Outcomes of Acute Stroke Patients Requiring Mechanical Ventilation: Study Protocol for the SPICE Multicenter Prospective Observational Study.

BACKGROUND: Care pathways and long-term outcomes of acute stroke patients requiring mechanical ventilation have not been thoroughly studied. METHODS AND RESULTS: Stroke Prognosis in Intensive Care (SPICE) is a prospective multicenter cohort study which will be conducted in 34 intensive care units (ICUs) in the Paris, France area. Patients will be eligible if they meet all of the following inclusion criteria: (1) age of 18 years or older; (2) acute stroke (i.e., ischemic stroke, intracranial hemorrhage, or subarachnoid hemorrhage) diagnosed on neuroimaging; (3) ICU admission within 7 days before or after stroke onset; and (4) need for mechanical ventilation for a duration of at least 24 h. Patients will be excluded if they meet any of the following: (1) stroke of traumatic origin; (2) refusal to participate; and (3) privation of liberty by administrative or judicial decision. The primary endpoint is poor functional outcome at 1 year, defined by a score of 4 to 6 on the modified Rankin scale (mRS), indicating severe disability or death. Main secondary endpoints will include decisions to withhold or withdraw care, mRS scores at 3 and 6 months, and health-related quality of life at 1 year. CONCLUSIONS: The SPICE multicenter study will investigate 1-year outcomes, ethical issues, as well as care pathways of acute stroke patients requiring invasive ventilation in the ICU. Gathered data will delineate human resources and facilities needs for adequate management. The identification of prognostic factors at the acute phase will help to identify patients who may benefit from prolonged intensive care and rehabilitation. TRIAL REGISTRATION: NCT03335995.

[Medico-economic assessment of the Pontoise Hospital stroke unit]. / Évaluation médico-économique de l'unité neurovasculaire du centre hospitalier de Pontoise.

INTRODUCTION: Annually, approximately 120,000 people in France have a stroke. Various controlled studies have pointed out the benefits of treatment in a stroke unit (SU). The objective of this study was to evaluate, from a medical point of view, the economic impact of the Pontoise Hospital SU. PATIENTS AND METHODS: Based on the national cost study (NCS [étude nationale des coûts: ENC]) we analyzed data of five diagnosis related groups (DRG) which have a principle diagnosis in relation with stroke. This work was limited to strokes and transient ischemic events in adults and excluded sub-arachnoid hemorrhage. Medical and economic parameters were collected over the period from January to October 2006 and compared with those of the same period in 2005, that is to say before the opening of the SU. RESULTS: Three hundred and twenty-three hospital stays occurred between January 1st and October 31st, 2006 and 216 during the same time period before the opening of the SU, an increase of approximately 50% of all stroke-related admissions in our hospital. The number of stays carried out in the neurology unit increased by 29%. There was no significant difference between the two periods regarding age (median 69 versus 70 years) and sex- ratio. Average length of stay (ALS) was the same (9 days). There were no significant differences concerning the death rate (5.6% versus 6.2%) and that of discharge to home (44.6% versus 44.4%). The cost by stay in 2006 was 3534 euros [median; min 664-max 57,542] versus 3541 euros in 2005 [681-35,149] (p=0.57). Analysis by DRG highlighted an increase in the cost for serious strokes, cerebral infarctions and hemorrhages. For transitory ischemic events, the cost and the ALS decreased. CONCLUSION: After the opening of the SU, there was an increase in the activity without an increase in the total cost. This could be related in part to the limited means allocated to the stroke unit at its opening (in particular medical staff). The NCS can be used to evaluate the activity of a stroke unit. This work could be completed on a larger number of units or in several units of different size.

[Peripheral neuropathy and hepatitis C virus infection: more than cryoglobulinemia]. / Neuropathie périphérique et infection par le virus de l'hépatite C: ne pas s'arrêter à la cryoglobulinémie.

