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The authors have analyzed clinico-neuropathologically nine cases of the definite sporadic form of Creutzfeldt-Jakob disease (CJD). All cases were female, with mean age of 62.7 years. Eighty-nine percent of the patients exhibited prodromal and initial psychiatric symptoms; definite signs of dementia, and myoclonus were present in 100% of cases. The EEG was abnormal in all cases and pseudoperiodic paroxysms were present in 56% of the patients. Their evolution time ranged from 3 to 19 months. Neuropathologically, brain and cerebellar atrophy, spongiosis, astrocytosis and neuronal loss were present in 100% of the patients. In 5 (56%) of these 9 cases, prion protein (PrP) amyloid plaques were detected in the cerebellum, by optical- and electronmicroscopy. There was a positive correlation between the number of plaques and the evolution time. The authors outline the similarities of their cases in the elderly with the new variant of CJD described in young people. Os autores analisaram, do ponto de vista clínico e neuropatológico, nove casos da forma esporádica definida da doença de Creutzfeldt-Jakob (DCJ). Todos eles eram mulheres, com idade média de 62,7 anos. Oitenta e nove por cento dos pacientes exibiram sintomas psiquiátricos prodrômicos e iniciais; sinais típicos de demência e mioclonias estavam presentes em 100% deles. O EEG foi anormal em todos os casos e apresentou paroxismos pseudoperiódicos em 56% dos pacientes. O tempo de evolução da doença variou de 3 a 19 meses. Do ponto de vista neuropatológico, atrofia cerebral e cerebelar, espongiose, astrocitose e perda neuronal estavam presentes em 100% dos pacientes. Em 5 (56%) dos 9 casos, foi evidenciada, por microscopia óptica e eletrônica, a presença de placas amilóides de proteína prion (PrP) no cerebelo. Havia correlação positiva entre o número de placas e o tempo de evolução da doença. Os autores salientam as semelhanças desses seus casos de pacientes idosos com a nova variante de DCJ descrita em pessoas jovens.

Background and Purpose Cerebral infarction and prolonged regional hypoperfusion have been described in patients with transient ischemic attacks (TIAs). The aim of this study was to compare the sensitivity of single-photon emission CT (SPECT) with that of brain CT and to evaluate the clinical significance of differentiation of TIA patients with or without focal hypoperfusion. Methods From a hospital-based population, we studied the SPECT and CT findings in 76 consecutive patients, without a stroke history, who presented with TIA in the carotid artery territory. The recorded variables were the time of SPECT imaging ( Results The overall SPECT sensitivity was 36% (27/76). When brain CT and SPECT were performed in the same patients, the SPECT sensitivity was significantly higher than that of CT (19/59 [32%] versus 8/59 [14%]; P =.007). The SPECT sensitivity was not dependent on the time of investigation, duration of attacks, history of TIA(s), or the clinical presentation. The vascular risk and etiologic factors were not significantly different between the patients with or without prolonged focal hypoperfusion. Logistic regression did not identify any variable to discriminate the two groups. Conclusions Despite its better sensitivity compared with CT, SPECT performed without the acetazolamide test provides no additional clinically useful information on the vascular risk factors and etiology in TIA patients.

Paracentral homonymous hemianopia is a rare form of homonymous visual field defect. Vascular disorders in the occipital cortex are the most common cause of homonymous scotomas. The authors report a case of left homonymous paracentral scotoma associated with a left facial palsy, a left arm paresis and moderate confusion. Six months later a sectorial optic atrophy appeared. The neurophysiological evaluation with visual evoked potentials and pattern-electroretinogram, showed defective paramacular afferences with repercussion on the retinal ganglion cells. Magnetic resonance imaging (MRI) showed an ischaemic lesion in the right parathalamic region involving the lateral geniculate nucleus and/or the optic tract; the lesion extended into the anterior limb of the internal capsule, explaining the motor deficits. The MRI did not disclose lesions in the posterior visual pathway nor in the occipital cortex. The pathophysiologic mechanism is discussed.

As beta-2 transferrin is almost specific to the cerebrospinal fluid, its detection in rhinorrhea or otorrhea fluids demonstrates the occurrence of CSF fistula. We describe a highly sensitive method based on immunoaffinity-mediated capillary blotting for the detection of this cathodic isoform of transferrin in minute amounts (3 microliters) of rhinorrhea or otorrhea fluids. Application of this method in a series of 10 patients with CSF fistula is reported.

