[Paraplegia associated with intramedullary spinal cord and epidural abcesses, meningitis and spondylodiscitis (Staphylococcus aureus)]. / Paraplégie flasque due à des abcès intramédullaires et épiduraux à staphylocoques dorés. Méningite et spondylodiscite associées.

INTRODUCTION: Intramedullary spinal cord abscesses are rare, frequently associated with meningitis, sometimes with epidural abscesses. They are frequently responsible for paraplegia. Staphylococcus aureus is the predominant organism. MRI shows an intramedullary collection giving a low-intensity signal on T1-weighted images with peripheral contrast uptake on enhanced TI-weighted studies and a high-intensity signal on T2-weighted images with generally extended adjacent medullary edema. They may be multiple. CASE REPORT: We report the case of a man who presented meningitis with intramedullary and epidural abscesses. The number of the lesions did not allow chirurgical drainage. The paraplegia did not resolve despite appropriate antibiotic therapy. CONCLUSION: Appropriate antibiotic therapy and early surgical drainage, if feasible, are key factors for better outcome and prognosis.

[Familial orthochromatic leukodystrophy: clinicopathological study of two cases]. / Leucodystrophies orthochromatiques familiales.

This paper reports the clinico-pathological data in a French family with orthochromatic leukodystrophy. The parents were first cousins and had seven children. Among those, two sisters and one brother presented with neurological signs, with onset around the 5(th) decade, including a dementing syndrome of frontal type, a tetrapyramidal syndrome, seizures, and, in one sibling, a cerebellar syndrome. CT scan or MRI showed diffuse involvement of the white matter. The neurological signs worsened progressively leading to death within 11 and 22 months. Neuropathological examination was performed in two cases. It revealed characteristic orthochromatic leukodystrophy. In one case, the presence of pigmented macrophages and astrocytes was suggestive of Van Bogaert and Nyssen disease. However there were some atypical features including the absence of pigmented cells in the second case whose clinical course was shorter, and the cavitary appearance of the white matter changes with a relative increase in the number of oligodendrocytes raising the issue of a possible link between this condition and cavitary orthochromatic leukodystrophies.

[Cerebral hemosiderosis related to hereditary ceruloplasmin deficiency. Clinical familial case study]. / Hémosidérose cérébrale liée à un déficit héréditaire en céruloplasmine. Etude clinique d'un cas familial.

A 59-year-old patient progressively developed dementia, hallucinations and facial dyskinesia. Brain T and T2-weighted MRI images showed low signal intensity on basal ganglia specially striatum, posterior thalamic and dentate nuclei. He had no evidence of ceruloplasmin and a high level of ferritin in the serum. Liver biopsy confirmed accumulation of iron in the cytoplasm of many hepatocytes. Similar clinical and biological signs were also observed in two brothers. All the three siblings were homozygous for a hereditary ceruloplasmin deficiency. This new clinico-pathological entity, first described in 1987, is different from Wilson's disease, Hallervorden-Spatz's disease and idiopathic hemochromatosis and linked to a mutation of the ceruloplasmin gene located on chromosome 3.

["Polydipsia, intermittent hyponatremia and psychoses" syndrome: a diagnosis and therapeutic management of a case]. / Le syndrome "polydipsie, hyponatrémie intermittence et psychoses": diagnostic et conduite thérapeutique à propos d'un cas.

Polydipsia can be defined as an impulsive behavior leading to absorption of large amounts of water (4 to 20 litres a day), without any underlying organic disease. Its prevalence in a population of chronic psychiatric patients can be as high as 6 to 17%. Schizophrenia represents 80% of cases reported. Some patients with polydipsia may develop hyponatremia, leading to a PIP syndrome (Polydipsia intermittent hyponatremia and psychosis). Hyponatremia or water intoxication appears when three conditions are present: an abnormal regulation of thirst, an inappropriate ADH secretion and/or an excessive renal sensitivity to ADH, with an increased sensitivity of the central nervous system to hyponatremia. The clinician must first identify patients at risk to develop water intoxication and start treatment before any severe physical complication occurs. Pharmacological treatments aiming at an increase of renal free-water excretion--do not show a constant efficacy in the correction of hyponatremia, they have no action on polydipsia. The new atypical neuroleptics such as clozapine and risperidone seem to open new perspectives in the treatment of polydipsia. Controlled studies should be performed in this field.

[Interhemispheric disconnection syndrome caused by partial infarction of the corpus callosum: neuropsychological study and MRI]. / Syndrome de déconnexion interhémisphérique par infarctus partiel du corps calleux: étude neuropsychologique et IRM.

A partial syndrome of hemisphere disconnection was observed in a 63 year-old woman, following an anterior and middle corpus callosum infarct on MRI. Notably, we found left ideomotor apraxia, diagonistic apraxia, left-year extinction on dichotic listening, but no left-hand anomia nor left visual field anomia. A left tactile extinction in dichaptic condition is interpreted as resultant of a dysregulation of the attentional balance between the two hemispheres. This detailed neuropsychological study permits a correlation between the callosal syndrome and the lesion. We suggest that an occlusion of the anterior callosal artery could explain this limited ischemia.

