Extensive form of progressive multifocal leucoencephalopathy associated with laryngeal carcinoma.

A case is reported of the rare association between progressive multifocal leucoencephalopathy and carcinoma. A 66-year-old man underwent a laryngectomy for carcinoma. Four years later he developed a local recurrence. Simultaneously there were behavioural disturbances and a left motor neglect followed by dense hemiplegia and coma. The patient died a further 5 months later. Anergy was demonstrated by skin tests. CT scan showed asymmetrical non-enhancing low-density areas in the hemispheric white matter, brain-stem and cerebellum and neuropathological examination confirmed extensive myelin loss with typical papovavirus inclusions in oligodendrocytes identified by electron microscopy.

Value of body mass index in the detection of severe malnutrition: influence of the pathology and changes in anthropometric parameters.

BACKGROUND & AIMS: We have estimated the prevalence of severe malnutrition in groups of patients hospitalized for different medical causes and assessed the sensitivity of BMI in the diagnosis of severe malnutrition. DESIGN: A prospective study enrolled 1052 patients: 396 patients with liver cirrhosis including 165 non-ascitic patients (NAP), 124 patients with mild ascites (MAP), 107 patients with tense ascites (TAP), 251 patients after cardiac surgery (SCP), 81 patients with cardiac diseases (MCP), 85 patients with stroke (SP), 36 patients with degenerative neurological diseases (DNP), 68 patients after surgery of a hip fracture (HFP), 91 patients with palliative care for cancer (CP) and 44 elderly patients with medical affections (EP). BMI, mid-arm muscular circumference (MAMC) and triceps skinfold thickness (TST) were measured within 48 h after admission. Patients with MAMC and TST below the 5th percentile of a reference population when aged < or = 74 or the 10th percentile when aged > or = 75 were defined as severely malnourished. Sensitivity of BMI < 20 to detect malnutrition was assessed. RESULTS: The prevalence of severe malnutrition was the highest in TAP (39.1%) HFP (25.6%) and MAP (24.3%) and the lowest in SCP (4%), SP (4.8%), DNP (5.7%) and MCP (7.4%) (P < 10(-4)). In multivariate analysis, low TST was associated with female gender (P < 10(-4)) mild and tense ascites (P = 0.038, P = 0.0004), low MAMC with male gender (P < 10(-4)), low BMI with female gender (P = 0.0082), hip fracture (P = 0.0407) and cancer (P = 0.0059). The sensitivity of BMI to detect severe malnutrition was the highest in HFP, CP and EP (100%, 80% and 100% respectively) and the lowest in TAP, MCP and SP (40%, 33.3% and 50% respectively). After exclusion of TAP, sensitivity of BMI to detect malnutrition correlated significantly with the coefficient of correlation between MAMC and TST observed in each group (r = 0.821, P = 0.0066). CONCLUSION: Ascitic cirrhotic patients and elderly patients after surgery of hip fracture had the highest prevalence of severe malnutrition. BMI had the highest sensitivity when both TST and MAMC were damaged to the same extent. BMI < 20 has a high sensitivity in the diagnosis of severe malnutrition in elderly and cancer patients but not in cirrhotic patients with tense ascites, cardiovascular and neurological patients.

[Non-specific angiitis and the central nervous system]. / Angéites non spécifiques et système nerveux central.

