C. Laterre

A Case of Prosopagnosia With Some Preserved Covert Remembrance of Familiar Faces

This paper presents the detailed analysis of a case of prosopagnosia in a 54-year-old male farmer following bioccipital vascular disease. In-depth clinical investigations confirmed the diagnosis of prosopagnosia and revealed the absence of any associated defect, except for a slight aspecific disturbance of the short-term memory. Further study of this case indicated that the trouble was not concerned with the class of complex visual stimuli, was not even concerned with facial expressions or unknown faces, was not a perceptual defect, but was related mainly to the operation of individualization. The memory hypothesis was thus retained and supported. Moreover, exploration of the difficulty indicated that the deficiency was limited to defective access to conscious information concerning faces and information associated with these faces (name, context, etc.), effectively stored in memory.

GABA induces down-regulation of the benzodiazepine-GABA receptor complex in the rat cultured neurons.

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.

Comparative correlations of HMPAO SPECT indices, neurological score, and stroke subtypes with clinical outcome in acute carotid infarcts.

BACKGROUND AND PURPOSE The prognostic value of single-photon emission computed tomography (SPECT) remains controversial. The aim of this study was to compare the prognostic value of stroke severity, stroke subtypes, and SPECT indices and to determine which predictive factors have an independent effect on clinical outcome. METHODS We studied 55 consecutive patients with acute (< 12 hours) carotid infarct within 36 hours of symptom onset with SPECT. Clinical presentation was assessed by the Canadian Neurological Scale and stroke subtypes. SPECT indices were the degree and size of hypoperfusion and crossed cerebellar diaschisis as assessed by a semiquantitative analysis. Outcome was evaluated by the functional status and mortality (Rankin Scale score at 1 month). RESULTS The Rankin Scale score correlated with the degree (r = .580; P < .00001) and size (r = .616; P < .00001) of hypoperfusion. The mean degree and size of hypoperfusion were significantly higher in patients with poor outcome. Crossed cerebellar diaschisis had no significant predictive value. Statistical analysis determined threshold values for the Canadian Neurological Scale score and the degree and size of hypoperfusion for the functional status and mortality. The degree and size of hypoperfusion had no higher performance than the Canadian Neurological Scale score. The negative predictive value was excellent for both clinical and SPECT indices. Multivariate analysis selected only the size of hypoperfusion as an independent predictor for the functional status (P = .004) and the Canadian Neurological Scale score for mortality (P = .009). CONCLUSIONS SPECT performed within 36 hours of onset predicts clinical outcome, but different clinical and SPECT indices with threshold values should be chosen according to the relevant outcome end point.

Free kappa and lambda light chain levels in the cerebrospinal fluid of patients with multiple sclerosis and other neurological diseases

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.

Intrathecal synthesis of anti-mycobacterial antibodies in patients with tuberculous meningitis. An immunoblotting study.

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.

Persisting Perfusion Defect in Transient Ischemic Attacks A New Clinically Useful Subgroup

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 (<36 or > or = 36 hours), clinical presentation, history of previous TIA(s), duration of the presenting attack (<2 or > or = 2 hours), vascular risk factors, and etiology. We used both visual and semiquantitative analyses for the SPECT evaluation. Acetazolamide challenge was not performed. 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.

Highly sensitive detection of beta-2 transferrin in rhinorrhea and otorrhea as a marker for cerebrospinal fluid (C.S.F.) leakage

SummaryAs 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 μl) of rhinorrhea or otorrhea fluids. Application of this method in a series of 10 patients with CSF fistula is reported.

Lymphocytic meningoradiculitis and encephalomyelitis due to Borrelia burgdorferi: a clinical and serological study of 18 cases.

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.

Anti-HIV antibodies in the CSF of AIDS patients: a serological and immunoblotting study.

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.

Contribution of PET studies in diagnosis of corticobasal degeneration

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.