[Subcortical aphasia, and hemidystonia caused by putaminocaudate infarction occurring in early childhood]. / Troubles du langage de type sous-cortical et hémidystonie séquelles d'un infarctus putamino-caudé datant de la première enfance.

A case of subcortical aphasia with hemidystonia in a 14 year-old boy, due to a putaminocaudate infarct having occurred at the age of one is reported. The language disorder was similar to that of adult subcortical aphasia.

[Intramedullary neurenteric cyst without an associated malformation. Case report]. / Kyste neuro-entérique intra-médullaire sans malformation associée. Un cas.

The authors report a case of an intramedullary neurenteric cyst without any associated dysraphic lesion. Unlike extramedullary intradural forms, this type of malformation remains rare. Our observation is one of the first to have been evaluated by magnetic resonance imaging. Theories concerning the embryogenesis of neurenteric cysts as well as their clinical characteristics and surgical treatment are discussed.

[Role of the thalamus in subcortical aphasias]. / Le rôle du thalamus dans les aphasies sous-corticales.

27 cases of patients who presented language disorders of aphasic nature consecutive to a strictly subcortical lesion of vascular origin are reported. From a topographic point of view, the population is divided into 3 groups: thalamic lesions (15 cases), striatal lesions (9 cases), isolated lesions of the white matter (3 cases). The results of the neurolinguistic analysis of the aphasia show a great symptomatological variety. Nevertheless, in spite of this apparent diversity, certain semiologic elements appear to be common to all of the observed linguistic profiles, no matter where the lesion is: hypophonia, non fluent speech, verbal paraphasias, normal repetition, comprehension generally good. A discussion is proposed as to the specific part which certain structures, notably the thalamus, might play in the origin of these various disturbances.

Mononeuritis in acute viral hepatitis.

We describe the cases of 4 adults with acute viral hepatitis A or B in whom mononeuritis affecting a cranial nerve or a nerve of a limb developed. The features of this neuropathy were the following: (a) the prevalence of mononeuritis in patients with acute viral hepatitis was low; (b) this complication developed in the early phase of acute viral hepatitis in most of our patients; (c) the onset of mononeuritis was sudden in most of them; (d) the course of mononeuritis was protracted. Mononeuritis might be the consequence of ischemia resulting from vasculitis.

[Right hemiasomatognosia and sensation of amputation caused by left subcortical lesion. Role of callosal disconnection]. / Hémiasomatognosie droite et sentiment d'amputation par lésion gauche sous-corticale. Rôle de la disconnexion calleuse.

A 72-year-old right-handed hypertensive man presented with a right brachial monoplegia, and hypesthesia of the right half of the body to touch and pricking, sparing the face. A CT scan 2 weeks later showed a spontaneous hyperdense area corresponding to a left subcortical parietal hematoma. The patient used his spontaneous language to express body image disturbances: intense prolonged feeling of amputation related to the upper limb and foot on the right side, hemiasomatognosia without anosognosia, autotopagnosia. Also associated were a right-sided visual negligence and a more general inability to handle spatial data leading to a temporospatial disorientation. Mild language disorders were suggestive of subcortical aphasia: normal incitation and repetition; with semantic paraphasias, poor verbal fluency. Furthermore comprehension of orders or propositions concerning spatial data were poor. Finally, there were signs suggestive of callosal disconnection: paradoxical extinction of the left ear during dichotic listening, agraphia and anomia of the left hand, ideomotor apraxia of the left upper limb, difficulty in visual transfer. The lesion interrupted thalamic tracts to parietal regions and callosal fibers linking parieto-occipital association areas. This twofold lesion was analyzed for each of the neuropsychological disturbances observed. Certain aspect of cerebral function in this patient were reminiscent of "split-brain" disorders. The left hemisphere which "speaks" fails to understand the feelings of the right hemisphere: unfamiliarity of places, sensations of illness, resulting in an unadapted speech. The pathophysiology of feeling of amputation is discussed.

[Cerebromeningeal location of chronic lymphoid leukemia. Rapid immunochemical diagnosis and complete remission by intrathecal chemotherapy]. / Localisation cérébroméningée d'une leucémie lymphoïde chronique. Diagnostic immunochimique rapide et rémission complète par chimiothérapie intrathécale.

Central nervous system (CNS) involvement is a rare complication of chronic lymphoid leukaemia (CLL). The occurrence of lymphocytic meningitis in the course of CLL suggests either CNS involvement by the leukaemic process or infection (especially tuberculosis) related to continuous immunodepression. We report a case of CLL in which leukaemic cells had surface IgM and produced IgM kappa without significant depression of other immunoglobulins. When the patient developed meningitis, measurement of serum and CSF albumin IgG and IgM levels showed that 88% of CSF IgM resulted from local synthesis, while 70% of CSF IgG resulted from serum transsudation. These results suggest that a large number of neuromeningeal lymphoid cells produce the same class of IgM as leukaemic cells and therefore constitute a localization of the disease. Complete remission of the meningitis by intrathecal anti-leukaemic chemotherapy confirmed this hypothesis.

