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A twenty-five year old woman developed a progressive right hemiparesis which remitted within three months, without treatment. The diagnosis was a first relapse of multiple sclerosis. After a 10 year fully asymptomatic period, the patient developed weakness of the legs with falls and swallowing difficulties. Fasciculations and amyotrophy were present in the limbs and the tongue. There were no sensory abnormalities. The electromyogram confirmed the peripheral neurogenic degeneration with signs of anterior horn involvement. Motor and sensory nerve conductions were normal. Muscle weakness and atrophy increased in the limbs and the bulbar territory and the patient died nine months later. The autopsy showed characteristic "old" plaques of multiple sclerosis in the cerebrum with anterior horn cell and pyramidal tracts degeneration, typical of amyotrophic lateral sclerosis, in the spinal cord. Although exceptional, this association of amyotrophic lateral sclerosis and multiple sclerosis leads to the discussion of an etiological immunological dysregulation common to these two diseases.

A 34-year old right-handed man was suffering from recurrent cerebro-vascular insults. CT-scans revealed several subcortical lacunar infarcts, and leukoaraïosis. Arteriography of the left and the right carotid arteries was performed respectively on the 4th and the 9th year of the disease, and did not elicit significant extracranial and intracranial vascular lesions. There were no arguments in favor of infectious, inflammatory, or auto-immune vascular diseases. The patient had tardive hypertension and dementia, and died at the age of 44. Pathological findings, limited to the brain and cervical spinal cord, revealed numerous ischemic lacunar infarcts. Histological lesions were consistent with the diagnosis of arteriosclerotic leukoencephalopathy. There were oedema, palor, and loss of myelin in the white matter, and nonspecific diffuse arteriosclerotic lesions that were particularly pronounced in the intimal part of the arterial wall. No inflammatory process nor amyloid deposits were found. Despite the onset of the disease in a young adult and the late occurrence of hypertension, our case report shares most of the pathological features of the Binswanger's type of arteriosclerotic encephalopathy.

Two cases are reported of two women who had developed progressive hypopituitarism without visual disturbances. Pituitary tumors were evoked by radiological findings. The patients underwent surgical removal of invasive intrasellar tumor by transphenoidal route. Giant-cell granuloma was histologically demonstrated with negative special staining for tuberculosis acid-fast bacilli, bacteria and fungi. Secondary granuloma due to tuberculosis, brucellosis or neurosarcoidosis was ruled out by serological grounds. The prognosis was discusses through a follow-up of 7 years in the first case and 7 months in the second one. The appropriately documented 31 cases collected from the literature were compared with our two cases.

Neoplastic angioendotheliosis is a rare, severe, disease characterized by neoplastic proliferation of mononuclear cells within the lumen of small blood vessels. The clinical signs are usually dermatological and neurological. We report the case of a 57 year old woman who died after 20 months of subacute dementia. She had had also transient recurrent episodes of right paresthesiae and paresis. CSF proteins were increased. MRI showed areas of high signal in the white matter. Post-mortem showed widespread ischemic lesions, mainly in the subcortical white matter. Microscopically there was marked proliferation of cells in most CNS blood vessels. Similar cells were present in adrenals, liver, uterus, lungs and kidneys. Immunohistochemical studies showed intensive staining for leukocyte common antigen and negative staining for factor VIII-related antigen, a specific endothelial cell marker.

We report 3 cases of epilepsy with bilateral occipital calcifications followed up for several years. These cases were compared with 21 published cases and were found to differ from the classical Sturge-Weber syndrome on several points: 1) the disease appeared around the age of 5 years and consisted of focal epilepsy without neurological or mental disorders; 2) the epilepsy was easy to control during 2 to 5 years. This was followed by a diffuse encephalopathy with severe, treatment-resistant epilepsy, Gerstmann's syndrome, optic ataxia, cerebellar syndrome and slow activity at EEG. It appears from these 3 cases that: 1) occipital calcifications may be unilateral at the onset of the disease; 2) visual evoked potentials are affected at a late stage, and 3) CT scans are of considerable value in the prognosis of benign epilepsy in childhood.

The phenotypic characteristics of 7 subependymal giant cell astrocytomas (GSECG) (6 of these being associated with tuberous sclerosis) are studied using morphological and immunohistochemical methods with antiserums against vimentine, glial fibrillary acid protein (GFA), S100 protein, and neurofilaments. The glycoproteic secretion of the tumor cells was also analyzed after exposure to Concanavalin A (CON A) by a direct fluorescent method. Our results suggest that some GSECG originate from specialized ependyma (circum-ventricular organs). They have the same location (foramen of Monro), present some common ultrastructural features (cytoplasm with lumen containing cilia), are positive with certain immunohistochemical markers (staining with S100 protein in 4 cases, with vimentin in 3 cases) and show a strong glycoproteic secretion (positive with CON A). Therefore, some GSECG might be considered hamartomas of specialized ependyma, with a reduced evolutivity potential.

