A 47-year-old female developed proximal limb weakness after hysterectomy for uterine fibromatosis. Muscle strength slowly improved, but relapse occurred at age 52. She presented with progressive gait difficulty: proximal limb weakness, and painful calves. Family history was not contributory Cranial nerves, deep tendon reflexes, and sensation were normal. Serum creatine kinase was normal. An IgG kappa monoclonal protein was found Nerve conduction studies were normal, bur EMG showed brief small polyphasic motor unit action potentials with early recruitment in proximal muscles. Muscle biopsy showed abundant rods, atroptric muscle fibres, and type 1 fibre predominance. The sarcolemma was immunoreactive for IgG kappa. Plasmapheresis was unsuccessful, but methylprednisolone and azathioprine led To moderate improvement of muscle strength, associated with reduced monoclonal protein levels. This is rite third case report, describing the association of monoclonal gammopathy and late-onset nemaline myopathy presence of a monoclonal protein at the sarcolemma and responsiveness to immunosuppressive treatment ore suggestive of a dysimmune origin.

A patient with unilateral, painless, chronic progressive upper limb sensorimotor deficit showed electrophysiological evidence of a focal demyelinating neuropathy with almost complete conduction block across the brachial plexus. Magnetic resonance imaging disclosed marked brachial plexus hypertrophy. Intravenous immunoglobulin led to fast and complete recovery, maintained by intermittent perfusions. Hypertrophic brachial plexus neuropathy can be a presentation of focal chronic inflammatory demyelinating polyradiculoneuropathy. Objective and quantitative assessment of hand function is useful to evaluate treatment results and to optimize treatment regimens.

We report the case of a 64-year-old man with an acute isolated and prolonged transient amnesic state. The underlying aetiology remained obscure. Brain magnetic resonance examination demonstrated only non-specific slight and transitory signal intensity abnormalities within the left hippocampus on T2-weighted images. Electroencephalographic studies showed repetitive periodical and prolonged bursts of rhythmic theta activity, originating from the left fronto-temporal region, but no typical epileptiform discharges. As an epileptic origin could not be excluded, anti-epileptic treatment was administered. Subsequently, electroencephalographic abnormalities disappeared completely after a few days. On follow-up, repetitive clinical and neuropsychological comprehensive evaluations showed that the mnesic capacities improved slowly and gradually. The gradual improvement concerned time and place orientation first of all and was followed thereafter by improvement of explicit memory and the capacity to learn new information. Clinically, at this stage, the patient appeared appropriately orientated, according to the medical staff and his family, although memory tests remained abnormal. The memory deficit lasted -3 months but was followed by complete recovery. In our discussion, we consider possible aetiologies for transient amnesia with emphasis on an epileptic as opposed to a vascular aetiology by focal hippocampal ischaemia.

The idiopathic hypereosinophilic syndrome (HES) is a rare disease, characterised by persistent eosinophilia (> 1500/mm3), without underlying cause, provoking multiple organ system injury. Morbidity and mortality are mostly associated with the HES cardiopathy. Neurological signs are also frequent. Neurological dysfunction can be central (encephalopathy, organic psycho-syndrome) and peripheral (polyneuropathy, mononeuropathia multiplex, autonomic neuropathy, polymyositis). The encephalopathy is not always caused by distant thrombo-embolic events originating from the HES cardiopathy. We describe a patient with idiopathic HES central nervous system dysfunction, in the absence of cardiopathy. Furthermore we briefly discuss pathophysiological aspects, treatment modalities and the prognosis of the HES, in relation to our patient.

The case of a 19-year old patient suffering of transient metamorphopsia restricted to familiar faces and familiar objects is reported. This clinical sign resulted from a small right occipitotemporal haemorrhage due to a sub-cortical metastasis. The patient claimed that faces are distorted and look more pleasant. There were neither visual field defects nor visual agnosia. MRI revealed a small high signal area in the right fusiform gyrus. The structural and functional aspects of the metamorphopsia are documented and discussed in relation to aperceptive prosopagnosia. More specifically, it is suggested that facial metamorphopsia and aperceptive prosopagnosia express the same underlying disorder differing only in terms of severity.

The case of a 19-year old patient suffering of transient metamorphopsia restricted to familiar faces and familiar objects is reported. This clinical sign resulted from a small right occipitotemporal haemorrhage due to a sub-cortical metastasis. The patient claimed that faces are distorted and look more pleasant. There were neither visual field defects nor visual agnosia. MRI revealed a small high signal area in the right fusiform gyrus. The structural and functional aspects of the metamorphopsia are documented and discussed in relation to aperceptive prosopagnosia. More specifically, it is suggested that facial metamorphopsia and aperceptive prosopagnosia express the same underlying disorder differing only in terms of severity.

The case of a 19-year old patient suffering of transient metamorphopsia restricted to familiar faces and familiar objects is reported. This clinical sign resulted from a small right occipitotemporal haemorrhage due to a sub-cortical metastasis. The patient claimed that faces are distorded and look more pleasant. There were neither visual field defects nor visual agnosia. MRI revealed a small high signal area in the right fusiform gyrus. The structural and functional aspects of the metamorphopsia are documented and discussed in relation to aperceptive prosopagnosia. More specifically, it is suggested that facial metamorphopsia and aperceptive prosopagnosia express the same underlying disorder differing only in terms of severity.

