[Unconjugated pteridines and neuromeningeal infections]. / Ptéridines non conjuguées et infections neuro-méningées.

Concentrations of unconjugated pteridines (neopterin, monapterin, biopterin, pterin) were measured in the cerebrospinal fluid (CSF) of 310 patients, using a high performance liquid chromatography (HPCL) method. Our cohort included 209 controls (C), 15 patients with meningism (M), 22 with viral meningitis (VM), 17 with bacterial meningitis (BM), 9 with herpetic meningoencephalitis (HME), 2 with tuberculous meningoencephalitis (TME) and 36 with peripheral systemic infections (PI). These measurements, expressed as nmol/litre, showed a gradation of neopterin concentrations according to the type of infection: 20.1 + 6.5 in group C; 46.9 +/- 29.9 in group PI; 274.3 +/- 231.7 in group VM; 699.2 +/- 711.2 in group BM, 1,101.9 +/- 1,107.9 in group HME and 1,169 +/- 1,171.9 in group TME. There was no such gradation with biopterin. Comparisons of means showed that total concentrations in the pathology groups were very different from those observed in controls and in the neuromeningeal infections of the PI group. There was no correlation between the number of lymphocytes and the concentrations of neopterin or biopterin in the CSF. It is concluded that the concentration of neopterin in the CSF is a sensitive but little specific marker of infection, independent of CSF cellular reaction. Measuring this concentration makes it possible: 1) to evaluate the status of immune defences; 2) to predict that a meningitis will become chronic, and 3) to detect a possible parenchymal participation in a meningeal infection.

[Internal carotid occlusion and essential thrombocythemia. 2 cases]. / Occlusion carotidienne interne et thrombocythémie essentielle. 2 cas.

We report 2 cases of occlusion of the internal carotid artery in young, non-atherosclerotic patients with essential thrombocythemia (ET). This complication of ET seems to be rare, but is probably underdiagnosed since transient ischemia, which is frequent in ET patients, is seldom explored by angiography. The excess of thrombocytes and the thrombopathy may account for the microvascular complications, but in the absence of any other contributory factor, occlusions of large arteries, such as the ICA, is more difficult to explain.

[Hemiballismus. Hemichorea. Striatal infarction]. / Hémiballisme. Hémichorée. Infarctus striatal.

Lesions of other structures than corpus Luysii, may rarely cause hemiballism. A case is reported of hemiballism due to infarction of the territory supplied by the lenticulo-striatal arteries. CT and MR imaging showed lesions of the caudate nucleus and putamen. This suggested that the cause of the hemiballism was the suppression of the regulatory activity of the caudate nucleus on the pallidum.

[Obsessive-compulsive behavior and progressive supranuclear palsy]. / Comportement compulsif d'allure obsessionnelle et paralysie supranucléaire progressive.

A case of progressive supranuclear palsy characterized by a loss of self-activation and a compulsive behaviour of the obsessive type is reported. The pathological examination was remarkable for the intensity of pallidal lesions and their diffusion to both the external and internal segments. While the loss of self-activation seemed to result from a damaged cortico-subcortical circuit forming a limbic loop, the compulsive behaviour of the obsessive type may have resulted from the interruption of a frontal-caudal-pallidal-luysian circuit.

[Recurrent meningitis, hydrocephalus and tegmen tympani dehiscence]. / Méningite récidivante, hydrocéphalie et déhiscence du tegmen tympani.

Cerebrospinal fluid (CSF) otorrhea is the usual presenting symptom of spontaneous tegmen tympani defects. A case associated with recurrent meningitis and CSF rhinorrhea without otorrhea is described. The coexistence of an hydrocephalus had led initially to ventriculoperitoneal shunting, which did not prevent meningitis recurrence. Previously reported in cases of anterior fossa and sella defects, an aqueduct stenosis was here associated with the tegmen tympani defect.

[Painless apoplectiform paralysis of the brachial plexus]. / Paralysie plexique apoplectiforme non douloureuse.

A 40 year-old glass-blower suddenly experienced a painless weakness of his left shoulder and arm, more severe in C5 and C6 territories. The disorders regressed. Il was suspected that this brachial plexus neuropathy was of ischemic origin. However the absence of pain was surprising.

