[Cerebellar infarction in the area of the posterior cerebellar artery. Clinicopathology of 28 cases]. / Les infarctus du territoire de l'artère cérébelleuse postéro-inférieure. Etude clinico-pathologique de 28 cas.

We report a neuropathological study of cerebellar infarctions involving the territory of the posterior inferior cerebellar artery (PICA) in 28 cases. Fifteen cases involved the PICA territory only. In 13 cases infarctions in the anterior inferior cerebellar artery (AICA) territory and/or in the superior cerebellar artery (SCA) territory were also present. A thorough post-mortem study of the arterial supply of the brain from the heart up to the cerebellar arteries, including the cervical spine segment of the vertebral arteries was performed in 27 cases. The territory of the cerebellar infarcts has been ascertained. In 15/28 cases (54 percent), infarction involved the PICA territory only (17 infarcts). All of these cases had a benign outcome and death was due to another cause. Six of these were recent infarctions. None had evidence of swelling and tonsillar herniation. Infarcts were generally of small size and involved the entire PICA territory in only 2 cases. Most of these cases were unexpected discovered at autopsy. Cerebellar infarction in the territory of the medial branch of the PICA (9/17 infarcts) drew grossly a set square with a dorsal base and a ventral top headed for the IVth ventricle. Five out of these cases were associated with infarction in the dorsal and lateral medullary territories. Retrospective clinical study showed that they had been unnoticed or overshadowed by other neurological disorders (4 cases), or presented as Wallenberg's syndromes (4 cases), or as a pure vestibular syndrome (due to an infarction involving only the cerebellum) mimicking an acute labyrinthine disorder (1 case). Infarctions in the territory of the lateral branch of the PICA (5/17 infarcts) always occurred without medullary involvement. All of them were unexpectedly discovered at autopsy, and were unnoticed during the life (3 infarcts) or were overshadowed by other neurological disorders (2 infarcts). That was also the case in 2 cases of infarction in the whole PICA territory (3/17 infarcts). Thus infarctions strictly localized to the entire PICA territory only were rare. Thirteen/28 cases (46 p. 100) of infarction in the whole PICA territory were associated with infarction in the AICA and/or the SCA territories. This resulted from an association with other infarctions and not from an abnormally large territory of the PICA. Cerebellar swelling with brain stem compression and tonsillar herniation occurred 8/13 cases (62 p. 100). There were other massive median and paramedian brain stem infarctions involving midbrain, pons or medulla in 55 p. 100 of 13 cases.(ABSTRACT TRUNCATED AT 400 WORDS)

[Management of amyotrophic lateral sclerosis]. / Approches de la prise en charge dans la sclérose latérale amyotrophique.

There is no specific treatment for patients with amyotrophic lateral sclerosis. Nevertheless several associations of patients have been created to stimulate the research to find the causes and treatments of the disease and to help the patients. In spite of the absence of a specific treatment, new symptomatic therapeutic resources have been recently introduced in order to modify the management and in some cases to improve the condition of patients with ALS. Among these resources, it is worth outlining those which are devoted to the medulla trouble such as the permanent gastric tubes. The respiratory insufficiency is an early phenomenon and is considered the main prognostic factor. Its management should become soon one of the main therapeutic targets. In some countries the indications of devices for respiratory assistance at home are not rare. It is not impossible that in a near future we shall propose such devices to our patients. Beside these therapeutic resources which are difficult to manage, there are numerous drugs used to improve spasticity, cramps or drooling. Physiotherapy and speech therapy are of great importance. The management of such patients needs the cooperation of all the medical and paramedical members and the family whose role is essential.

The clinical spectrum of alcoholic pellagra encephalopathy. A retrospective analysis of 22 cases studied pathologically.

A retrospective clinical study of 22 heavy alcohol drinkers is reported in which postmortem study showed diffuse chromatolysis of neurons identical to that found in neurological pellagra, associated in 13 cases with Marchiafava-Bignami disease and/or Wernicke-Korsakoff disease. The clinical features included confusion and/or clouding of consciousness, marked oppositional hypertonus ('gegenhalten') and myoclonus. Because of the frequent coexistence of other alcoholic encephalopathies in the same patient, alcoholic pellagra was often unrecognized. Fifteen patients received thiamine and pyridoxine therapy without niacin. It appeared to aggravate the neurological state or to trigger the development of alcoholic pellagra encephalopathy in 9 cases. The relationship between pellagra occurring during thiamine and pyridoxine therapy and 'nicotinic acid deficiency' is discussed. Multiple vitamin therapy should be given in the treatment of undiagnosed encephalopathies in alcoholic patients.