Two sexually active female patients presented with acute meningitis. The CSF abnormalities were severe and persistent. In spite of the absence of genital lesions, serological studies revealed a primary infection by herpes simplex virus type 2. An immunoblot study revealed intrathecal synthesis of anti-herpes antibodies early in the course of the disease.

Using data from Belgian neuropathological archives, completed with the results of a comprehensive study of available medical records, we found 100 patients who fulfilled diagnostic criteria for probable or definite Creutzfeldt-Jakob d1551isease (CJD). Mean age at death was 63 years. The median disease duration was 9 months. Progressive mental deterioration was present in all cases, whereas signs of cerebellar dysfunction and myoclonus were found in approximately 80% of the patients. In 50% of the population, the EEG revealed characteristic abnormalities. Ninety-six patients suffered from the sporadic type of CJD, while 4 suffered from a hereditary form. In our series, we could find no evidence for the new variant, neither for an iatrogenic cause.

Five patients with clinical features of corticobasal degeneration (CBD) were studied with PET imaging. The main clinical findings included a unilateral extrapyramidal motor disorder, without significant response to levodopa, as well as clumsiness, dysarthria, apraxia and a clear asymmetry of neurological signs. PET studies with 18F-labeled 2-deoxy-2-fluoro-D-glucose disclosed mainly a significant hypometabolism in the thalamus and motor cortex controlateral to the more affected limbs. Additional relationships between individual clinical signs and PET data were also found. We concluded that PET findings supported the clinical diagnosis of CBD, although the specific pattern related to this condition needs to be more precisely defined. Further studies are especially needed to correlate clinical data and PET results with pathological examination.

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, but EMG showed brief small polyphasic motor unit action potentials with early recruitment in proximal muscles. Muscle biopsy showed abundant rods, atrophic 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 the 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 are suggestive of a dys-immune origin.

Using data from Belgian neuropathological archives, completed with the results of a comprehensive study of available medical records, we found 100 patients who fulfilled diagnostic criteria for probable or definite Creutzfeldt-Jakob d1551isease (CJD). Mean age at death was 63 years. The median disease duration was 9 months. Progressive mental deterioration was present in all cases, whereas signs of cerebellar dysfunction and myoclonus were found in approximately 80% of the patients. In 50% of the population, the EEG revealed characteristic abnormalities. Ninety-six patients suffered from the sporadic type of CJD, while 4 suffered from a hereditary form. In our series, we could find no evidence for the new variant, neither for an iatrogenic cause.

Serum and cerebrospinal fluid (CSF) of 13 patients have been examined to confirm and precise the diagnosis of herpes simplex virus encephalitis (HSVE). By amplifying the DNA with a nested polymerase chain reaction (PCR), we could demonstrate the herpetic origin of these cases of encephalitis. DNA of HSV type 1 or type 2 was directly identified and differentiated, by the use of both type-specific primes in the same reaction. The primer sequences were chosen in the glycoprotein D region for HSV type 1, and in the glycoprotein G region for HSV type 2. Only one case was due to the latter. In all but one cases, an immunoaffinity-mediated capillary blotting study was also performed. This technique showed the occurrence of oligoclonal CSF-specific IgG bands, while the antigen-driven immunoblotting demonstrated intrathecal production of oligoclonal anti HSV antibodies. In most of the cases, repeated CSF analysis allowed us to study the sequential detection of viral DNA and of intrathecal synthesis of virus-specific IgG in relation to the clinical course. All the patients were treated with acyclovir. In one case, a relapse was clinically suspected, but the PCR remained negative.

The authors describe the clinical and biological data of seven patients with anti-Hu antibodies. Six of them displayed a small cell lung carcinoma (SCLC), but no cancer was detected in the 7th patient in spite of an extensive workup. The clinical heterogeneity of the anti-Hu syndrome is emphasized. The major symptoms were linked to a severe sensory neuropathy in three cases, to cerebellitis in two cases, to dysautonomia in one case, and to gastro-intestinal pseudo-obstruction in one case. One patient also displayed EMG abnormalities characteristic of the Lambert-Eaton myasthenic syndrome. Two patients developed opsoclonus or ocular flutter associated with severe confusion in the late stage of their disease. In four patients, the neurological signs and symptoms preceded the discovery of the SCLC, and in two cases the initial detection of anti-Hu antibodies prompted the successful search for this tumor. Immunopathological events injuring the peripheral and central nervous system are briefly discussed.