Central nervous system manifestations of systemic lupus erythematosus are reported in 25 to 60 p. cent of cases and include mental disturbances, epilepsy, focal deficits, and headache. Cerebrospinal fluid (CSF) changes are inconstantly observed. Cerebral scintigraphy may be useful. CT Scan imaging shows infarcts, hemorrhages, or cortical atrophy. Cerebral angiography is usually normal. Pathological examination shows frequent arteriolar lesions in the CNS but their appearance is rarely that of an angiitis. The mechanism of the CNS lesion involves both angiitis and antineuronal antibodies. Diagnosis is difficult when presenting signs are those of CNS involvement, particularly as the ESR can be normal. Serum complement and anti-DNA antibody levels are frequently normal when there is an isolated CNS involvement. Overall prognosis is poor in cases with CNS lesions. The use of corticoids is discussed. Cerebral angiitis is an exceptional finding during the course of rheumatoid arthritis and scleroderma. Central neurological manifestations of Sharp's and Gougerot-Sjögren's syndromes have recently been reported. CNS lesions are reported in 10 to 20 p. cent of patients with panarteritis nodosa and include mental disorders and disturbance of vigilance, epilepsy, focal deficits, meningeal signs and headache. The CSF is often normal. The CT scan provides images of infarcts, hemorrhages or cortical atrophy. Cerebral angiography may show segmental stenoses and distal occlusions. Pathological examination of the CNS shows mainly lesions in the intracerebral and leptomeningeal arterioles and small caliber arteries. Treatment is by corticoids and immunosuppressors. Angiitis of the CNS is rare and of late onset in Wegener's granulomatosis. Hypersensitivity angiitis rarely affects the CNS, those cases where it is involved being of poor prognosis and probably related to panarteritis nodosa. CNS manifestations in giant cell arteritis and Takayasu's arteritis result from neck artery lesions. The CNS is affected in 20 p. cent of cases in Behcet's disease with resulting isolated aseptic meningitis or a meningo-encephalomyelitis, and intracranial hypertension. The CSF is nearly always abnormal. Cerebral angiography should be directed towards the search for cerebral thrombophlebitis. Pathologically lesions predominate in the brain stem. Meningeal lesions are almost constantly present. Neurological involvement is of poor prognosis, and early corticotherapy must be instituted. The nosological autonomy of Buerger's thromboangiitis obliterans is questioned. Lymphomatoid granulomatosis is a particular type of angiitis in which malignant cell infiltration occurs. The CNS is affected in 20 p. cent of cases, and whatever the treatment the prognosis is poor. Granulomatous angiitis of the CNS has the distinctive feature of lesions affecting the CNS vessels either exclusively or predominantly. The antemortem diagnosis is based on biopsy of the leptomeninges, and treatment with corticoids and immunosuppressors may prove effective.

[Central nervous system involvement in systemic lupus erythematosus]. / Atteinte du système nerveux central au cours du lupus érythémateux disséminé.

Three cases are presented, in two of which the CNS lesions revealed the presence of systemic lupus erythematosus (SLE). The diagnosis of SLE was certain according to the criteria of the ARA, and it was further confirmed by results of renal needle puncture biopsy. Case 1: A 16-year-old adolescent developed choreic movements followed, one month later, by psychotic symptoms suggesting a mixed hebephrenic-catatonic schizophrenic affection. Cutaneous lesions and signs of renal insufficiency 3 months later established that these disorders were related to SLE. A favourable outcome was observed rapidly for the systemic signs, recovery from neuropsychic symptoms being obtained after 3 months only but then in a few days. This course suggests the diagnosis of a "functional psychosis" of lupus origin. Case 2: A 24-year-old woman developed left hemiparesis followed by febrile coma. The slowly favourable course of the disease led to the appearance of a progressive dementia, with numerous epileptic seizures. Although tests for antinuclear antibodies were negative and the ESR was normal, several minor biological anomalies were suggestive of a systemic disease and the diagnosis of SLE was finally established. Corticotherapy produced only slight transient improvement. This progression towards dementia with progressive cerebral atrophy is most probably related to cerebral lupus lesions, the initial coma in the absence of any other apparent cause possibly being the first sign. Case 3: A 47-year-old woman developed simultaneously or separately episodes of arthralgia and uveitis of unknown origin over a 12-year period, and attacks of regressive multilocular neurological deficiency over a 15-year period.(ABSTRACT TRUNCATED AT 250 WORDS)

[Non-dominant hemisphere syndrome as a result of a right thalamic infarct: an anatomoclinical case]. / Syndrome de l'hémisphère mineur par infarctus thalamique droit: un cas anatomoclinique.

