Value of multiple sclerosis diagnostic criteria. 70 autopsy-confirmed cases.

We have evaluated the sensitivity of the most recent and most frequently used criteria for the diagnosis of definite multiple sclerosis by the retrospective study of the clinical files of 70 pathologically confirmed cases. For each case, the date of diagnosis was determined separately using different sets of criteria. The delay of diagnosis was then calculated. The diagnosis was made significantly earlier when certain criteria were used. This was more marked in the earlier years of the disease. We also found that cases of multiple sclerosis with progressive courses were diagnosed later than cases with other courses, whatever the criteria used. This was statistically significant only for a single criterion.

Miller Fisher syndrome: clinical and electrophysiologic evidence of peripheral origin in 10 cases.

Clinical and electrophysiologic data recorded in patients with Fisher syndrome characterized by ophthalmoplegia, ataxia, and areflexia are presented. Cases with limb weakness or pleocytosis in the CSF were excluded, according to Fisher. Ten patients were selected. All had hand and foot numbness. A large amount of protein without cell reaction was found during the third week of illness in serial CSF examinations. EMGs showed very slight limb involvement without spontaneous activity, and in most cases, facial muscles exhibited a denervation pattern. Distal motor nerve conduction velocity on limbs and F wave latencies were normal, whereas the sensory nerve action potentials were altered in all but one case when tested (seven out of eight cases). By means of blink reflex study performed in four patients, no significant pattern of brainstem dysfunction was discovered. The authors discuss the preeminent role of peripheral nerve lesions with regard to the ataxia and ophthalmoplegia.

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.

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.

Chorea and polycythaemia.

Two patients with chorea and polycythaemia vera are described. The literature on this rare association is reviewed and its pathophysiology discussed.

[An experimental study of the psychostimulant action of amantadine]. / Etude expérimentale de l'action psycho-stimulante de l'amantadine

According to previous experimental and clinical data, we have tested the hypothetic psycho-stimulating activity of amantadine on experimental sensitized models. We obtained a strong action on the spontaneous sleep of rats at a dose of 10 mg/kg and a less striking action in diminishing the effects of barbitol on the righting reflex of mice and in reducing the narcosis of rats induced by mebubarbital. This action does not seem to be due to a release of catecholamine because the lesions produced by electrocoagulation in the ascending aminergic pathways or pretreatment by alpha-methylparatyrosine (AMPT) do not block it. It is suggested that there may be a non-aminergic receptor of amantadine which is different from the amphetamine receptor.

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.

Peroneal muscular atrophy. Part 1. Clinical and electrophysiological study.

144 patients with the clinical syndrome of peroneal muscular atrophy or Charcot-Marie-Tooth disease were studied. Thirteen were recognized as the spinal form of the disease since all had normal motor nerve conduction velocity and sensory nerve action potential. The remaining patients could be classified according to their values of motor conduction velocity for the median nerve. Two main groups were therefore identified: 55 patients whose nerve conduction was below 30 m/s belonged to group I, which corresponds to the previously reported hypertrophic form or hereditary motor sensory neuropathy (HMSN) type I. Sixty-four patients whose nerve conduction was above 40 m/s belonged to group II which corresponds in the majority of cases to the neuronal form or HMSN type II. Twelve patients could not be classified since the motor nerve conduction velocity for median nerve ranged between 30 and 40 m/s. These could belong to either of the two main groups or might form an intermediate group, the existence of which is discussed. Clinical genetic and electrophysiological features of the two main groups are discussed and compared.

[Recurrent peripheral nerve palsies and concomitant chronic neuropathy. Neuromusclar electrophysiologic study and neuropathologic examination: apropos of 5 cases]. / Paralysies tronculaires récidivantes et neuropathie chronique concomitante. Etude électrique neuromusculaire et examen neuropathologique: a propos de 5 observations

The authors report 5 personal cases (3 in the same family) of patients suffering from recurrent peripheral nerve palsies usually induced by pressure and review the relevant literature. There is evidence in such cases of a mild, chronic, diffuse, subclinical neuropathy which can be recognized by electrophysiological examination. Examination of peripheral nerve biopsy material in such cases shows evidence of both axonal and Schwann cell abnormalities with evidence of neurogenic atrophy of skeletal muscles. This chronic subclinical neuropathy which is often familial can be distinguished from other chronic primary neuropathies with or without myelin sheath proliferation and hypertrophy.

