Medullary actions of tetanus toxin. An electrophysiological study in man.

Brain-stem reflexes have been studied in four patients with generalized tetanus. Inhibitory cutaneous reflexes were abolished or severely depressed. The same held true, to a lesser degree, for excitatory cutaneous reflexes with long polysynaptic pathways. Proprioceptive reflexes behaved variably. The observations are discussed in regard to the possible sources of enhanced motoneuronal activity in tetanus. It is concluded that, besides depression of inhibitory synapses on motoneurons, interneuronal damage may contribute to the tetanus symptomatology.

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.

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. These observations suggest that Binswanger encephalopathy does not differ from multi-infarct dementia.

Transitional forms of Arnold-Chiari and Dandy-Walker malformations.

The morphological findings in 12 cases of Arnold-Chiari and 3 cases of Dandy-Walker malformations are described and compared to those in 2 cases of congenital hydrocephalus of unknown origin, 1 case of isolated meningo-myelocele and 2 cases of normal newborn brains. Lesions common to both types of malformation indicate a same time-related embryogenetic defect of the roofplate of the rhombencephalon, that does not allow the inferior vermis and the choroid plexus to turn inward into the fourth ventricle.

Ischemic lesions of the hippocampus and their relation to Ammon's horn sclerosis. A neuropathological study of two cases and a comparison to the vascular anatomy.

Two adult brains with small ischemic lesions in the hippocampus, due to impairment of the supply from the posterior cerebral artery, are presented. The first case corresponds to what is described in the literature as "incisural sclerosis" and shows no difference in vulnerability between the Sommer and the Spielmeyer sector. In the second case the hippocampal lesion is due to an embolic occlusion of the posterior cerebral artery and consists of selective necrosis of the subiculum, the Sommer sector and part of the endfolium of the pyramidal layer, the Spielmeyer sector remaining noninfarcted. The postmortem angiograms of 12l hippocampi of adults, as well as full term born and premature infants, show that the h1 and h2 sectors and part of the h3 sector of the hippocampus are supplied by the same "sulcus" arteries. Although there is a selective vulnerability to ischemia in some sectors of the hippocampus, which is typical for Ammon's horn sclerosis, this cannot be explained by a difference of arterial supply or by compression of arteries during the process of birth.

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.

Papova virus-like particles in a nigral type of Creutzfeldt-Jakob disease.

In this case of Cruetzfeldt-Jakob disease cortical biopsy demonstrated a large number of papova virus-like particles in the axons, dendrites, astroyctic processes and blood-vessel walls, while necropsy findings disclosed a striking status spongiosus and neuronal degeneration in the substantia nigra. The patient also suffered from a testicular feminization syndrome and was treated with immunosuppressive drugs for pemphigus vulgaris. The ready demonstration of viral particles, the rapid course of the disease and the unusual necropsy findings are discussed and related to the pre-existing diseases of the patient.

Acid phosphatase activity of cerebrospinal fluid cells in leptomeningeal haemorrhage.

One hundred and twenty samples of haemorrhagic spinal fluid were examined by acid phosphatase staining. This enzyme activity starts to appear in mono-histiocytic cells 2 days after bleeding and increases up to the 5th day. After 1 week the activity decreases rapidly. Similar results are found in mixtures of incubated clear spinal fluid, to which blood is added. Acid phosphatase staining is a useful additional method for determination of the age of a leptomeningeal bleed.

Granulomatous angiitis of the nervous system: a clinicopathological study of one case.

The clinical history is presented of a 69-year-old man with a disease starting with a herpes zoster infection and an acute ascending myelopathy, and ending with an intracerebral hemorrhage. The postmortem examination revealed multiple angiitis lesions, restricted to the central nervous system. In review of the 31 previously described cases there were four other patients in whom the granulomatous angiitis of the nervous system (GANS) was associated with a herpes zoster infection. The relation between both disorders is discussed.

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. It was also difficult to separate apoplectic seizures from those occurring due to scar formation after a cerebral infarct, as postapoplectic epilepsy was induced either by the occurrence of a new stroke or by hemodynamic disturbances. Severe generalized or local hemodynamic and metabolic disturbances were also noted in cases of convulsions resulting from a cerebral infarct.