The aim of this study is to sum up our current knowledge on the so-called "normal pressure hydrocephalus" syndrome, revisited by the authors as the "adult chronic hydrocephalus" syndrome (A.C.H.). It is based upon 1) a review of the literature dealing with the subject (518 references) and 2) an original series of 243 cases summarizing a 10 year experience of 2 french neurosurgical teams (Lyon: 123 cases, Clermont-Ferrand: 120 cases). Part I is devoted to the review of pathologic and pathophysiologic data. Both autopsy and microscopic findings clearly show 2 categories of anomalies in the brains of patients showing this condition: "non specific" lesions may be either causative (leptomeningeal thickening, villositar sclerosis) or secondary to hydrocephalus (ependymal wall disruption, sub-ependymal "edema"). Conversely, "specific lesions" are either degenerative (Alzheimer-type) or ischemic in origin. They seem unrelated to hydrocephalus, but may play a role in the ventricular enlargement by favoring changes in brain elastic properties. Data available from brain biopsies (13 cases) have confirmed the high incidence of such "specific lesions" of the parenchyma. Following the Hakim's initial hypothesis, several mechanisms have been proposed to explain the progressive ventricular dilation despite a fall in C.S.F. pressure. Experimental and clinical data (namely those referring to the pathogenesis of secondary A.C.H.) are reviewed. The sequence of events following acute ventricular obstruction is analysed. The ultimate state of chronic uncompensated hydrocephalus involve multiple factors among which a trans-cerebral mantle pressure gradient may play a major role. The intervention of compensatory mechanisms (reduction of C.S.F. production, establishment of alternative pathways of C.S.F. absorption) is also discussed. Part II is devoted to the presentation of the clinical material. Series of the literature including more than 20 surgical cases are briefly reviewed together with the authors' material and methods. Criteria of selection of patients, methods of clinical evaluation and of statistical analysis of results are exposed. Of the 243 patients of the present series, 225 were managed by C.S.F. shunt (V.A.: 137, V.P.:60, L.P.:28). Results were as follows (mean follow-up:31 mths +/- 28): good results (including excellent results): 128 (52.6%), fair: 52 (21.3%), poor and aggravated: 49 (20%), early deaths: 14 (5.7%), 12 of those post-operatively. The rate of surgical complications was 35% (S.D.H.: 16.8%, sepsis: 4%, seizures: 4%, shunt malfunction: 10.6%) leading to reoperation in 25.7%. Long-term survival rate was 75% (172 alive patients and 57 late deaths).(ABSTRACT TRUNCATED AT 400 WORDS)

We report two clinico-pathological cases with an initially hidden brainstem tumour presenting as chronic hydrocephalus of "idiopathic type". Diagnosis was established respectively one and two years after a successful shunting procedure, as repeated CT scan was performed because of gait deterioration. The first case was a bifocal glioblastoma invading the leptomeninges of the posterior fossa and spinal cord, and resulting in a communicating hydrocephalus. The second case was an ependymoma of the fourth ventricle leading to e non-communicating hydrocephalus. Rarity of such cases is emphasized.

Two cases of mainly interstitial myositis with epitheloid and giant cells are reported. In the first case, the late amyotrophic-type paralysis of the two girdles, of the clinical myositis type, was associated with severe myocardial damage, the atrioventricular conduction disorder requiring a pace-maker. This observation would appear to be a true polymyositis of the collagen diseases, in view of the associated skin affection, death occurring within a year, a large increase in serum muscle enzymes and urine creatine, the existence of a rheumatoid factor and striated muscle antibodies, and a large increase in M and G immunoglobulins. In the 2nd case, the amyotrophic affection of the pelvic girdle was associated with progressive blindness, diabetes insipidus, and anterior pituitary insufficiency. Death occurred after two years and autopsy showed a suprasellar dysgerminoma (ectopic pinealoma) without any visceral localization of sarcoidosis. The authors discuss the concept of granulomatous polymyositis, autonomous with respect to the sarcoidosis, and sometimes symptomatic of an inflammatory connective tissue condition or a malignant tumor.