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.

To assess the potential of thymidine for imaging brain tumors, 20 patients with untreated (n = 14) and recurrent (n = 6) supratentorial intracranial tumors were studied with PET by using 2-[11C]thymidine (Tdr), and the results were compared with [18F]fluorodeoxyglucose (FDG) PET data.Blood analysis consistently revealed a rapid clearance of the intact Tdr together with the appearance of CO2/HCO3- that, with time, accounted for approximately 70% of the total blood activity. As soon as 10 min after tracer injection, brain images showed a low and homogeneous Tdr distribution over the normal brain structures (cortex-to-blood ratio approximately 1). Visual and quantitative analysis revealed increased Tdr uptake (tumor-to-cortex ratioor = 1.2) in 11 of 14 untreated tumors and in 5 of 6 recurrent tumors. No correlation was found between Tdr uptake and tumor grade. In 12 of the 14 untreated tumors, FDG uptake was low (tumor-to-cortex ratio: 0.83 +/- 0.79), but a FDG hot spot was visualized in 8 of 10 high-grade and in none of the 4 low-grade tumors. FDG uptake was consistently low in recurrent tumors (tumor-to-cortex ratio: 0.49 +/- 0.19), and PET-FDG was negative in 3 of the 6 cases.These data indicate the feasibility of brain tumor imaging with Tdr and suggest the potential clinical usefulness of the method in the detection of tumor recurrences. The specificity of the method remains, however, to be investigated.

Using positron emission tomography (PET), thirteen studies of regional brain glucose utilization were performed in 12 patients with postanoxic syndrome due to cardiac arrest. Investigations were carried out at least one month after brain anoxia. Seven subjects were in a persistent vegetative state. The others had regained normal consciousness with various residual neurological signs. When compared with normal values obtained in 16 normal, age-matched subjects, mean cerebral glucose metabolism was drastically decreased (+/- 50%) in vegetative cases, and to a lesser degree (+/- 25%) in conscious subjects. The most consistent regional alterations were observed in the parieto-occipital cortex (9 cases), the frontier between vertebral and carotid arterial territories. Other selective anomalies were found in the frontomesial junction (5 cases), the striatum (3 cases with dystonia), and the visual cortex (2 cases with cortical blindness). This study suggests that cerebral anoxia results in a global brain hypometabolism, which appears related to the vigilance state, as well as in regional disturbances preferentially located in arterial border zones. Although our findings remain to be confirmed in larger series, they suggest that PET provides a useful index of residual brain tissue function after anoxia and may assist in the monitoring of postanoxic encephalopathies.

A 21-year-old woman developed hyperemesis gravidarum, complicated by Wernicke's encephalopathy and central pontine myelinolysis. For the latter, time course of the lesions on magnetic resonance imaging suggests a pathogenetic role for hypophosphatemia rather than for hyponatremia.

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.

We describe an affinity-mediated capillary blotting technique for the detection of free kappa or lambda light chains in native cerebrospinal fluid (CSF) after isoelectric focusing in agarose gel. Interferences by light chains bound to immunoglobulins were carefully excluded. An absolute amount of 20-50 ng of free kappa or lambda Bence-Jones proteins were detectable by this method, under the form of several discrete bands with isoelectric points between 5 and 8.5. No free light chains were observed in CSF and sera from patients without neurological disorders (n = 26). Such bands were present in most CSF samples in the case of central nervous system (CNS) infections, except in aseptic meningitis. In a group of 48 multiple sclerosis (MS) patients, 44 (92%) displayed oligoclonal free kappa bands restricted to the CSF; oligoclonal IgG bands were observed in 40 cases, and oligoclonal free lambda bands in 33. In this group, the presence of CSF free light chain bands was highly correlated with their absolute levels (p less than 0.001). In other neurological diseases (n = 44), oligoclonal free kappa and free lambda bands were detected much more rarely, in seven (16%) and four (9%) cases respectively. Surprisingly, the CSF from three unrelated patients with Huntington's disease (out of five tested) contained both oligoclonal IgG and free kappa bands.