A 71 year old man had a massive left sensory deficit and hemiplegia, with left heminanopia, visual neglect and constructional apraxia. Moreover he experienced an extra-left arm and illusions of movements. 3 weeks later he suffered "thalamic" pain on left side; he died suddenly 6 weeks after the stroke. Post-mortem examination revealed: a) a right inner temporal and occipital infarction; b) a right thalamic infarction in the thalamogeniculate and paramedian territories; c) an infarction in the adjacent right internal capsule. Considering this case and pertinent literature on clinicopathological studies of right thalamic infarction, the authors suggest that a simultaneous ischaemia of thalamogeniculate and paramedian territories should be necessary to induce somatognosic and visuospatial disturbances.

[Amnesia from bilateral ischemic lesions of the thalamus (author's transl)]. / Amnésie par lésion ischémique bi-thalamique.

A 43-year-old woman developed during post-partum an axial amnesia of ischemic origin. Stereotactic correlations were obtained by matching CT scan images with the true reference plane of the examination, according to an original method. This led to the diagnosis of infarction in the territory of a paramedian thalamomesencephalic artery. The amnesia probably resulted from bilateral lesions of Vicq d'Azyr's bundle. Atypical features in this case were the absence of disorder of vigilance and oculomotor disorders. This striking fact in this patient was recovery of some recognition capability contrasting with the persistence of a major evocation disorder.

[Pigmentary orthochromatic leukodystrophy. Van Bogaert and Nyssen disease]. / Leucodystrophie orthochromatique pigmentaire. Maladie de van Bogaert et Nyssen.

A 43 year old woman with a strong likelihood of familial history, developed progressively a spastic tetraparesis associated with intellectual deterioration and terminal epileptic fits. She died 11 years after onset of the clinical disorders. Neuropathological study revealed an orthochromatic leukodystrophy. Macrophages and glial cells of the white matter contained a brown-yellow, autofluorescent pigment which stained positively with PAS, Perls stain for iron and Masson-Fontana. 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 oligodentrocytes in other forms of orthochromatic leucodystrophy. Ten similar cases of pigmentary type of orthochromatic leucodystrophy have been reported previously; only one had had an ultrastructural study.

[Cerebral tomoscintigraphy with N-isopropyl-p-I123-iodoamphetamine. Value in cerebral ischemic pathology]. / Tomoscintigraphie cérébrale utilisant la P. iodo I123N isopropyl-amphétamine. Intérêt en pathologie ischémique cérébrale.

Thirty patients with previous cerebral ischaemic accident were explored by both computerized tomography (CT) and radionuclide tomography after injection of 123I-labelled N-isopropyl-iodo-amphetamine. All lesions that were visible at CT were also visible at radionuclide tomography. However, in 7 patients with normal CT results the latter method showed areas of cerebral activity anatomically correlated with neurological signs or vascular lesions. Cerebellar diaschisis was observed in 50% of established lesions in the carotid territory. Radionuclide tomography therefore appears as a very sensitive method to be used in ischaemic pathology with transient or regressive accidents.

[Anatomo-radiological correlations in astrocytic cerebral tumors]. / Corrélations anatomo-radiologiques en matière de tumeurs cérébrales astrocytaires.

We have developed a method for creating brain slices in the same plane and intervals as pre-mortem CT scan images. Our experience with this very simple technique on 20 brains confirms the practicality, reliability and usefulness of this tool for correlating complex CT images with the pathologic anatomy. Corresponding case-material of cerebral astrocytomas with be presented.

[Polyglucosan body disease]. / La maladie des corps polysaccharidiques.