Two sporadic cases of striato-nigral degeneration were characterized by the onset at the age of 61 of an akinetic-hypertonic syndrome and of a minimal or absent rest tremor, with a pyramidal syndrome, sphincter disorders, dysphonia, difficulty in swallowing and an unfavorable development in the space of two or three years not affected by L-Dopa. In one case, total insomnia was demonstrated by means of 3 polygraphic recordings during the night, two of them consecutive, and aprobenecide test showed a definite fall in H.V.A. and 5 H.I.A.A. in the lumbar cerebrospinal fluid. Anatomical verification showed, in one case, isolated putamino-nigral degeneration, and in the other associated with lesions of the olivo-cerebellopontine system. Much of the excess pigmentation found only in the putamen was melanotic in character and resulted perhaps from the striato-nigral degeneration, making it possible to classify this more accurately among the other multisystem degenerations, especially olivo-ponto-cerebellar atrophy and Shy-Drager syndrome

At the age of 6 years a patient developed disorders of character, intellectual deterioration, tremor, falls and epileptic seizures. This was followed by extrapyramidal and pyramidal disorders with a fatal outcome at age 21. There was no family history. Histopathology showed evidence of Hallervorden-Spatz disease, remarkable by the diffusion of spheroids into the central nervous system gray matter and by the presence of innumerable Lewy bodies in the substantia nigra and locus coeruleus. Similar findings have been reported in only 3 other cases of typical Hallervorden-Spatz disease. They suggest a preferential affection of monoaminergic neurons.

The autors study 76 human brains, 36 of which are dissected. They observe a "T1 T2 bridge" over the first temporal and second temporal gyri more often on the left side than on the right. They find the maximal width of the superior face of the second temporal gyrus predominant on the right side in 70% of cases, on the left side in 15% and equivalent on both sides in 15%. The fusiform gyrus is found wider on the left side in 53% of cases, on the right in 22% and equivalent on both sides in 25%.

The authors report a clinically typical case of trichopoliodystrophy (Menkes' disease) ; Neuropathological studies showed classical alterations, particularly irregular thickenings of Purkinje cell dendrites, and some less usual modifications : presence of an ectopic neuronal layer in the cerebellar molecular layer, suggestive of Obersteiner cells stopped in their progression ; normal appearance of myelin and elastic structures of systemic arteries. Ultrastructural studies showed some calcic concretions in mitochondria of hepatocytes and Purkinje cells, and lamellar structures in the cytoplasm of Purkinje cells, probably of ergastoplasmic origin. The activity of dopamine-beta-hydroxylase (DBH, or norepinephrine synthesising enzyme) and of phenylethanolamine - N-methyltransferase (PNMT, or epinephrine synthesising enzyme) was studied in 22 samples of brain stem. Activity was reduced in 3 samples, and increased in 4 : 6 of these 7 samples were from structures involved in cardiovascular and respiratory control. These results suggest the existence of a central vegetative neuropathy, which could explain the cardiovascular and respiratory disturbances sometimes reported in Menkes' disease ; but this neurochemical study gave no account of the sleep organization alterations of the patient.

Clinical and pathological findings in a case of subacute combined degeneration of the cord are described. The patient was a severe alcoholic but there was no vitamin B12 deficiency. Lesions were similar to those observed in Lichtheim's disease. Biological tests demonstrated frank folic acid deficiency, with very low serum and red cells levels. Clinical features included only mild macrocytic anemia, and flexion quadriplegia after a period of disorders of equilibrium. Higher functions were unaffected. Apart from alcoholism, no other toxic or digestive causative factors were found.

6 thyroid gland and 3 cervical lymph node clear-cells carcinomas are investigated by means of indirect immunofluorescence detection of thyroglobulin. Immunofluorescence was positive only in 1 case, which was a primitive thyroid carcinoma. In 7 other cases, its negativity pointed at the metastatic nature of the lesion, from a clear cell carcinoma of kidney (in 4 cases, nephrectomy was very remote, and in 3 cases, the renal lesion was discovered after our examination of the thyroid tumour). One observation showed negative staining reaction, and was a propagation of a parathyroid carcinoma to the thyroid gland. It is concluded that thyroglobulin can be used as an immunohistochemical marker to establish whether a clear cell cervical tumour originates from thyroid gland, or is a metastatic neoplasm.

The action of VM 26 on cerebral glioblastomas was suggested by studies of phase II or protocols in which the drug was used in association. The randomised protocol presented here, involving 10 treated subjects and 11 controls, showed that VM 26 was active in terms of the duration of survival of patients undergoing surgery for a glioblastoma. The mean survival was 16.4 months as against 9.6 months in the controls (statistically significant difference at p less than 0.05). This activity would tend to characterise the drug as one of the essential factors in the chemotherapy of all malignant gliomas, in particular since it is itself free of all haematological complications, even at high doses, and causes scarcely any allergic problems.

The topographical distribution of neurons containing LHRH has been investigated in newborn hypothalamus using the peroxidase anti-peroxidase technique. In control subjects, LHRH immunoreactive (LHRH-IR) perikarya have been mainly observed essentially in the infundibular nucleus. The preoptic region displayed a moderate density of LHRH-IR cell bodies. High LHRH innervation was observed in the anterior hypothalamus in the lamina terminalis and in the mediobasal hypothalamus in the median eminence, and in the peri- and paraventricular regions. In sudden death infant syndrome, a comparable mapping was observed, except a low density in the mediobasal peri- and paraventricular areas.