Postmortem studies on posthypoxic and post-methyl bromide intoxication: case reports.

In two cases of action myoclonus following hypoxic or shock encephalopathy, neuropathological examination disclosed mild or moderate scattered changes involving thalamus, griseum centrale mesencephali, and nucleus centralis superior. Other areas were affected only in one of these cases (striatum, nucleus subthalamicus or hippocampus, nuclei pontis, and cerebellar cortex). In another case (an alcoholic patient), the changes, which involved only corpus mamillare and thalamus, were those of Wernicke-Korsakoff encephalopathy. In one case of oscillatory myoclonus following septic shock, there were marked cerebellar changes involving deep nuclei and mild abnormalities in the thalamus and inferior olive. The last case of action myoclonus following acute methyl bromide intoxication was characterized by marked changes in the inferior colliculi and moderate or mild abnormalities of thalamus, griseum centrale mesencephali, nucleus centralis superior, nucleus reticularis tegmenti pontis, nuclei pontis, and dentatus. The findings are compared with the data of seven previously reported neuropathological examinations in action myoclonus following hypoxic encephalopathy.

[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.

[Bilateral isolated trigeminal neuropathy (author's transl)]. / Neuropathie isolée du trijumeau. Forme bilatérale.

Spillane and Wells (1959) were the first to describe the syndrome known as isolated trigeminal neuropathy in which deficit in the trigeminal nerve occurs either unilaterally or bilaterally without evidence of any local lesion. It may be a truly isolated disorder but can also be the predominant element of a more diffuse neuropathy provoking, more particularly, ageusia and anosmia. The bilateral forms are fairly frequently related to connective tissue diseases but many are of unknown aetiology. A case is reported of the bilateral form with ageusia and anosmia, cubital deficit, and slowing of the conduction rate in the limbs. No aetiology was found.

[Isolated sensory deficiency caused by infarction in the region of the anterior choroidal artery]. / Déficit sensitif isolé par infarctus dans le territoire de l'artère choroïdienne antérieure.

A patient presented with a pure left sensory deficit of sudden onset. CT scan showed a small capsular infarct in the anterior choroidal artery territory involving the superior thalamic peduncle in the posterior limb of the internal capsule.

[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.

[Frequency and symptomatology of disorders of micturition during cerebrovascular accidents. Interest of complementary examinations (author's transl)]. / Fréquence et séméiologie des troubles mictionnels au cours des accidents vasculaires cérébraux. Intérêt des examens complémentaires.

A total of 228 patients with cerebrovascular accidents have been studied since 1975. Micturition disorders were present in 124 cases (54 p. 100), including total incontinence in 45 p. 100, imperative micturition in 37 p. 100, and complete retention in 18 p. 100 of the patients. They are associated in a significant manner with severe psycho-intellectual disorders, an extensive hemiplegia, bilateral pyramidal signs, and ruological abnormalities. Useful complementary examinations and some therapeutic modalities are briefly reviewed.

[2 cases of paralysis of the posterior branch of the radial nerve due to compression of the nerve by a lipoma]. / 2 cas de paralysie de la branche postérieure du nerf radial due a la compression du nerf par un lipome

The authors report two cases of paralysis of the posterior branch of the radial nerve as a result of compression of the nerve by a benign tumour, in both instances a lipoma. Analysis of the sixteen cases appearing in the literature reveals that the symptoms and signs are constant, characterised by the progressive development of paralysis of the muscles supplied by the deep branch of the radial nerve with the appearance of a tumefaction around the lateral part of the elbow. X-rays of the elbow confirm the diagnosis in the presence of a radiotranslucent polylobulated image. Surgical excision is followed by regression and the disappearance of a-l neurological signs after an average period of 8 months. In addition to a lipoma, compression may be caused by repeated contusion of the nerve by the border of the superficial part of the short supinator muscle in association with "tennis elbow". Section of this part of the muscle border results in complete recovery.

