Efficiency of cognitive control recruitment in the very early stage of multiple sclerosis: a one-year fMRI follow-up study.

Functional magnetic resonance imaging (FMRI) studies have established that patients with multiple sclerosis show stronger activation in the lateral prefrontal cortices (LPFC) than healthy control subjects during effortful cognitive tasks. The aim of the present study was to assess the impact of these activation changes on cognitive performances. In addition to 19 controls, who were tested at a single time-point to define a standard pattern of fMRI activation during the performance of the Paced Auditory Serial Addition Task (PASAT), 13 patients with clinically isolated syndrome underwent a longitudinal fMRI examination while performing the PASAT at the beginning of the study (M0) and one year later (M12). Relative to the M0 scores, PASAT performances improved in eight patients (group A) and either decreased (n = 4) or remained unchanged (n = 1) (group B) in five patients at M12. Random effect analyses (SPM2; Wellcome Institute, London, England) were performed to compare intra-group time-related effects on brain activation (paired t-test between M0 and M12), and inter-group differences were also compared between the two groups of patients (analysis of covariance with PASAT performances as the covariate). Relative to group B, group A showed larger increase in activation between M0 and M12 in the right LPFC. In the whole group of patients, interaction analyses showed that the differences in the PASAT scores between M0 and M12 were correlated with the differences in activation observed in the right LPFC. This longitudinal study shows that in patients with early multiple sclerosis, the increased levels of activation in the right LPFC was associated with improved individual working memory and processing speed performances.

A longitudinal study of callosal atrophy and interhemispheric dysfunction in relapsing-remitting multiple sclerosis.

OBJECTIVES: To determine if callosal atrophy and interhemispheric dysfunction can be detected in the early stages of relapsing-remitting multiple sclerosis (MS) and to evaluate their progression in relation to the disability and evolution of lesions seen on magnetic resonance imaging during a 5-year period. METHODS: We compared 30 patients who had clinically definite early-onset replasing-remitting MS and mild disability with control subjects. Regional and segmental callosal size and extent of white matter abnormalities on magnetic resonance imaging, as well as performance on tasks exploring interhemispheric transfer of motor, auditory, and sensory information were assessed. Patients with MS were evaluated at baseline and after 5 years. Physical disability was determined at both times using the Expanded Disability Status Scale score. RESULTS: Patients with MS were seen with significant callosal atrophy and functional impairment of interhemispheric transfer at baseline that worsened during the 5-year study. A significant correlation was found between the magnitude of disability and the severity of morphological and functional callosal involvement at baseline. This association persisted at year 5. Baseline clinical characteristics such as age and prestudy relapse rate were unrelated to callosal size or interhemispheric performance. However, the number of baseline T2-weighted lesions was correlated with callosal involvement and this relation persisted at year 5. CONCLUSION: Patients who had relapsing-remitting MS in the early stages of the disease and mild disability had significant callosal involvement that progressed over time. The relationship between disability, T2-weighted lesions load, and degree of morphological and functional callosal impairment confirm the potential value of using callosal dysfunction as a surrogate marker of disease progression in MS.

[Angiodysplasia of moya-moya type disclosed by choreic unvoluntary abnormal movements during oral contraception. Apropos of 2 cases]. / Angiodysplasie de type Moya-Moya révélée par des mouvements anormaux involontaires choréiformes au cours d'une contraception orale. A propos de 2 cas.

Two seventeen year-old women, developed acute onset left choreic movements following two months and two weeks use of oral contraceptives. Left hemiparesia appeared a few days later, while involuntary movements discontinued. Cranial CT scan and MRI showed bilateral ischemic lesion in the frontal region for the first case and isolated lesion in the right centrum ovale for the second. Angiography showed nearly complete obstruction of the terminal portion of the internal carotid artery with an outline Moya-Moya network. After discontinuing oral contraceptives, there has been no relapse of neurologic dysfunction for more than three years for the first case and twelve months for the second one. The role of perfusion insufficiency in limb-shaking carotid transient ischemic attack is discussed and the possible relations between oral contraceptives, chorea and angiographic features resembling Moya-Moya disease are evaluated.

Gordona terrae central nervous system infection in an immunocompetent patient.

The bacterial genus Gordona includes seven species of mycolic acid-containing actinomycetes which are cultured from environmental sources and rarely from clinical samples. They have been implicated in primary pulmonary diseases, nosocomial wound infections, and central nervous system infections in two immunocompromised patients. We isolated Gordona terrae from the cerebrospinal fluid sample of an immunocompetent patient with meningitis and multiple brain abscesses and detected Gordona terrae DNA in the abscesses. The outcome was good at the 4-year follow-up, after prolonged treatment with trimethoprim-sulfamethoxazole. Phenotypic identification of this isolate was confirmed by analysis of the 16S rRNA gene sequence, which shared 100% homology with that of G. terrae reference strains. Physicians and clinical microbiologists must be aware of the occurrence of Gordona species infection not only among immunocompromised patients but among all patients. Accurate identification of Gordona species may be accomplished by molecular techniques.