A 78 year-old male presented with a bilateral pyramidal syndrome, urinary incontinence and mild intellectual slowing. He died seven months after onset of the neurological signs from cerebral infarct and heart failure. Neuropathological examination showed predominant involvement of the cerebral white matter including diffuse myelin pallor, astrocytic gliosis and small necrotic foci. Polyglucosan bodies were diffuse in the cerebral cortex, white matter, brainstem, cerebellum and proximal part of the cranial nerves. In these latter, some polyglucosan bodies were found within myelinated axons but the inclusions mostly involved astrocytic processes. This case is characteristic of the polyglucosan body disease. It is compared with the autopsy and biopsy cases previously reported in the literature.

Almitrine neuropathy. A nerve biopsy study of 8 cases.

We report the quantitative and qualitative, light and electron microscopic studies, including teased fiber preparations of nerve biopsies obtained from eight patients, treated with almitrine, presenting with the characteristic association of a sensory peripheral neuropathy with a recent body weight loss. The data were consistent with an axonal damage affecting myelinated fibers, predominantly large ones and to a lesser degree unmyelinated fibers, some degree of segmental demyelination. Marked axonal regeneration was observed when the nerve biopsy was delayed after withdrawal of the drug, Micro-angiopathy secondary to the formation of concentric lamellae from the basement membrane was observed in five patients suffering from chronic hypoxemia. Almitrine is an agonist of the chemoreceptors. The pathogenesis of the toxic neuropathy induced by this compound remains obscure. Clinical features do not fit with a purely hypoxic mechanism at the origin of the neuropathy.

[Treatment of malignant glioma. Apropos of 39 cases]. / Traitement des gliomes malins. A propos de trente-neuf cas.

Thirty-nine patients with malignant gliomas (36 glioblastomas, 3 astrocytomas grade III; age median 52.5) were treated after surgery by combination chemotherapy followed by irradiation. The median survival on the actuarial curve is 7 months. An attempt is made to evaluate each therapeutic modality by analysis of clinical and CT parameters.

Posterior callosal infarction. Clinicopathological correlations.

Clinicoanatomical observations on a patient with partial interhemispheric disconnection associated with complete ischaemic destruction of the splenium and of the posterior part of the body of the corpus callosum are presented. Neuropathological examination of the areas containing degenerated white matter indicated that the lesions affected the transcallosal fibres that link the cortex of the occipital lobes and the superior parietal lobules (SPL). The white matter situated in the temporal lobes was intact. This suggests that in man, and contrary to what has been described in the monkey, the callosal pathway followed by the temporal fibres is rostral to the one followed by the parietal or at least by the SPL fibres. The most prominent disconnection syndrome elements were left tactile anomia (in spite of a rather good tactile-motor integration between the hemispheres), left visual anomia, agraphia of the left hand and 'diagnostic' apraxia. The fact that the anterior part of the corpus callosum was intact accounts for the preservation of interhemispheric transfer of somatic sensory information and for the absence of left extinction during the dichotic listening test. An attempt is made to give a more detailed explanation of the results obtained during the different tests.

Adult form of Leigh's disease: a clinico pathological case with CT scan examination.

The clinical and pathological findings of a 31-year-old woman, in whom the diagnosis of Leigh's disease was made, are reported. CT scan examination with contrast enhancement showed symmetrical areas of low density, in both thalami, anterior limbs of internal capsules and corpus callosum. Longstanding chronic lesions involved the optic chiasma and the cerebral peduncles and consisted of myelin loss, status spongiosus, astrocytic gliosis and marked capillary proliferation. The neurons were spared. In the basal ganglia, internal capsules and corpus callosum, these lesions were more recent and consisted of focal necrosis, perivascular oedema and few lymphocytic perivascular cuffings.

A 5-year epidemiological study of extended-spectrum beta-lactamase-producing Klebsiella pneumoniae isolates in a medium- and long-stay neurological unit.