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.

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. Etiologically, the most attractive hypothesis appears to be that the tumour was induced by radiation therapy administered 24 years previously.

Paralytic pontine exotropia in subarachnoid hemorrhage. A clinicopathological correlation.

A syndrome of combined right lateral gaze palsy and right internuclear ophthalmoplegia, followed by a left exotropia in a case of subarachnoid hemorrhage is presented. At necropsy a single small pontine end-zone infarction was found, involving the medial part of the right paramedian pontine reticular formation, the tectospinal tract and the ventral part of the right medial longitudinal fasciculus. The fila radicularia of the right abducens nerve passed through the necrotic area. The mechanism of these characteristic ocular motor disturbances in relation to the post mortem findings and the topopathogenesis of the pontine infarction are discussed.

Migraine-like headache in intraventricular tumours.

In a series of 39 intraventricular tumours, 7 patients had paroxysmal headache as the most important early complaint. The striking similarities with migraine and the misleading role of antimigraine drug therapy are illustrated. Special emphasis is placed on the importance of additional investigations such as funduscopy, EEG, skin X-rays and Ct-scan.

The angioarchitecture of the astroblastoma.

The angioarchitecture of an astroblastoma of the corpus collosum is studied on serial carotid angiograms, made during life, and on post mortem angiograms, performed by filling of the cerebral arterial system with a colloidal bariumsulphate solution and by using the translucidation technique. The results of these investigations are compared to the histological aspect of the tumour. This study reveals the close relationship between the astroblasts and the bloodvessels. The angioarchitectural pattern of this tumour is characterized by new formation of capillaries. It is considered as an additional support to classify the astroblastoma as a separate type of glioma.

Cranial subdural metastases: a clinicopathological study.

During a period of 10 years, 2782 brains were examined at post-mortem and in 17 cases metastases were attached to the dura mater, 8 presenting as multiple nodules 6 as a single nodule and 3 as diffuse dural thickening. The primary growths were, in equal proportions sarcomas, epitheliomas and lymphoid tumours. In contrast to the frequent location of metastases in the axial skeleton (9 cases), brain involvement was rarely observed (2 cases). The clinical findings were non-specific. The difference in biological behaviour between subdural and intracerebral metastases is stressed.

Intracranial bilateral symmetrical calcification on CT-scanning. A case report and a review of the literature.

The case of a 57-year-old woman with idiopathic hypoparathyroidism is presented. A CT-scan showed extensive bilateral symmetrical calcification in the region of the basal ganglia, nuclei of the cerebellum and the cerebral and cerebellar white matter. A review of the literature showed that bilateral symmetrical calcification detected by CT is usually small in extent and is most often confined to the globus pallidus. It is most commonly found in patients older than 50 years, who only rarely have symptoms associated with it. The finding is, though, non-specific and may occur in a variety of pathological conditions both with and without an aetiological relationship. Further study of the cerebral parathormone responsive adenylate cyclase enzyme proves hopeful to elucidate the aetiology of idiopathic bilateral symmetrical calcification.

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.

The arterial angioarchitecture in lacunar state.

The statistical data, obtained in seventy five brains with lacunar state, show that the lenticular nuclei and the periventricular arterial end-and borderzones are the main seats of the infarcts. In teen brains the relationship between the deep arterial angioarchitecture and the location of thirty lacunes is studied by means of the translucidation technique after filling the arterial system with a colloîdal barium sulphate solution. The deep perforating arteries are poorly filled and show several narrowings and post-stenotic dilatations. In twenty-four lacunes, an occlusion of the responsible artery is found. In the six remaining lacunes, no occlusion of the main artery is observed but the side-branches are not demonstrated. The significance of these findings and the relationship between the location of the occlusion and the topography of the lacune are discussed.

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. It is suggested that the infarction and the hydrocephalus further progress in "avalanche effect", which is fatal unless prompt surgical decompression can be carried out.