the vicinity of patent leptomeningeal anastomoses and the fibrosis of the leptomeninges, are probably predisposing factors for its occurrence.

Communicating hydrocephalus in treated leukemic patients.

7 brains out of a series of 124 necropsies of treated leukemic patients show a communicating hydrocephalus, without invasion of the central nervous system by the malignant blood cells. In 4 cases it is proven that the hydrocephalus is due to obstruction of the liquor drainage at the level of the arachnoidal villi and dural sinuses, by the leukemic invasion of these structures. It is shown that, even in case of preventive brain irradiation and intrathecal-administered cytotoxic drugs, these structures remain areas, in which the leukemic cells are difficult to destroy.

Vertebrobasilar insufficiency due to Tumoural emboli.

A case, which is clinically characterized by a subacute vertebrobasilar syndrome, is presented. The necropsy reveals multiple infarcts in brainstem, cerebellum and left cerebral hemisphere, due to tumoural emboli of a non-detected primary tumour. It is postulated that the latter has to originate in the lungs in order to produce this unique type of cerebral arterial embolism.

Nemaline bodies in a progressive infantile neuromuscular disorder.

Nemaline bodies are described in a case of a progressive neuromuscular disorder, which is difficult to classify. The clinical syndrome is not characteristic of a nemaline myopathy. It is argued that the finding of nemaline bodies is in itself not specific of any neuromuscular disease.

Periventricular leukomalacia in adults. Clinicopathological study of four cases.

The pathological findings in four patients with courses characterized by acute coma and respiratory insufficiency occurring in obscure circumstances are presented. Carbon monoxide intoxication was excluded. After an early partial recovery from coma, the patients remained in a persistent vegetative state, with a tetrapyramidal syndrome.

Morphological properties of experimentally produced target fibres in tenotomized rat gastrocnemius muscle.

The gastrocnemius muscles of 3 groups of 10 rats, sacrified 5, 7, and 12 days respectively, following tenotomy, were submitted to different types of fixation, fixative and embedding. The occurrence of target fibres is shown not to be an artefact due to the histological procedures. Further examination demonstrates that the target phenomenon occurs in the shortest fibres on the medial side of both heads of the gastrocnemius muscle and that it consists mainly of a disarrangement of the contractile elements of the muscle fibres.

Cerebellar infarction and internal hydrocephalus.

Four cases of acute cerebellar infarction producing hydrocephalus are presented. Only the patient, who is submitted to surgical decompression of the posterior fossa, survives, while the other three die. In the latter the necropsy shows a hemorrhagic cerebellar softening in the territory of the inferior posterior cerebellar artery, causing tonsillar herniation and impairment of the liquor drainage at the level of the inferior part of the 4th ventricle.

Infantile spinal muscular atrophy. Unusual fiber typing and distribution in a muscle biopsy.

A right gastrocnemius muscle biopsy of a 13-month-old floppy male infant, which appeared at a more advanced age to suffer from an infantile spinal muscular atrophy, showed unusual histochemical changes: the chequer-board distribution was replaced by three groups of muscle fibers with a same mean narrow diameter of 12.5 micrometer. The smallest groups could easily be recognized as type I and type IIB fibers, while the largest group, involving more than 75% of the whole biopsy, revealed an intermediate hybrid fiber population, which would be classified as type I, if based on their phosphorylase and myofibrillar ATPase activities alone.

Development and inhibition of the target phenomenon in tenotomized rat muscle.

The sequence of development of the target phenomenon in tenotomized gastrocnemius muscle was studied: the presence of target fibres was preceded by the occurrence of contraction bands and of "moth eaten" appearance of the fibres. This phenomenon was far more pronounced and occurred earlier in type II than in type I fibres. This target phenomenon and the contraction artefacts could be inhibited in the tenotomized muscles by simultaneous neurotomy or immobilization of the muscle with a plaster cast.

Acute viral polymyositis with predominant diaphragm involvement.

A 20-month-old mentally retarded girl developed an acute upper respiratory infection, followed by breathing difficulties, leading to death. Picorna virus-like particles were demonstrated in a mildly affected quadriceps femoris muscle biopsy, while the necropsy findings demonstrated an acute polymyositis with predominant diaphragm involvement. The mental retardation was due to micropolygyria of the brain.

Ultrastructural study of a muscle biopsy from a patient with subacute myelo-optic neuropathy.

A man, aged 46, who had been taking Clioquinol in high doses for a long period, developed a characteristic neurological syndrome of subacute myelo-optic neuropathy rather abruptly. Electron microscopical examination of the muscle biopsy, obtained five months after the onset of the disease, revealed severe degenerative changes of the presynaptic nerve endings and some unique paracrystalline inclusions in the sole plate region. The latter may represent the morphological expression of the toxic agent which is held responsible for the subacute myelo-optic neuropathy.

Thrombosis of the mesencephalic artery. A clinico-pathological study of two cases and its correlation with the arterial vascularisation.

Two cases of thrombosis of the mesencephalic artery are described : the location of the occlusion and of the infarcted area in the upper mesencephalon is correlated to the clinical syndrome, in particular to the existence or the absence of an oculomotor paralysis. In addition 27 normal brains are examined by means of the translucidation and the radiographic techniques after filling of the arterial system with a colloidal barium sulphate solution. This study supports the subdivision of the upper mesencephalon into five vascular territories and justifies the denomination of mesencephalic artery, which supplies a transitional region between the diencephalon and the brainstem.