[Pigmentary orthochromatic leukodystrophy. Van Bogaert and Nyssen disease]. / Leucodystrophie orthochromatique pigmentaire. Maladie de van Bogaert et Nyssen.

A 43 year old woman with a strong likelihood of familial history, developed progressively a spastic tetraparesis associated with intellectual deterioration and terminal epileptic fits. She died 11 years after onset of the clinical disorders. Neuropathological study revealed an orthochromatic leukodystrophy. Macrophages and glial cells of the white matter contained a brown-yellow, autofluorescent pigment which stained positively with PAS, Perls stain for iron and Masson-Fontana. Electron microscopy showed electron dense, membrane bound intracytoplasmic lamellar inclusions with curved or straight parallel arrangement or fingerprint pattern, in white matter macrophages, astrocytes and oligodendrocytes. Cortical cells contained lipofuscin which was normal in type and amount. This suggests that the material in white matter glial cells and macrophages is ceroid pigment; however, the distribution is not that seen in ceroid-lipofuscinosis. Similar inclusions have been found in oligodentrocytes in other forms of orthochromatic leucodystrophy. Ten similar cases of pigmentary type of orthochromatic leucodystrophy have been reported previously; only one had had an ultrastructural study.

[Cerebrospinal fluid proteins form the colloidal benzoin reaction to the present: a study of intrathecal immunity]. / Les protéines du liquide céphalo-rachidien du benjoin colloïdal à nos jours: l'étude de l'immunité intrathécale.

The benzoin reaction, described by Guillain, Laroche and Lechelle (1920) was probably the first test evaluating local (intrathecal) immunity in inflammatory diseases of the nervous system. Significant advances have taken place during the past 25 years: 1) Exact and precise determination of cerebrospinal fluid total protein by Lowry's method, and separation of gamma-globulins by precipitation or electrophoresis with identification of the oligoclonal aspect, the picture of intrathecal IgG synthesis. 2) Immunochemical determination of immunoglobulins and complement components by electro-immunodiffusion and detection of intrathecal anti-nucleic acid antibody synthesis. Different empirical formulae have been proposed to evaluate the intrathecal immunity. This evaluation allows: 1) a neuro-immunological classification of cerebrospinal fluid patterns. 2) a better appreciation of evolutivity in some neuro-immunological diseases (e.g. multiple sclerosis). 3) an evaluation of the antibody specific activity (ASA) and a comparison between this ASA in plasmatic and in intrathecally synthesized IgG. The study of complement components, also, can give some informations on the evolutivity of the immunological processus, suggesting, in some patients, an intrathecal formation of immune-complexes.

Postradiation segmental myoclonus selectively inhibited by REM sleep (sleep-wake myoclonus).

A 46-year-old male patient was irradiated following left orchiectomy for seminoma. Three months after radiation, segmental myoclonus appeared involving all skeletal muscle groups up to the level of D10, the area of radiation. Urodynamic testing showed the presence of segmental myoclonus in the urethral sphincter. An around-the-clock 'continuous segmental myoclonus' during sleep and wakefulness with a frequency of 1 c/s and an amplitude of 400 microV selectively inhibited by REM sleep was not found in the literature. This continuous segmental myoclonus caused by a radiation myelopathy at D10-D11 was followed up for 2 years. It was partially controlled by carbamazepine.

Serum and CSF humoral immunity in Guillain-Barré syndrome: clinical correlations.

Paired samples of CSF and serum obtained from 29 patients affected with Guillain-Barré syndrome (GBS) were analyzed for various protein levels, including immunoglobulins and complement components. An attempt was made to correlate these findings to the clinical stage, severity, and duration of the disease. Intrathecal IgG synthesis was detected and quantified by means of a previously reported formula. It is practically constant in the GBS during the stage of stabilized paralysis, and is significantly greater in this stage than in the stage of progressive paralysis. Moreover, it increases with severity and duration (longer than 3 months) of the disease. Evidence of intrathecal C3 consumption is also presented.

'Expanding cerebral lacunae' in a hypertensive patient with normal pressure hydrocephalus.