[Anterior choroidal artery syndrome. Neuropsychological study of 4 cases]. / Le syndrome de l'artère choroïdienne antérieure. Etude neuropsychologique de 4 cas.

A neuropsychological study was carried out in 4 cases of infarction in the territory of the anterior choroïdal artery. In 3 cases the lesions were on the right side. A syndrome of the minor hemisphere was present with severe visual neglect, constructional apraxia, alexia due to disorders of visuo-spatial strategy, anosognosia and motor impersistence. In the case with a left-sided lesion there was no neglect but a mild aphasia with impaired fluency, semantic paraphasias, perseveration and a decreased psycho-linguistic ability. The mechanisms of neglect in right-sided lesions are considered.

[Contribution of the right hemisphere to language in aphasic patients. Disappearance of this language after a right-sided lesion]. / Contribution de l'hémisphère droit au langage des aphasiques. Disparition de ce langage après lésion droite.

Assessments vary as to the contribution of the right hemisphere to language in aphasic patients. Results of a clinicopathologic study in two right-handed subjects with aphasia are reported. The lesions involved the territories of the left middle and anterior cerebral arteries. Oral productions in one case, studied during the three weeks of survival, were limited to automatic series, recitation of a fable, and completion of sentences. Follow-up in the other case was possible during 2 years of language rehabilitation. At the end of this period, repetition of an echolalic type was possible; a propositional expression had appeared, reduced to substantives and verb infinitives; denomination was possible but rich in semantic paraphasias. A second infarction in the right sylvian region caused the recovered language to disappear and to be replaced by an abolition of all communication. Proof was thus obtained that the progress accomplished was dependent on the right hemisphere. These findings are discussed in the light of observations of patients following left hemispherectomy and of the capacity of the right hemisphere to generate language as demonstrated in patients after commissurotomy. A dynamic interpretation of the taking over of expression by the right hemisphere during some aphasias is proposed.

[Verbal and nonverbal dichotic listening test. Influence of the hemispheric activation state in normal subjects]. / Test D'écoute dichotique verbal et non verbal. Influence de l'état d'activation hémisphérique chez le sujet normal.

The dichotomic listening test provides data for assessment of cerebral hemisphere activities in response to a given stimulus. Variations in results following verbal and non-verbal dichotomic listening tests to familiar sounds are described, as well as modifications when the activity of one or other hemisphere has been previously altered. Findings demonstrate that voluntary excentration of gaze modifies inter-hemispheric equilibrium and affects the results of the non-verbal dichotomic listening test.

[Echolalic repetition and severe comprehension disorders. Left hemisphere deactivation syndrome? (author's transl)]. / Répétition écholalique et troubles sévères de la compréhension. Syndrome de dé-activation de l'hémisphère gauche?

An aphasic patient presented with suppression of spontaneous speech, correct repetition, and disturbed comprehension and denomination. The association of a lesion on CT scanning affecting the deep regions of the left hemisphere, and extinction of the right ear to dichotic audition, leads to a discussion of the role of the right hemisphere in remaining psycholinguistic capabilities.

[Visual agnosia without right hemianopia in a right-handed patient (author's transl)]. / Agnosie visuelle sans hémianopsie droite chez un sujet droitier.

A right-handed 77-year-old man presented with a left homonymous lateral hemianopsia involving the macula. Consciousness, memory, and oral language were normal, but the patient experienced great difficulties in identifying objects and images by sight, even though he recognized their geometrical shapes. He also had prosopagnosia, and alexia without agraphia but no agnosia for colours. The patient died suddenly 6 weeks after the onset of the disorders. Pathological examination demonstrated the presence of a bilateral occipital infarct involving on the right, the inferior border of the calcarine fissure, and the lingual and fusiform gyri, and on the left the fusiform gyrus. This supports the role in man of the inferior occipitotemporal convolutions in the perceptual integration of visual informations, and suggests that this region of the cortex is analogous with the inferotemporal cortex of monkeys which has been shown to be a determinant for the elaboration of visual objects.

[Hemorrhage of the head of the left caudate nucleus: disorganization of speech and graphic expression, and disturbances in gestures (author's transl)]. / Hémorragie de la tête du noyau caudé gauche. Désorganisation du discours et de l'expression graphique, perturbations des séries gestuelles.

A 72 year-old woman was found to have hemorrhage limited to the head of the left caudate nucleus. Clinical symptomatology consisted of disturbances in oral, written, and graphic expression. There were no difficulties in comprehension, dysarthria, or phonemic paraphrasing, but speech and graphic expression were incoherent. Perseverance of ideas, semantic inconsistencies, inability to keep a stable objective during speech and graphic activity, are the principal characteristics of a disorder which is related to a prelinguistic stage, and illustrate the role of the caudate nucleus in the selection processes which are a preliminary step for speech organization.