Cerebrospinal fluid (CSF) and serum samples from eight patients with bacteriologically proven (6) or clinically suspected (2) tuberculous meningitis were tested for the presence of anti-mycobacterial IgG antibodies by an affinity-mediated immunoblot technique. This technique is based on agarose gel isoelectric focusing of paired CSF and serum samples diluted to the same IgG concentration, and transfer of the specific IgG antibodies onto mycobacterial antigen-loaded nitrocellulose sheets. An intrathecal synthesis of anti-mycobacterial oligoclonal IgG antibodies, often superimposed on diffuse polyclonal production was shown in all patients but not in patients with tension headache or other neurological disorders. Similar results were obtained when a purified mycobacterial antigen, A60, was used for coating the nitrocellulose sheets in place of a whole mycobacterial homogenate, indicating that A60 was a major immunogen. The number of anti-mycobacterial oligoclonal IgG bands increased with time, and persisted for years even in clinically cured patients. Some IgG bands had no detectable anti-mycobacterial activity, at least with the antigens preparations used in this study. The demonstration of such anti-mycobacterial IgG bands in the CSF could be a useful adjunct for the diagnosis of tuberculous meningitis, especially in the case of negative cultures.

We report four patients with Behçet's disease characterized by initial and predominant neurological signs and symptoms. In three cases, a clinical picture of relapsing meningoencephalitis preceded the appearance of the classical signs of the disease for several months or years; in the fourth case, an acute febrile aseptic meningitis coincided with the development of bipolar aphthosis and uveitis. Disease activity was linked to a blood inflammatory syndrome and neutrophilic leucocytosis. Acute phases were associated with CSF mixed pleocytosis and high protein content. Brain CT scans and MRI were very effective to detect lesions which are mainly located in the brain stem and basal ganglia. High-dose corticosteroids and, in cases of relapses, immunosuppressive drugs were required to treat these severe forms of Behçet's disease.

CSF and serum samples from 16 AIDS patients were tested for the presence of anti-HIV antibodies either by classical serological methods or by an immunoblot technique based on agarose gel isoelectric focusing and transfer of the specific IgG antibodies onto HIV antigens-loaded nitrocellulose sheets. This method enabled the demonstration of an intrathecal synthesis of anti-HIV oligoclonal IgG antibodies, often superimposed on diffuse polyclonal production, in 14 patients. The two negative cases were devoid of neurological signs or symptoms. However, two patients classified in stage II of the disease (asymptomatic infection) displayed an intrathecal synthesis of anti-HIV antibodies.

Clinical features of 18 patients with either lymphocytic meningoradiculitis (n = 17) or chronic encephalomyelitis (n = 1) due to Borrelia burgdorferi are reported. Arthropod bites were recorded in only seven patients. High titres of anti B burgdorferi antibodies were detected in sera from all patients and in CSF from 12 out of 17 patients tested. All patients had CSF abnormalities especially pleocytosis and oligoclonal bands. A falsely positive syphilitic serology was observed in the CSF from the patient with encephalomyelitis.

Free kappa and lambda light chains were assayed by particle-counting immunoassay in cerebrospinal fluid (CSF) from patients with various neurological disorders. Detection limits were 25 and 50 ng/ml, respectively. Values of free kappa chain were higher than 50 ng/ml (upper reference limit) in 155 of 191 (81%) multiple sclerosis (MS) patients, in 100 of 168 (60%) patients with central nervous system (CNS) infections but in 41 of 217 (19%) patients with other neurological disorders. Free kappa chains were also assayed in 273 matched sera. The mean concentration in the control group (1.58 micrograms/ml; SD: 0.41) did not differ significantly from those in MS sera (1.63 micrograms/ml; SD: 0.43). The free kappa chain index was increased in 86% of MS patients and in 40% of patients with CNS infections. Regarding free lambda chains, CSF values were higher than 240 ng/ml (upper reference limit) in most neurological disorders (50-100%). However, the use of a lambda chain index increased the specificity of the assay as this index was higher than the upper reference value in 86% of MS patients and in only 23% of patients with infectious diseases. In MS, high levels of free kappa and lambda indices correlated significantly (P less than 0.01) with either the presence of oligoclonal bands or a high IgG index. Local synthesis of free light chains is an additional marker of an ongoing immune response within the CNS, especially in MS.