Median nerve somatosensory evoked potentials (SEPs) and jerk-locked EEG back-averaging (JLA) were performed in 2 patients with left-hand myoclonus. Both SEPs were characterized by a normal parietal N20 and an enhanced N27-P27 fronto-parietal complex, but the giant central N40 described by most authors was only observed in case 1. Similarily, the aspect of JLA clearly differed in both patients. We hypothetize that both patients exhibited signs of an increased drive of the motor cortex by inputs coming from the sensory cortex. This increased drive could be mediated by the cortico-cortical connexions from areas 1-3 to area 4. In contrast, we suggest that signs of primary hyperexcitability of the motor cortex were only present in case 1, as suggested by the presence of an enhanced N40 in SEPs and the results of JLA studies.

In human brain, [3H]glibenclamide binds with high affinity (KD about 3.5 nM) to sulfonylurea binding sites which are associated with ATP-sensitive potassium (KATP) channels. Regarding to the important neuromodulatory action of KATP channels in some neuronal populations, sulfonylurea binding sites were measured in several cortical areas (frontal and temporal cortex, hippocampus) and striatum (caudate nucleus and putamen) in controls and patients with Parkinson's disease or progressive supranuclear palsy. There was no modification of [3H]glibenclamide specific binding in the cerebral regions studied in both pathologies. These results indicate that KATP channels do not seem to be involved in the pathophysiology of these degenerative processes. Brain samples from five patients with Huntington's disease were studied. A small decrease in sulfonylurea binding sites was measured in the frontal cortex, caudate nucleus and putamen which could be due to the loss of either neurons or nerve endings. This low decrease contrasts with the dramatic diminution of many other markers associated with the profound striatal degeneration occurring in Huntington's disease.

We developed a highly sensitive and specific immunoaffinity-mediated capillary blot technique for the detection of oligoclonal IgM bands in CSF and sera from patients with various neurological disorders. Pre-treatment of the samples by dithiothreitol was necessary to obtain a migration of the IgM molecule into the gel of isoelectric focusing. IgM was then transferred by immunoaffinity onto a polyvinylidene difluoride sheet previously coated with anti-IgM antiserum. The limit of detection was found to be 6 ng in 15-μl samples. The presence of CSF-restricted IgM bands was considered the result of an intrathecal synthesis and was observed in 13 out of 46 (28%) patients with MS, more frequently in acute relapses (9 out of 21, 43%). including 6 cases out of 13 presenting the first bout of the disease. Similar IgM bands were also detected in 15 out of 46 (38%) patients with CNS infections, especially in cases of neurosyphilis and neuroborreliosis. The presence of CSF oligoclonal IgM bands was linked to an increase of the IgM index in the MS group, but not in the group of infectious diseases as a whole. The occurrence of CSF-restricted oligoclonal IgM bands seems to be the most specific indicator of an intrathecal synthesis of this isotype.

A highly sensitive antigen-mediated capillary blot technique was developed for the detection of virus-specific oligoclonal IgG in paired CSF and serum samples from patients with various neurological diseases. In multiple sclerosis, intrathecal synthesis of oligoclonal antibodies was present against measles (70%), rubella (60%), varicella zoster (40%) and mumps (30%); in most cases (75%), such synthesis involved two or more viruses. In contrast, antibodies against a non-neurotropic virus (cytomegalovirus) were rarely produced in CSF from MS patients (5%). However, this 'polyspecific' reaction was not restricted to MS samples but was also observed in neurolupus and in the late phase of infectious diseases of the central nervous system. These anti-viral antibodies could be produced without de novo replication of the corresponding viral genome and are likely mere bystanders of an ongoing immune response.

An intrathecal synthesis of IgA has been reported in various neurological disorders. However, the frequency of its occurrence and the electrophoretic characteristics of the locally produced IgA remained a matter of controversy. We developed a sensitive immunoaffinity-mediated capillary blot technique for the detection of polyclonal and oligoclonal IgA in the CSF of 115 patients with various neurological disorders. Paired CSF and serum samples containing 50 ng IgA after appropriate dilutions were submitted;to isoelectric focusing in agarose gels; IgA was then blotted onto a polyvinylidene difluoride sheet coated by an anti-IgA antiserum or by infectious antigens. The immunoblots were revealed by an alkaline phosphatase-conjugated anti-IgA antiserum. Only five samples displayed CSF-restricted oligoclonal IgA bands, including two out of 33 from MS patients. In herpetic encephalitis (n = 5) and varicella-zoster meningitis (n = 2), a strong intrathecal production of virus-specific IgA antibodies was detectable. In such cases, faint oligoclonal IgA antibodies were superimposed on a polyclonal background. A weak local production of anti-Borrelia burgdorferi IgA antibodies was present in two out of four cases of neuroborreliosis.

The serum and CSF of two women with a severe subacute pancerebellar syndrome contained high titers of antibodies directed against antigens present in the perikaryon of Purkinje cells. In Western blots performed on human Purkinje cells extracts these antibodies reacted with two groups of proteins the molecular weights of which were estimated to 34-38 and 62 kilodaltons (anti-Yo antibodies). Complementary investigations revealed a tubar adenocarcinoma in the first case, an ovarian carcinoma in the second. The tubar tumoral cells also contained the protein of the highest molecular weight. Compared to the serum, the CSF contained a higher proportion of anti-Yo antibodies per mg IgG, and a fraction of the latter was likely synthesized intrathecally. These antibodies may be involved in the almost total disappearance of the Purkinje cells, as observed at autopsy of both cases.