[Acute myelopathies in young patients and multiple sclerosis. prospective study of 20 cases]. / Myélopathies aiguës du sujet jeune et sclérose en plaques. Etude prospective de 20 cas.

The risk of progression to multiple sclerosis (MS) after an episode of acute non compressive episode involving the spinal cord remains uncertain. A follow-up study was performed to determine the risk of early progression to MS in 20 patients presenting with clinically isolated lesions of the spinal cord, combined clinical evaluation, spinal and brain magnetic resonance imaging (MRI), visual, brainstem auditory and somatosensory evoked potentials (VEPs, BAEPs, SEPs), and cerebrospinal fluid (CSF) electrophoresis analysis. Spinal cord MRI demonstrated more lesions in cervical region (74 p. 100) than thoracic or lumbar regions (26 p. 100). Six patients (30 p. 100) had an initial brain MRI that was strongly suggestive of MS and 5 patients (25 p. 100) had only one MS-like abnormality. Eight patients (40 p. 100) had abnormal VEPs, 3 (15 p. 100) abnormal BAEPSs and only 44 p. 100 (8/18) abnormal SEPs. In contrast, CSF analysis showed oligoclonal bands (CSFOB) in 15/19 patients (79 p. 100). The diagnosis of MS was performed initially in 13 cases (65 p. 100) (clinically definite MS (CDMS) in 30 p. 100, laboratory-supported definite MS (LSDMS) in 61 p. 100 and clinically probable (CPMS) in one case). During the follow-up period (18 +/- 7 months), 8 patients (40 p. 100) presented one or more exacerbations and time to the first recurrence was 8 +/- 5 months. Seven of these 8 patients were initially treated by infusion of methylprednisolone. Among these patients, all of them had CSF OB and initial brain MRI was strongly suggestive of MS in 3 of them. During this follow-up period, brain MRI showed emergence of lesions in 4 cases with normal initial examination and 3 of them presented exacerbations. At the follow-up term, the diagnosis of MS was performed in 15 cases (75 p. 100) CDMs in 66 p. 100, LSDMS in 26 p. 100 and CPMS in one case). This confirms the predictive value of brain MRI and CSF OB for the diagnosis of MS in patients who present with clinically isolated acute syndrome of the spinal cord.

[Spinal dural fistula with peri-medullar venous drainage]. / Les fistules durales rachidiennes à drainage veineux périmédullaire.

Clinical and neuroradiological findings of 8 patients with a spinal dural arteriovenous fistula are reviewed. Disturbance of micturition or defecation and weakness of the legs were always present and the most frequent initial symptom was a progressive spastic paraparesis. Duration of symptoms before diagnosis was 2 years. Lumbar puncture showed elevation of proteins and myelography demonstrated dilated perimedullar posterior veins. In every case, magnetic resonance imaging of the spinal cord (T2- weighted images) revealed intramedullary high signal intensity of the conus medullaris and selective angiography confirmed the site of the dural fistula. Each patient was treated with endovascular method consisting in liquid adhesive embolization (0.2 cc of N-butyl cyanoacrylate) with hyperselective catheterism of the dorsospinal artery. Embolization procedure was successful in 6 cases with large improvement of leg weakness and partial regression of disturbed micturition and defecation. The pathophysiological mechanisms explaining the clinical signs are discussed.

[Ptosis and asthenia manifesting a mitochondrial myopathy]. / Ptosis et asthénie révélateurs d'une myopathie mitochondriale.

We report a case of mitochondrial myopathy discovered in a 55-year old woman who was being investigated for the cause of her asthenia. Physical examination showed ptosis of the upper eyelid and proximal muscle deficit. Histological examination of a muscle biopsy disclosed rare fibres with mitochondrial aggregates. Biochemical exploration of muscle tissue revealed a double enzyme deficit involving complexes I and IV of the respiratory chain. Clinical improvement was obtained after the patient was put on coenzyme Q10. We conclude that a diagnosis of mitochondrial myopathy must be considered in patients, including middle-aged adults, presenting with muscular asthenia.

[Amnesic syndromes]. / Les syndromes amnésiques.

Amnesic syndromes are rare, but they constitute very useful models to study the neurobiological substrates of human memory. The causes and semiological formulae of these syndromes are varied, but they are all characterized by anterograde amnesia combined with a retrograde amnesic disorder and by elective damage of episodic and declarative memories, the semantic and procedural memories, as well as intelligence, being usually spared. Amnesic syndromes are divided into two main types: the simplest one, type 1, is thought to be a defective storage, whilst the more complex type 2 is regarded as defective encoding associated with disturbances in retrieval dependent on more diffuse meso-diencephalic, septal, hypothalamic and sometimes frontal lesions. The lesional substrate of amnesic syndromes comprises the hippocampo-mammilo-thalamo-cingulum axial complex and its connections with the mesencephalon and the basal forebrain.