[3H]GBR 12935 bound with high affinity to dopamine uptake sites in rat striatum where a close parallelism was observed between the subcellular localization profiles for [3H]dopamine uptake and [3H]GBR 12935 specific binding. Using the same ligand, we characterized the dopamine uptake sites in human striatum: the mean KD value was 3.2 nM and the specific binding was inhibited by several dopamine uptake blockers but with slightly lower affinities than those observed in the rat. The subcellular localization profile revealed a synaptosomal enrichment of the specific binding in human striatum. [3H]GBR 12935 binding was decreased in the putamen and caudate nucleus of subjects with Parkinson's disease (33 and 46% of control values, respectively) and progressive supranuclear palsy (38 and 57% of control values, respectively). It is very unlikely that the remaining binding sites in both diseases correspond to piperazine acceptor sites that are not involved in dopamine uptake. However, we cannot exclude the possibility that some of these remaining dopamine transporter sites are not functional, since the reduction in [3H]GBR 12935 specific binding was less marked than the decrease in the dopamine content of the same areas.

S100 protein (S100) was assayed by particle counting immunoassay in serum samples from 50 healthy individuals, 325 patients with various neurological disorders, and 20 patients with malignant melanoma. The detection limit for this protein was 0.3 microgram/L. We detected none in healthy individuals or in 50 patients with multiple sclerosis, 23 with dementia, or 20 with meningitis. S100 was detectable in serum of only a few patients with meningoradiculitis (2/20), peripheral neuropathy (2/30), encephalitis (1/14), Guillain-Barré syndrome (1/25), or AIDS (2/20). In contrast, we observed high concentrations in 29 of 75 patients with tumors of the central nervous system, especially in meningioma (6/9), glioblastoma (9/23), and neurinoma (5/5). Values for S100 were mainly abnormally high (greater than 0.3 microgram/L) in serum from patients with cerebrovascular disorders (43/48) or with metastases of melanoma (9/11).

Twenty-seven "subdural" effusions in infants were treated, according to our previously suggested method: a simple external drainage, followed ater one week by an internal peritoneal shunt, if the effusion did not dry up by this time. Daily electrophoresis of the fluid obtained by external drainage has been performed in 20 cases, either until the disappearance of the effusion or until the peritoneal shunting after 7 days. In 8 persistent and in 5 rapidly disappearing effusions, cerebro-spinal fluid was immediately present or appeared during the first week, simultaneously with a lowering of the total protein content. In the other 7 rapidly disappearing effusions, the protein content remained high and a conspicuous plasmatic transsudation was disclosed; the possibility that cerebro-spinal fluid could be present even in some of those last cases is discussed. The fact that some effusions display, from the onset, the characteristics of cerebro-spinal fluid, leads to the question of their subarachnoid rather than subdural localisation. For this reason, we prefer to use the less committed denomination of pericerebral effusions, in which we may distinguish subdural effusions, with or without cerebro-spinal fluid, and subarachnoid effusions. Nevertheless, it is not always easy to establish the pathogenic character of an effusion revealed by CT-scan. Pressure measurements by fontanometry appear to be a useful examination for that purpose.

A young female was diagnosed as having X-linked muscular dystrophy of the Duchenne type. Chromosome studies, including trypsin-Giemsa banding, Quinacrine fluorescence, and nucleolus organizer region (NOR) silver staining revealed an X-autosome reciprocal translocation t(X;21) (p21;p12). Utilizing both [3H] thymidine autoradiography and the BrdU-Hoechst 33258-Giemsa technique, lymphocytes and fibroblasts were found to show a preferential inactivation of the normal X suggesting the presence of a single mutant gene on the translocated X. This patient is one of seven reported cases of an X-linked muscular dystrophy associated with an X-autosome translocation. In all seven cases the exchange point in the X chromosome is in band p21 at or near the site of the Duchenne gene.

The authors report ten cases of normal pressure hydrocephalus treated by ventricular drainage. They pay particular attention to the neuropsychological and psychometric aspects and endeavour to identify the most characteristic nosological features by comparing their own clinical observations with those reported in the literature.