[Huntington's chorea and cerebellar atrophy. Apropos of amanatomo-clinical case]. / Chorée de Huntington et atrophie cérébelleuse. (A propos d'une observation anatomo-clinique)

The authors report the clinical and pathological findings in a 46 years old woman with Huntington's chorea and cerebello-olivar atrophy. Eight previously reported cerebellar atrophies in Huntington's chorea with pathological examination are reviewed. The primary or secondary nature of the cerebellar lesions is discussed.

[Multiple intracranial arterial aneurysm of mycotic appearance. Repeated vascular embolic accidents. Myxoma of the left atrium]. / Anévrysms artériels multiples intracraniens d'sspect mycotique. Accidents vasculaires répétitifs d'allure embolique. Myxome de l'oreillette gauche

The authors describe a case of myxoma of the left auricle disclosed by repeated cerebral embolism and multiple aneuryms of mycotic appearance of the cerebral arteries in a woman exhibiting no cardiac symptoms. The immediate post-operative results were excellent. In the light of 7 similar cases in the literature, diagnostic and prognostic points are analysed.

[Necrotic myelopathies and neoplastic pathologie. Three clinico-pathological cases (author's transl)]. / Myélopathies nécrosantes et pathologie néoplasique. Trois observations anatomo-cliniques.

Three clinico-pathological cases of necrotic myelopathies with a distant malignancy are presented. Two cases had a lymphosarcoma and one case a prostatic carcinoma. They were compared to 13 well studied other cases collected in the literature. These myelopathies were related to solid visceral tumours in 8 cases and to lymphomas in 5 cases. The disease could be individualized on clinical grounds (flaccid paraplegia with bladder and bowell incontinence and sensory loss without clear-cut upper boundary developing over a few weeks with normal CSF and fast impairement of general condition), and, on pathological features. It is characterized by one or several spinal cord necrosis areas, often asymetrical, involving mostly white matter, without any vascular topography. Axons are involved as well as myelin sheats. There is mild inflammation and no specific vascular alteration. There is no metastases in the cord, meninges, vertebral column or nerve root. No vascular occlusion is found. The mechanism of the disease is unknown. The frequent occurence of lymphomas could suggest the presence of immunopathological factors.

[Supranuclear lateral gaze palsy of pontine origin. Report of 2 clinicopathologic cases with electrooculographic and electromyographic data]. / Paralysies supranucléaires de la latéralité d'origine protubérantielle. A propos de deux observations anatomo-cliniques avec enregistrements électro-oculographiques et électromyographiques.

Electro-oculographic, electromyographic and pathological findings in two cases of supranuclear lateral gaze palsy of pontine origin have allowed us to define the clinical and physiopathologic features of the Pontine Reticular Formation (PRF) syndrome, and to formulate hypothesis about the terminal portion of the Occipito-Pontine Tract (OPT) involved in horizontal pursuit eye movements. The unilateral PRF syndrome is characterized by abnormal eye movements in the direction of the lesion. In the ipsilateral hemifield of movement there is paralysis of all movements from midline to extreme ipsilateral side, except oculo-cephalic reflex which remains intact (dissociated palsy); in the contralateral hemifield all saccades from extreme contralateral side to midline are suppressed, and this constitutes a specific abnormality of the PRF syndrome: quick phase of optokinetic and vestibular nystagmus are absent, while voluntary gaze is preserved but remarkably slow. It is suggested that this last fact is due to simple disfacilitation arising from undamaged PRF. Electromyographic findings suggest that the division between excitatory and inhibitory fibers of descending supranuclear oculomotor tracts ending in the abducens nuclei probably occurs in the lower pans. Anatomopathologic findings in the two cases show that the OPT runs in front of the Medial Longitudinal Fasciculus or in the lateral tegmentum and that it decussates, at least once, below the upper pons.