A hypertensive patient who had been treated successfully for normal pressure hydrocephalus (NPH), died from a left thalamic haemorrhage. Neuropathological examination showed recent and old thalamic haematomas and numerous parenchymal cavities or 'cerebral lacunae'. Two lacunae bulged into the lateral ventricles, and had all the characteristics of so called 'expanding lacunae'. They were surrounded by a single layer of epithelial-like cells, contained a normal, patent, arteriole, and presented as space occupying lesions. Only two similar cases have been reported previously. The complexity of the neuropathological features of 'cerebral lacunae' is emphasized and the relationships between cerebral hypertensive disease, NPH, and expanding lacunae are discussed.

The epidemiology of Creutzfeldt-Jakob disease: conclusion of a 15-year investigation in France and review of the world literature.

During the 15-year period 1968-1982, a total of 329 patients dying of Creutzfeldt-Jakob disease (CJD) were identified in continental France. Annual mortality rates stabilized at 0.5 to 0.6 cases per million (1.1 to 1.2 cases per million in Paris). Six percent of cases were familial. Although the frequency of CJD was related to population density, no contacts could be established among the great majority of patients. No association with socioeconomic factors, preceding trauma or surgery (excepting one iatrogenic neurosurgical case), or exposure to animal sources of infection was identified. Evidence from this and other epidemiologic studies suggests that CJD is a minimally contagious disease that may be principally acquired in early life from presymptomatic patients, asymptomatic carriers, or chance contamination by environmental sources. It is possible that CJD could also occur sporadically as a noncontagious disease by a mechanism akin to oncogenes in carcinogenesis.

[Aphasia without amusia in a blind organist. Verbal alexia-agraphia without musical alexia-agraphia in braille]. / Aphasie sans amusie chez un organiste aveugle. Alexie-agraphie verbale sans alexie-agraphie musicale en braille.

A 77 year old right handed male was blind since the age of 2. He presented with an infarction involving the territory of the left middle cerebral artery involving the temporal and the inferior parietal lobes. He had learned to read and write language as well as read and write music in braille, ultimately becoming a famous organist and composer. There were no motor or sensory deficits. Wernicke's aphasia with jargonaphasia, major difficulty in repetition, anomia and a significant comprehension deficit without word deafness was present; verbal alexia and agraphia in braille were also present. There was no evidence of amusia. He could execute in an exemplary fashion pieces of music for the organ in his repertory as well as improvise. All his musical capabilities: transposition, modulation, harmony, rythm, were preserved. The musical notation in braille remained intact: he could read by touch and play unfamiliar scores, he could also read and sing the musical notes, he could copy and write a score. Nine months after the stroke his aphasia remained unchanged. Nevertheless he composed pieces for the organ which were published. Such data highly suggest the independence of linguistic and musical competences, defined as the analysis and organization of sounds according to the rules of music. This independence in an extremely talented musician leads to a discussion of the role of the right hemisphere in the anatomical-functional processes at the origin of musical competence. The use of braille in which the same constellations of dots correspond either to letters of the alphabet or musical notes supports the independence between language and music.

Peripheral neuropathy in systemic vasculitis: clinical and electrophysiologic study of 22 patients.

Twenty-two patients with evidence of systemic vasculitis and peripheral neuropathy were clinically and electrophysiologically investigated in a retrospective study. Ten had a polyarteritis nodosa, 6 a probable polyarteritis nodosa, and 6 a Churg-Strauss syndrome. Nine patients presented clinically with mononeuropathy or mononeuropathy multiplex considered typical of ischemic involvement of peripheral nerve; nine had more diffuse neuropathy, two of them a symmetric polyneuropathy. EMG examination revealed more diffuse neuropathy than clinically predicted. Findings were of acute or subacute axonal neuropathy.

Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases.

In this consecutive series of 230 patients with neuropathologically verified Creutzfeldt-Jakob disease (CJD), the disease was found to affect men and women with approximately equal frequency in a peak plateau between the ages of 55 and 75 years (mean, 61.5 years). Familial cases accounted for 4 to 8% of the total series. Nonspecific prodromal symptoms occurred in one third of the patients, and the neurological presentation, although usually a gradually evolving mental deterioration, was of rapid onset in 20% of patients and in 36% of patients consisted exclusively of neurological symptoms. The great majority of these symptoms were of cerebellar or visual origin. Extrapyramidal muscular rigidity, myoclonus, and characteristic periodic electroencephalographic (EEG) complexes were observed comparatively late in the illness, and some type of involuntary movement or periodic EEG activity was seen in over 95% of the patients. The median duration of illness was 4 months (mean, 7.6 months); 90% of patients died within a year of onset.

Nerve and muscle microvasculitis in peripheral neuropathy: a remote effect of cancer?