PURPOSE: To study characteristics of peripheral neuropathies associated with hepatitis C virus infection. METHODS: A study of two groups of four patients with peripheral neuropathy and infected with hepatitis C virus, and a literature review. RESULTS: We observed four cases of peripheral neuropathy in patients with hepatitis C virus infection with persistent negativity of mixed cryoglobulinemia, and with no purpura, rheumatoid factor, or low C4 level. Neuropathy improved with treatment in three patients between 8 and 28 months after treatment was begun. We report four other observations of peripheral neuropathy with mixed cryoglobulinemia. Worsening of peripheral neuropathy was observed 2 to 13 weeks after initiation of interferon alpha despite improvement of hepatic parameters. After stopping interferon, peripheral neuropathy worsened (one case), stabilized (one case), or improved (two cases). DISCUSSION: In patients infected with hepatitis C virus, peripheral neuropathy can be due to mixed cryoglobulinemia, periarteritis nodosa, and perhaps hepatitis C virus itself as suggested by our first observations. Low doses of interferon alpha may worsen peripheral neuropathy in patients with mixed cryoglobulinemia, as suggested in our later observations.

[Familial orthochromatic leukodystrophy: clinicopathological study of two cases]. / Leucodystrophies orthochromatiques familiales.

This paper reports the clinico-pathological data in a French family with orthochromatic leukodystrophy. The parents were first cousins and had seven children. Among those, two sisters and one brother presented with neurological signs, with onset around the 5(th) decade, including a dementing syndrome of frontal type, a tetrapyramidal syndrome, seizures, and, in one sibling, a cerebellar syndrome. CT scan or MRI showed diffuse involvement of the white matter. The neurological signs worsened progressively leading to death within 11 and 22 months. Neuropathological examination was performed in two cases. It revealed characteristic orthochromatic leukodystrophy. In one case, the presence of pigmented macrophages and astrocytes was suggestive of Van Bogaert and Nyssen disease. However there were some atypical features including the absence of pigmented cells in the second case whose clinical course was shorter, and the cavitary appearance of the white matter changes with a relative increase in the number of oligodendrocytes raising the issue of a possible link between this condition and cavitary orthochromatic leukodystrophies.

Systemic vasculitis in patients with hepatitis C.

OBJECTIVE: To analyze the main characteristics of patients infected with hepatitis C virus (HCV) presenting with different types of vasculitis syndrome. METHODS: We retrospectively compared 2 groups of patients with HCV presenting with systemic vasculitis: 10 with biopsy proven polyarteritis nodosa-type systemic vasculitis (PAN, Group 1) and 7 with mixed cryoglobulinemia syndrome (MC, Group 2). RESULTS: Patients of Group 1 presented with different features than Group 2: life threatening systemic vasculitis (10 vs 0; p < 0.01), severe multifocal sensorimotor mononeuropathies versus distal moderate sensory polyneuropathies, malignant hypertension (5 vs 0; p = 0.04), cerebral angiitis (2 vs 0), ischemic abdominal pain (2 vs 0), kidney and liver microaneurisms (2 vs 0), increased erythrocyte sedimentation rate and C-reactive protein (7 vs 0; p < 0.01), renal insufficiency (5 vs 0; p = 0.04), HCV genotype 1b (3 vs 6; p = 0.06), and lower activity of chronic hepatitis (p = 0.02). Neuromuscular biopsies showed lesions of vasculitis in all patients, but the type of vasculitis was different in Group 1 compared to Group 2: medium size artery involvement (7 vs 0; p < 0.01), necrotizing vasculitis (10 vs 0; p < 0.01), and mononuclear cell infiltrate in perivascular areas (0 vs 7; p < 0.01). Using prednisone, plasma exchanges, and interferon-alpha, complete recovery was obtained in all PAN-type patients except one. In Group 2 patients, interferon-alpha did not have any effect on the peripheral neuropathy. CONCLUSION: HCV infection may be associated with different types of systemic vasculitis, i.e., polyarteritis nodosa or mixed cryoglobulinemia. Because of differences in clinical and pathological features and therapeutic strategy, PAN-type vasculitis should be distinguished from MC-type vasculitis in HCV patients.

Hepatitis C virus infection with peripheral neuropathy is not always associated with cryoglobulinaemia.