[Mitochondrial myopathy. Encephalopathy with lactic acidosis and cerebral infarction]. / Myopathie mitochondriale. Encéphalopathie avec acidose lactique et infarctus cérébraux.

A 21 year-old man presented with a history of sudden onset of aphasia and headache. CT showed a left parietal hypodensity and pallidal calcifications. The ECG showed a Wolff-Parkinson-White's syndrome. The patient then developed successively focal epileptic seizures, temper disorders, a cardiomyopathy, a pepper and salt retinopathy with hemeralopia, a left hemiplegia, deafness, and fever of unexplained origin. Left carotid angiography showed thin, irregular or occluded branches of the middle and anterior cerebral arteries. Blood muscle enzymes, lactate and pyruvate, were elevated with acidosis. Muscle biopsy revealed a mitochondrial myopathy and blood chemistry showed a severe deficiency of respiratory chain enzymes. Death occurred after 28 months. This case showed the diagnostic features of Melas, with some elements of the Kearns-Sayre syndrome. To our knowledge, this is the first case were serial angiographies allowed demonstration of arterial changes capable of explaining cerebral infarctions.

[Bilateral paramedian thalamic infarction: a case with MRI and gamma-emission tomography]. / Infarctus bithalamique paramédian: 1 cas avec IRM et tomographie d'émission gamma.

A 41-year-old woman presented with a history of sudden onset of a dementia with frontal signs and antero-retrograde amnesia. CT showed a bilateral paramedian thalamic infarct which was confirmed by MRI. The study of regional cerebral blood flow showed a decrease in both frontal regions. Results of imaging suggested lesions of the ventro-oral, latero-polar and intralaminar nuclei as well as of the mammillo-thalamic tract. The dorso-median nucleus appeared to be relatively spared.

[Borrelian meningoencephalomyelitis. A case]. / Méningonévraxite borrélienne. Un cas.

A 22-year old man had spastic paraparesis and cerebellar syndrome of 5 months duration. CSF showed lymphocytosis, elevated protein content, hypoglycorachia, hypochlorurachia and oligoclonal banding. CT scan and MRI were normal. Extensive laboratory procedures disclosed no bacterial, viral, fungic, parasitic or inflammatory disease. Anti-Borrelia burgdorferi antibodies were present in blood (1/4000) and in CSF (1/1024). With antibiotics (penicillin G 20 millions units per day for 10 days, followed by latamoxef 1.5 g per day for 3 months) and prednisone (50 mg per day for 2 months), the cerebellar signs disappeared, the paraparesis improved and the CSF abnormalities disappeared; blood and CSF anti-Borrelia antibody levels decreased. This case is an example of a severe form of CNS impairment by Borrelia burgdorferi. In Europe, most reports show progressive para- or tetraparesis with, sometimes, intellectual, cerebellar or cranial nerve impairment. In some cases, the signs are less diffuse or more acute. Lymphocytic meningitis is present with hyperalbuminorachia and oligoclonal banding; hypoglycorachia is mentioned in only one other report. Diagnosis is made by high blood and CSF antibody titers and demonstration of local synthesis. As in syphilis, borrelian meningoencephalomyelitis could be the third stage of the disease. Its treatment, often disappointing, consists of antibiotics (penicillin G or latamoxef) and in some cases corticosteroids.

Chorea associated with oral contraception.

Three patients developed chorea while receiving oral contraceptives. Two were young patients whose chorea developed long after treatment had been started and disappeared soon after it had been discontinued. The third patient had acute amphetamine-induced chorea after prolonged oral contraception. Prolonged administration of female sex hormones is a possible cause of chorea in women who have not previously had chorea or rheumatic fever.

Pigmentary type of orthochromatic leukodystrophy (OLD): a new case with ultrastructural and biochemical study.

A 34-year-old woman with no family history of orthochromatic leukodystrophy (OLD) developed progressive intellectual deterioration, a frontal syndrome and spastic tetraparesis. She died four years after the onset of the clinical illness. Neuropathological studies included light and electron microscopy of cerebral and nerve biopsies, and a complete postmortem examination. Light microscopy demonstrated OLD with pigmented macrophages and glial cells. Electron microscopy showed electron-dense, membrane-bound intracytoplasmic lamellar inclusions with curved or straight parallel arrangement, or fingerprint pattern, in white matter macrophages, astrocytes and oligodendrocytes. Cortical cells contained lipofuscin which was normal in type and amount. This suggests that the material in white matter glial cells and macrophages is ceroid pigment, however, the distribution is not that seen in ceroid-lipofuscinosis. Similar inclusions have been found in oligodendrocytes in other forms of OLD. Biochemical study did not show evidence of demyelination. Galactolipids were normal. Polyunsaturated fatty acids were decreased. The most striking feature was an increase in plasmalogens.