In a series of 50 cases in which nerve and/or muscle microvasculitis was seen on biopsy, seven were associated with malignancy. In two cases, the cancer was found after the discovery of microvasculitis. All patients exhibited sensory-motor neuropathy, which was often painful and asymmetrical, with a progressive course. ESR and CSF protein levels were always elevated. Motor conduction velocity was slightly reduced in three cases, unmeasurable in one case, and normal in three. Cancers involved were adenocarcinoma in five cases (three prostate and two lung), Hodgkin's disease in one and immunoblastic lymphadenopathy in one. A thorough search for cancer should be performed when microvasculitis is seen in nerve or muscle biopsy specimens, especially when ESR and CSF protein levels are elevated.

Duplex scanning of normal vertebral arteries.

Vertebral arteries were studied by Duplex scanning in 50 normal subjects. Pretransverse and C6-C5, C5-C4 intertransverse segments were visualized in all cases on both sides; segment C4-C3 was visualized in 100% of the cases on the right side and in 90% on the left; ostium was obtained in 94% of the cases on the right and in 60% on the left. The left vertebral artery was dominant in 48% of the cases while the right vertebral artery was dominant in 14%. Three vertebral arteries were hypoplasic. Duplex scanning was thus found to be an easily performed noninvasive method to study morphological and hemodynamic characteristics of vertebral arteries from their origin to the C4-C3 level.

Early onset multiple sclerosis. Clinical study of 12 pathologically proven cases.

Among 70 pathologically proven MS cases, we found 12 cases with onset before 25. Only females were affected. There was no positive familial history of MS. In every case the diagnosis was considered during life. Mean duration of the disease was 29.1 +/- 12.5 years. This was significantly longer (p less than 0.001) than in the control group (58 pathologically proven cases with onset after 25). There were no acute courses. Progressive courses (1 case) and remittent courses (5 cases) were less frequent than in the control group (p less than 0.001 and p less than 0.01, respectively). The frequency of remittent-progressive courses (6 cases) was similar in both groups. The length of remittent-progressive courses (30.2 +/- 16.2) and remittent courses (28.6 +/- 9.7) was longer than that of control group (p less than 0.001). The symptomatology of onset was similar in both groups and had no predictive value for prognosis. The overall frequency of signs and symptoms was not statistically different in both groups. For each sign, the percentage of affected patients, throughout the course of the disease, was always smaller in early onset MS than in control group, except for optic neuritis which was as frequent in both groups. However, this was statistically significant only for ocular motricity and nystagmus.

[Creutzfeldt-Jakob disease in France. Value of familial forms. Is there a gene controlling the length of the incubation period?]. / Maladie de Creutzfeldt-Jakob en France. Intérêt des formes familiales. Existe-t-il un gène gouvernant la durée de l'incubation?

An extensive search for patients who died of Creutzfeld-Jakob disease in France between 1968 and 1982 resulted in the discovery of 327 cases, 233 of which were histologically proven and 29 transmitted to animals; 17 patients belonged to 6 families. Further investigations among members of these 6 families yielded 21 additional cases, i.e. a total of 38 familial cases. Studies among sibships suggested an autosomal dominant pattern of transmission but did not exclude lateral contamination infancy. The patients' age at death was 10 to 15 years lower than that of the total of French cases and seemed to be a characteristic of each individual family. This suggests that, as in scrapie, a gene may control the length of the incubation period.

[Creutzfeldt-Jakob disease in the Paris area: ethnic origin of the patients]. / Maladie de Creutzfeldt-Jakob dans l'agglomération parisienne: origine ethnique des patients.

Twenty six (27 p. 100) of 96 patients dying from Creutzfeldt-Jakob disease in the Paris metropolitan area between 1968 and 1982 were born in foreign countries although the proportion of foreign-born residents account for only 15 p. 100 of the population. The annual mortality rate of Creutzfeldt-Jakob's disease in this group of foreign-born cases (1.33 per million) was twice that of French-born residents (0.64 per million). Twenty of the 26 foreign-born cases originated from countries surrounding the Mediterranean sea, and 5 of 7 familial cases belonged to 2 Tunisian families. There was no spatial clustering of foreign-born cases and no relationship between their Paris residence and the percent of foreign-born people living in the same area. The disproportionate frequency of Creutzfeldt-Jakob's disease among foreign-born residents thus seems to be a contributing factor to the higher prevalence of Creutzfeldt-Jakob's disease in Paris when compared to the rest of France. It further suggests that the prevalence of Creutzfeldt-Jakob's disease in Mediterranean countries may be greater than expected.