OBJECTIVES: To describe cases of peripheral neuropathy associated with chronic hepatitis C virus infection without mixed cryoglobulinaemia. METHODS: Four cases of peripheral neuropathy associated with chronic hepatitis C virus infection with persistent negativity of mixed cryoglobulinaemia were found. RESULTS: All patients had small increases of transaminase levels and a positive viraemia. Liver biopsy showed chronic active hepatitis in all but one case (Knodell 4-9, Metavir A0F0-A3F3). Neuromuscular biopsy showed axonal neuropathy associated with lymphoid infiltrates around small vessels in two cases. Rheumatoid factor was always negative and C4 complement level was always normal. In three patients, neuropathy improved with interferon alpha, interferon alpha + ursodesoxycholic acid, or steroids + plasma exchange. CONCLUSION: Peripheral neuropathy may be associated with hepatitis C virus infection without mixed cryoglobulinaemia.

[What's bugging him?]. / Quelle mouche l'a donc piqué?

The purpose of this study is to report the case of a 34 year old patient, from Zaïre for whom a wrong diagnosis could have been fatal. Recent events in his affective and professional life, associated with typical symptoms, suggested a major depression. However after two months of being admitted a psychiatric ward, the resistance to treatment together with some biological data suggested further investigations. They revealed a subacuse meningo-encephalitis of parasitological etiology. The psychiatric aspects of trypanosomiasis commonly known by clinicians in Africa justifies, in patients from that continent, a systematic test for trypanosomiasis.

Measuring HIV-1 RNA and interferon-alpha in the cerebrospinal fluid of AIDS patients: insights into the pathogenesis of AIDS Dementia Complex.

The aim of this work was to study the role of HIV replication and the role of endogenous secretion of interferon-alpha in the pathogenesis of AIDS Dementia Complex (ADC). To accurately establish the diagnosis of ADC, 39 consecutive HIV-positive patients who presented with immune and intellectual deficiency underwent an extensive neurological evaluation. This included magnetic resonance imaging, neuropsychological testing and a lumbar puncture. The levels of HIV-1 RNA were measured in the cerebrospinal fluid (CSF) and blood by HIV Monitor (Roche Diagnostics) and those of interferon-alpha by an in-house biological assay. The diagnosis of ADC was established in 22 cases, which included nine out of the ten patients who had a high CSF viral load (above 4 log HIV-1 RNA copy per ml). Patients receiving highly active antiretroviral therapy had low viral loads in blood and CSF. In all 22 ADC patients, viral load in the CSF correlated with the staging of ADC (r=0.46, P=0.03), the CSF level of interferon-alpha (r=0.42, P=0.05) and with the bicaudate ratio (r=0.43, P=0.06), a measure of cerebral atrophy in the region of the caudate nucleus. No correlation was observed between CSF and plasma HIV-1 RNA. These results show that HIV may play a role in the neurological impairment of ADC patients possibly in part through the deleterious effect of interferon-alpha on the central nervous system and that highly active combination therapy should reduce or prevent these complications.

[Cerebral hemosiderosis related to hereditary ceruloplasmin deficiency. Clinical familial case study]. / Hémosidérose cérébrale liée à un déficit héréditaire en céruloplasmine. Etude clinique d'un cas familial.

A 59-year-old patient progressively developed dementia, hallucinations and facial dyskinesia. Brain T and T2-weighted MRI images showed low signal intensity on basal ganglia specially striatum, posterior thalamic and dentate nuclei. He had no evidence of ceruloplasmin and a high level of ferritin in the serum. Liver biopsy confirmed accumulation of iron in the cytoplasm of many hepatocytes. Similar clinical and biological signs were also observed in two brothers. All the three siblings were homozygous for a hereditary ceruloplasmin deficiency. This new clinico-pathological entity, first described in 1987, is different from Wilson's disease, Hallervorden-Spatz's disease and idiopathic hemochromatosis and linked to a mutation of the ceruloplasmin gene located on chromosome 3.

[Interhemispheric disconnection syndrome caused by partial infarction of the corpus callosum: neuropsychological study and MRI]. / Syndrome de déconnexion interhémisphérique par infarctus partiel du corps calleux: étude neuropsychologique et IRM.

A partial syndrome of hemisphere disconnection was observed in a 63 year-old woman, following an anterior and middle corpus callosum infarct on MRI. Notably, we found left ideomotor apraxia, diagonistic apraxia, left-year extinction on dichotic listening, but no left-hand anomia nor left visual field anomia. A left tactile extinction in dichaptic condition is interpreted as resultant of a dysregulation of the attentional balance between the two hemispheres. This detailed neuropsychological study permits a correlation between the callosal syndrome and the lesion. We suggest that an occlusion of the anterior callosal artery could explain this limited ischemia.