[Acute psychiatric pathology disclosing subcortical lesion in neuro-AIDS]. / Pathologie psychiatrique aiguë révélatrice d'une lésion sous-corticale au cours d'un neuro-SIDA.

Maccario et al. described, in 1987, the case of an HIV-positive patient whose psychotic symptomatology was the expression of right centrum semi-ovale lesion. We report the case of a patient who suffered a sudden delirium, expression of a probable right lenticular cerebral toxoplasmosis. This 35-year-old male homosexual, who had no psychiatric history, suddenly developed in November 1988 the following psychiatric signs: he started to walk back and forth incessantly, he had the impression that he was the subject of the conversations of the passers-by, that all the posters and notices refer to him, and that he was God. He was admitted in a psychiatric department where the symptoms were progressively curbed by neuroleptics (cyamemazine 75 mg, and haloperidol 15 mg). The episode was not questioned by the patient, but attributed to bad eating habits. HIV-positivity had been discovered a year later (during systematic screening). A computerized tomographic (CT) scan performed subsequently to this delirium was interpreted as normal. Four weeks later the patient was referred to us. The psychiatric condition was stabilized in spite of a certain aggressiveness and the probable persistence of an underlying delirium state. Laboratory examinations showed the following: blood count revealed leukopenia (2.2 G/l) and thrombocytopenia (135 G/l; OKT4/OKT8 ratio was 0.08; CSF: normal; sputum culture evidenced the presence of pneumocystis carinii; EEG were normal. Neuropsychological symptoms concerned in particular a lack of concentration during the different tests with a definitive wavering of attention. Lexical retrieval was poor whatever the topic proposed.(ABSTRACT TRUNCATED AT 250 WORDS)

Transient global amnesia followed by vertebrobasilar ischemia in a case of dolichoectatic basilar artery.

A 59-year-old hypertensive patient presented with transient global amnesia without neurological signs or symptoms. During the following 3 years, he experienced several attacks of vertebrobasilar insufficiency and a cerebellar infarction. CT scan and MRI found a partially thrombosed dolichoectatic basilar artery but no significant lesion within the limbic system. These data suggest transient global amnesia was due to a transient ischemia in the basilar artery distribution.

[Binswanger's subcortical encephalopathy. Study of a case with predominant left hemispheric lesion (author's transl)]. / Encéphalopathie sous-corticale de Binswanger. Etude d'un cas comportant une atteinte hémisphérique gauche nettement prédominante.

We report the clinical and pathological study of a patient with vascular leucoencephalopathy almost restricted to the left posterior hemisphere. A 65 year--old right handed hypertensive woman has presented with a neuropsychological symptomatology of transcortical sensorial aphasia. We discuss the following points: 1) the patient's disease must be considered as Binswanger's subcortical encephalopathy despite the asymetric topography of the lesions ; 2) particular hemodynamic cerebral factors has possibly determined the asymetric nature of the disease ; 3) a lesion of the left temporo-parieto-occipital area that was strictly sub-cortical, with complete destruction of the posterior part of the arcuate fasciculus has presented with the clinical picture of a transcortical sensorial aphasia.

[NEOLOGISMS AND MOTOR APHASIA. (Afferent pathology?)]. / Néologismes et aphasie motrice. (Pathologie de l'afférence)

The authors describe a case of post-traumatic aphasia in a 39-year-old man. The linguistic disturbances were marked by the unusual association of oral expression consisting mainly of neologisms, normal comprehension and almost normal written expression. A physiological interpretation based on disturbance of treatment of the sensory afferent nerves from the bucco-phonator organs is suggested to account for the neologisms in this particular form of aphasia.

[Tardive spino-cerebellar degeneration with amyotrophia, complicating a severe case of pallido-luysian degeneration and diffuse histological lesions of senility. (Anatomo-clinical study of a case with nosographic discussion)]. / Dégénérescence spino-cérébelleuse tardive avec amyotrophies, comportant une atteinte pallido-luysienne sévère et des lésions histologiques diffuses de sénilité. (Etude anatomo-clinique d'un cas avec discussion nosographique)

Anatomo-clinical findings are reported in a case of central nervous system degeneration which began at age 47 and progressed over a three year period. Information obtained prior to the patient's death suggested probable spinocerebellar degeneration with amyotrophies. Postmortem anatomical examination confirmed this diagnosis but also revealed the existence of unsuspected lesions. These clinically non-manifested lesions involved severe pallido-luysian degeneration as well as numerous and diffuse senile plaques. The nosological implications of this case are discussed.