Thirty-eight different strains of extended-spectrum beta-lactamase (ESBL)-producing Klebsiella pneumoniae (ESBL Kp), isolated from urine and pus samples of 38 patients hospitalized in a medium- and long-stay neurology department between 1 January 1992 and 31 December 1996, were analysed by antibiotic resistance phenotyping, DNA macrorestriction by pulsed-field electrophoresis and isoelectric focusing of beta-lactamases. An epidemiological survey was conducted to identify risk factors for infection by ESBL Kp in this setting. The 38 isolates were distributed into 13 antibiotypes, three of which predominated (13, six and six isolates). The DNA macrorestriction pattern identified 15 genotypes, four of which predominated (11, six, four and four isolates). A combination of the two typing methods revealed several epidemic clones that emerged consecutively. Two main types of ESBL (SHV-2 and CTX-1) were identified by isoelectric focusing, the former predominating. The case-control study showed that the length of hospital stay, degree of malnutrition and dependency, and urinary sphincter status were the main factors significantly associated with ESBL Kp isolation.

Peripheral neuropathy in patients treated with almitrine dimesylate.

Sensory peripheral neuropathy developed in 5 patients treated with almitrine dimesylate, 60-100 mg/day. Onset was insidious, beginning symmetrically in the legs with stocking sensory loss and loss of ankle-jerks. Cerebrospinal fluid protein levels were slightly increased and there was electrophysiological and histological evidence of distal axonopathy. The interval between the first dose of almitrine dimesylate and onset of symptoms ranged from 2 to 4 months in 4 patients. All patients had noted a recent weight loss of 4-15 kg which may have resulted in release of previously bound drug.

Extensive oxidative metabolism of dextromethorphan in patients with almitrine neuropathy.

Almitrine bismesylate can induce a stereotypical sensory peripheral neuropathy probably through a toxic mechanism. High plasma concentrations of almitrine have been reported in a patient with neuropathy. Since large inter-individual variations in plasma drug concentrations are found it is possible that the development of toxicity may be linked to genetically determined polymorphic oxidation of the drug. Oxidation phenotyping was performed in fifteen patients with almitrine neuropathy using dextromethorphan, a test compound subject to oxidative metabolism similar to that of debrisoquine. All patients were of the extensive metaboliser phenotype. This result shows that, in contrast to perhexiline neuropathy, almitrine neuropathy is not related to slow oxidation of the compound with regard to the particular P-450 iso-enzyme involved in dextromethorphan and debrisoquine metabolism.

[Outbreak of infections of Klebsiella pneumoniae with extended spectrum beta-lactamase in a hospital unit with a long and medium stay]. / Epidémie d'infections à Klebsiella pneumoniae à beta-lactamase à spectre étendu dans un service de long et moyen séjour.

Fourteen extended-spectrum beta-lactamase (ES beta la) Klebsiella pneumoniae strains were isolated from 14 inpatients between February 1993 and February 1994, in a medium- and long-stay neurological unit. For this reason an epidemiological study was begun, based on strain typing and examination of patient files. Strain typing was carried out by two methods (i) the analysis of antibiotic resistance, showing 7 different antibiotypes among the 14 strains studied, (ii) the analysis of esterase and dehydrogenase electrophoretic polymorphism in polyacrylamide-agarose gel. The method was checked by analysing 11 Klebsiella pneumoniae strains with wild phenotype for beta-lactam antibiotics, which were isolated during the same period in the same unit. Simultaneously 6 other strains isolated during the same period in some other units of the hospital were analysed. Nine electrophoretic types were found among the 31 strains (wild and ES beta las). The analysis of the results showed that 8 isolates of the group of 14 ES beta las had the same antibiotype and electrophoretic type. This demonstrates that one epidemic strain was responsible for two outbreaks, the first one in April and the second one in August-September. A case control investigation was carried out to define the risk factors of infection. Files were examined for the 14 infected inpatients and for 20 control inpatients from the same unit during the same period. Statistical analysis was performed with Epi Info software 5 (CDC Atlanta). Length of stay, dependence and malnutrition levels, and urinary sphincter disfunction were the most significant risk factors.