After a lapse of not less than two years the authors study the functional and clinical evolution of 62 patients presenting myelopathy in cervical arthrosis treated by surgery. A scoring system for evaluating the functional deficit of the patients is used for quantitative assessment of the disorders. Surgery brought a frank improvement in 38 patients: it left the sensorimotor deficits of 19 patients unchanged. The handicap continued to deteriorate in three patients. Two patients died from non-neurological disorders. The data are compared with those in the literature which indicate an improvement rate of the same order of magnitude. Within certain limits, age and the degree of initial handicap do not seem to influence the long-term result of surgery. However, the time lapse between the appearance of the first signs of deficiency and surgery seem to constitute an important prognostic factor. The shorter the time lapse, the better the result.

We studied ten cases of disseminated sensory neuropathy. Both sexes were similarly involved. Except in one case, the first symptoms appeared between ages 20 and 50. The clinical disorders, their evolution and similar cases previously reported suggest and autonomous benign disease. The deficits resulted from a selective involvement of superficial cutaneous nervous fibers and appeared in different cutaneous areas. Some territories were more frequently affected (nervus peroneus, sural, radialis, cutaneous femoralis lateralis). Tinel's sign was often present. Patients sometimes described a sensation of electric discharge when elongating nerve trunks. In half of the cases the attacks of dysesthesia or of sensory loss followed one another within less than one year. The deficits were fully reversible in a third of the cases after a mean total evolution of 4 to 8 years. Blood and cerebrospinal fluid were always normal. Electromyography and motor conduction velocities were normal. Sensory conduction velocities were uncommonly decreased. On the other hand, the aspect of somesthetic potentials which were evoked from cutaneous areas involved by the disease were modified in most cases. Pathological examination of nerve biopsies which were performed in 5 cases showed non specific axo-myelinic degeneration in 3 cases and a severe sclerosis of the perineurium in one case. The etiopathogenesis of the disease is discussed.

A patient with infarct in the region of the right anterior choroidal artery presented neuropsychological signs corresponding to a minor hemisphere syndrome and disorders of horizontal oculomotricity similar to those obtained in unilateral parietal-occipital lesions. An anatomic interpretation of the latter finding is attempted.

Fetuin, an alpha 1-glycoprotein (MW : 48 kD) was firstly isolated from bovine foetal serum (4). Dziegielewska et al. (1) recently gave evidence that a fetuin-like antigen (FLA) was present in early human foetal samples but not later in gestation. We have shown that FLA was not detectable in normal human sera (< 5 μg)l) but was present at a concentration ranging from 10 to 80 μg/1 in a few disorders in which the immune System was involved and in sera of patients with tumours derived from the neutral crest or with nephroblastoma (2). In neurological disorders, FLA was found in sera from patients with multiple sclerosis (MS), Guillain-Barre Syndrome, neurosyphilis and peripheral, mainly diabetic, neuropathies, but was rarely detected in cerebrospinal fluid (2).

Diffuse multicentric sarcomas of the brain, also called cerebral malignant lymphomas, sometimes present symmetrical topography. This is well demonstrated by a CAT mirror image obtained by the authors in the reported case. The diagnostic value of the image is underlined in view of the possible forms of therapy, in particular radiation therapy, applicable to this type of tumour. (Acta neurol. belg., 1980, 80, 86-94).

Cultured neurons from embryonic rat brain display central type benzodiazepine receptors characterized by high-affinity binding of [3H]flunitrazepam which is allosterically enhanced in the presence of gamma-aminobutyric acid (GABA). A 48 h treatment of the cultured neurons with 1 microM diazepam, 0.1 microM clonazepam or 0.1 microM beta-carboline ester derivatives did not change either Bmax or KD values of the [3H]flunitrazepam specific binding. A 48 h incubation in the presence of GABA (1 mM) or muscimol (0.1 mM) induced a 30% decrease of the Bmax value of [3H]flunitrazepam specific binding without change of the KD value. The down-regulation was dependent on GABA concentrations and temperature, and was partially inhibited by bicuculline but not by the benzodiazepine antagonist Ro 15-1788. The other subunits of the benzodiazepine-GABA-chloride channel receptor complex also seemed to be down-regulated by GABA since there was a decrease of the specific binding of [3H]muscimol and [35S]t-butylbicyclophosphorothionate (TBPS) to the GABAA and chloride channel sites respectively. The GABA-induced down-regulation of the GABA-benzodiazepine receptor seems to be selective since the specific binding of ligands to other receptors was not affected. Our results suggests that activation of the low-affinity GABA subunit which is involved in cellular electrophysiological responses, induced the receptor down-regulation.