[Computerized tomography of the distribution of N-isopropyl-p-123 iodoamphetamine in cerebrovascular pathology. Comparison with studies using the 133-xenon inhalation method]. / Mesure tomographique de la distribution de la N-isopropyl-p-123 iodo-amphétamine en pathologie vasculaire cérébrale. Comparison avec la mesure par inhalation de Xéno133.

123Iodo-Amphetamine (IAMP) brain distribution was studied with a specially dedicated multilevel tomographic system in 30 patients with vascular ischemic (18) or hemorrhagic (12) disorders. Results were compared with clinical data, CT scan and rCBF measurement obtained by the 133Xe continuous inhalation method. Abnormalities with IAMP were found in 27 cases, in good agreement with the results obtained by the Xenon inhalation technique in 21 studies from the 22 where that method was possible. For 80% cases there was also a good agreement between the results obtained by the IAMP and CT scan: one case normal, and 23 observations with abnormalities. This is true for the localization of lesions, but IAMP appears able to shown remote abnormalities, i.e. crossed cerebellar diaschisis or functional deactivation, in the absence of CT scan lesions in the corresponding areas. This is of particular interest in the protracted regressive ischemic neurologic disorders (PRIND): CT scan were normal in all five observations but IAMP showed abnormalities in four cases. IAMP is however very expensive, so it has to be used only when 133Xe inhalation is technically impossible.

Lateral gaze disturbance in a case of Wallenberg's syndrome.

A 63-year-old Caucasian man was admitted for Wallenberg's syndrome following a left vertebral artery thrombosis. In addition to the classical symptoms, an axial lateropulsion to the left and ocular motor disorders (vertical diplopia, tonic deviation of the gaze to the left, skew deviation and horizonto-rotatory nystagmus) were present. These clinical signs are unusual, but in common Wallenberg's syndrome, neurophysiological tests often reveal slight abnormalities of oculomotor function: impairment of jerks, skew deviation, lateral deviation of the gaze in darkness. Interruption of cerebellar pathways is thought to be the cause of these symptoms. Their existence does not seem to change the outcome of these cases.

[Recurrent coma, papular mucinosis and benign dysglobulinemia]. / Comas répétés, mucinose papuleuse, dysglobulinémie bénigne.

A woman presented with a history of three regressive comas of undetectable etiology between the age of 52 and 57 years. An IgG lambda benign monoclonal dysglobulinemia was combined with a papular mucinosis (myxedematous lichen or the generalized form of Arndt-Gotton's scleromyxedema). In the 6 analogous cases documented in the literature the onset of coma occurred generally several weeks after an aggravation of the cutaneous lesions. The coma was preceded by an influenza-like syndrome followed by asthenia, malaise with vertigo and frequently epileptic seizures. During recovery, hallucinations and transient hepatic disorders were noted. Pruritus with pronounced hypereosinophilia preceded desquamation and regression of dermatologic lesions. These comas can lead to a fatal outcome (2 of 7 cases) or regress in 2 to 20 days usually without sequelae. The disease is probably of immunologic origin. The paraprotein or a serum factor could exert a direct toxic effect on brain. As in neurologic manifestations of malignant dysglobulinemia, explained initially by a "toxic encephalosis, clinical, angiography, biologic and immunologic data exist in favor of blood hyperviscosity. This hyperviscosity could result from polymer formation through intermediates immunoglobulins and other protein chains, or again from alteration of deformability of red cells by binding of paraprotein. Hyperviscosity syndromes are frequent in system diseases that are often associated with papular mucinosis. Whatever the exact mechanism of these "comas due to papular mucinosis", a logical choice is their treatment by immunosuppressants and plasmapheresis: in the case reported, the use of plasmapheresis as soon as premonitory signs had appeared probably prevented a fourth coma.