[Meningomyelitis disclosing Behçet's disease]. / Méningo-myélite révélatrice d'une maladie de Behçet.

The authors report a patient who developed a meningo-myelitis attributed to Behçet disease. Spinal MRI assessed spinal involvement. Among neurological manifestations of Behçet disease, isolated spinal cord involvement is infrequent. Dramatic improvement of neurological symptoms was obtained after steroid and immunosuppressive treatment.

[A contribution of brain imaging techniques to the diagnosis of psychiatric emergencies]. / Apports des techniques d'imagerie cérébrale au diagnostic de certaines urgences psychiatriques. A propos de deux observations.

This study was performed in the psychiatric emergency unit of René-Dubos General Hospital (Pontoise, France). Two cases of female patients with motor deficiencies suggesting a diagnosis of conversion disorder because of preceding psychological stressors and lability of symptoms, are presented. In both cases, the diagnosis was reconsidered after the use of RMI for multiple sclerosis in one case, HIV related encephalopathy in the other. Recent studies have shown that organic diseases with a psychiatric presentation represent 1,2 to 4% of patients examined in emergency setting. Criteria for the diagnosis of conversion disorders are discussed (they are classified as dissociative disorders in ICD-10 and as somatoform disorders in DMS-IV). A diagnosis of conversion disorder should not be made before a thorough clinical and neurological evaluation has been performed.

[Narcolepsy disclosing neurosarcoidosis]. / Narcolepsie révélatrice d'une neurosarcoïdose.

A 37-year-old man developed excessive daytime sleepiness, sleep attacks and cataplexy revealing an hypothalamic tumour. Multiple Sleep Latency Tests (MSLT) were characteristics of narcolepsy. Tissue typing was positive for HLA DR2 and DQ1. Most cases of narcolepsy are idiopathic without any evidence of brain pathology. Although symptomatic narcolepsy may occur occasionally with diencephalic lesions. The relationship between narcolepsy with diencephalic lesions is unsettled and will be discussed.

Aphasia and infarction of the posterior cerebral artery territory.

Spoken language disorders are rarely mentioned in superficial infarction of the posterior cerebral (PCA) territory. Two clinical types have been reported: transcortical sensory and amnesic aphasia. Between 1979 and 1990, we studied retrospectively 76 patients suffering from an occipitotemporal infarction located in the superficial territory of the posterior cerebral artery, all well documented by CT. Aphasia was one of the first and prominent signs in 18 cases. Middle cerebral artery concomitant infarction could have been the cause of language impairment in 10. In 8 patients aphasia was only explained by a PCA territory infarct. Three patients showed features of transcortical sensory aphasia. CT localization showed internal lobe and thalamic involvement of the dominant hemisphere. Five patients exhibited word finding impairment with various degrees of amnestic syndrome. The dominant internal temporal lobe was always affected. Dominant thalamus involvement was found in one case only. Some correlations between clinical features and anatomical support (vascular supply and anatomical structure) might be suggested in our 8 cases of aphasic disorders due to PCA infarcts. They are discussed and compared with data in the literature.

[Amnestic syndromes and confabulation in infarction of the posterior cerebral artery area]. / Syndromes amnésiques et fabulations au cours d'infarctus du territoire de l'artère cérébrale postérieure.

The Posterior Cerebral Artery (PCA) supplies the greatest part of the limbic system. Several authors have reported amnestic syndromes in PCA infarcts. Amongst 76 patients with a CT proven PCA infarct, 21 suffered anterograde amnesia. Confabulations were associated in 5 cases. We studied CT scans according to previously reported templates in the 21 amnestic patients: all patients with confabulations had a paramedian or a tuberothalamic infarct associated with a cortical infarct within the superficial territory of the PCA. None of the 16 remainders had a thalamic lesion. These results emphasize the role of the thalamus especially of the dorsomedial nucleus in the pathogenesis of confabulation. The underlying mechanism could imply fronto-cingular deafferentation.