Meningoradiculitis due to tick-bite is rare and poorly documented. Its etiology remains hypothetical, but is thought to be caused by a viral infection carried by the tick. It is characterized by a striking clinical picture, especially symptoms of a subjective nature. The involved area extends asymmetrically from the site of the bite with variable latency. The CSF is characteristic, showing definite lymphocytosis and a moderate protein reaction. The prognosis is excellent although recovery may take several months. In the event of facial paralysis, there is a risk of postparalytic spasms. Treatment is symptomatic; no drug has proved effective.

We report two patients with chronic encephalomyelitis due to Borrelia burgdorferi in whom the definite diagnosis was delayed because of atypical clinical features. The first patient presented with chronic spastic paraparesis, slight ataxia and nystagmus of several years' duration. A tentative diagnosis of multiple sclerosis was made in spite of important abnormalities of the CSF biological characteristics. The second patient presented with an acute aphasia and a bilateral Babinski's sign. He was thought to suffer from benign herpetic meningoencephalitis. Several months later, as the patient experienced relapses with cerebellar and spinal cord involvement, falsely positive tests for syphilis were found and an antibiotic treatment was given. High protein content, low glucose levels, pleocytosis and oligoclonal bands were observed in all CSF samples, but the definite diagnosis was based on the detection of serum and CSF antibodies against B. burgdorferi.

The authors describe various applications of an immunoblot technique which allows the qualitative determination of the specific antibody activity of oligoclonal IgG intrathecally synthesized in infectious diseases of the nervous system. After dilution of sera to the same IgG concentration as the paired CSF samples, 10 microliters of both fluids are applied side by side on agarose gel plates and isoelectrically focused. Precipitated IgG or specific IgG antibodies are then blotted onto a nitrocellulose sheet previously coated with either a rabbit anti-IgG antiserum or the antigen under study, respectively. The immunoblot is successively incubated with biotinylated anti-IgG antiserum and with the streptavidin-biotin-peroxidase complex before staining with 4 chloro-1-naphthol. This method was applied to samples from patients with subacute sclerosing panencephalitis, herpetic encephalitis, meningoradiculitis due to Herpes Zoster, neuro-AIDS, neurobrucellosis, meningoradiculitis or encephalomyelitis due to Borrelia burgdorferi, and tuberculous meningitis. In each case, specific oligoclonal IgG antibodies, superimposed or not on a diffuse polyclonal synthesis were detected in the CSF, but not, or more faintly, in the corresponding serum. This was taken as evidence for an intra-thecal synthesis of these antibodies. In contrast, when a "mirror effect" was observed, i.e. similar oligoclonal bands in both serum and CSF after dilution at the same IgG concentration, an intra-thecal synthesis was ruled out.

The authors discuss internuclear ophthalmoplegia associated with non-paralytic pontine exotropia of the contralateral eye. Impairment of the posterior oculogyric tract in its protuberantial segment, resulting in oculographic signs of alteration in the contralateral continuation. Seems to be responsible for the exotropia.

The authors describe three patients with a chronic sensory-motor polyneuropathy associated with a monoclonal IgM gammopathy. In each case, the M-component was shown to display an antibody activity against the Myelin-Associated Glycoprotein (MAG) in a western blot technique. Sera from the three patients stained specifically the myelin sheaths of normal human peripheral nerves after incubation with rabbit anti-IgM. Motor conduction velocities were very low in each case. Morphological studies of the sural nerve from one patient revealed a moderate loss of myelinated fibres, some onion-bulbs but also a microangiopathy and signs of axonal degeneration.

The level of C-reactive protein (CRP) was determined in the cerebrospinal fluid (CSF) by particle counting immunoassay. In non-neurological patients (N = 24), CRP was detectable only in 10 samples at concentrations ranging from 1.5 to 37 micrograms/l. The multiple sclerosis group did not differ from the controls. The highest CRP levels were found in viral and bacterial, including tuberculous, infections of the nervous system, with overlapping results for the various types of infections. However, in serum, the levels of CRP were much higher in pyogenic than in viral meningitis. We compared the CSF CRP/serum CRP ratio to the same ratio for albumin and found a significant correlation between the two ratios in viral, but not in bacterial, infections. These results suggest a local consumption of CRP during bacterial meningitis.