The authors report an observation in which four siblings were affected by both multiple cutaneous epitheliomatosis and complex but relatively stereotyped neurological disorders. Clinically, the main syndrome was cerebello-spinal ataxia with involvement of the anterior horns of the spinal cord with less marked pyramidal and extra-pyramidal features. Neuropathological examination of one of the cases revealed lesions of essentially cerebello-spinal degeneration suggestive of Menzel's disease. The possible connection between the neural and cutaneous lesions is discussed. All the various etiological categories possible have been ruled out; not one being entirely satisfactory, except for the very broad category of genetic neuro-dermatoses.

CT scan in 4 patients with Marchiafava-Bignami's disease in its acute alcoholic form, who died after 12, 18, 30 and 50 days respectively, demonstrated large symmetrical bilateral hypodense areas in the hemispheric white substance. These areas were either widespread or more marked posteriorly, and were independent of the corpus callosum. Pathologic examination of the brains showed necrotic regions, often with cavities, or areas of demyelinization of the centrum ovale correlating with the hypodense areas as seen on CT scans. In two cases CT scan further showed regions of linear hypodensity in front of and medial to the frontal ventricular horns which could have corresponded to the corpus callosum necrosis found on the brain sections. CT scan can thus contribute to the diagnosis of Marchiafava-Bignami's disease.

The authors report a fairly typical clinico-pathological case of acute hemorragic leucoencephalitis (A.H.L.E.). Both clinical and histological features appeared particularly acute. At autopsy a visceral evolving tuberculosis was diagnosed. Such an etiological circumstance has not been, apparently, reported in cases of A.H.L.E. published as such. Three other etiological circumstances were noticed: pyuria, treatment by cephalotin and treatment by gentamycine. The physiopathogenesis of A.H.L.E. remains obscure and will not be clarified before detailed immunological studies can be performed. But the disease is rare and brisk and diagnosis usually post-mortem.

The authors describe an anatomo-clinical case of trichopoliodystrophy in many ways similar to cases already published. The original features of the case are as follows: the ultrastructure of the duodenal mucosa is normal; the endoplasmatic reticulum of the putaminal neurons contains many-layered lamellar structures probably composed of protein; type II fibres predominate in the striated skeletal muscle suggesting abnormal neuromuscular maturation.

After a classical neuropathological study assessing the diagnosis, the activity of the GABA synthetizing enzyme, glutamate decarboxylase (GAD), was assayed in 6 brain areas, in 8 cases of Parkinson's disease, 2 cases of idiopathic orthostatic hypotension and 9 control cases carefully matched. The activity of GAD is not impaired, as classically believed, in parkinsonian brains, particularly in substantia nigra and pallidum. This preservation would indicate the absence of lesion of GABAergic neurones in Parkinson's disease. In the cases of other Parkinsonian syndromes, the number of cases studied is too limited to allow any generality; but they are, however reported because of their rarity.

Detailed observation of a case of laminar necrosis of almost the whole of the cerebral cortex has led to several deductions concerning the physiology of the cortex in man. It suggests that the cortex has a limited influence on sleep, interfering particularly in the electro-encephalographic translation of slow sleep and, curiously, in the organisation of sleep, paradoxical sleep is hardly affected at all. It confirms the importance of the human cortex in the acquisition of elementary skills and describes a new oculomotor pattern in a decorticate man.

A patient presented with disorders of saccadic and pursuit movements a wandering gaze, the impossibility to maintain fixation, a concentric shortening of visual attention mainly of the right side, an incomplete right homonymous hemianopia (respecting 10 to 20 degrees), and optical and auditory ataxia predominantly affecting the right hand. Pathology showed a large left parietal glioma in the white matter in the superior and inferior parietal lobes, with microscopic evidence of parietal cortex invasion. A hematoma on the right side was situated entirely parasagitally in the precuneus, approaching the cortex at this level. The two lesions were independent and the splenium was intact. The frontal lobes were normal. A review of nearly 30 cases of bilateral syndrome of the parieto-occipital junction showed that anomalies of visually guided ocular movements were always present. These cases require differentiation from those, such as the present case, which involve mainly a visuomotor coordination disorder and from those with predominant visual disorientation.

The activity of Phenylethanolamine-N-Methyltransferase (PNMT) was assayed in 12 brain structures in a group of 8 chronic psychotics, a sub-group of 5 schizophrenics and a group of 7 normal controls. It was found significant decreased in substantia perforata posterior and nucleus accumbens. These two structures belong to the meso-limbic system. These data, therefore, suggest an inhibition of PNMT and/or an alteration of putative adrenergic neurons in the meso-limbic system, in schizophrenia.