Central Neurological Involvement Research Articles

Central nervous system involvement in patients with HCV-related cryoglobulinemia: review and a case report

Few well-documented cases of central nervous system involvement in patients with mixed cryoglobulinemia and/or HCV infection have been reported. We can distinguish between acute or subacute diffuse and focal lesions (transient ischemic attack-like syndromes and cerebrovascular accidents). A search of two electronic databases (Medline and EMBASE) was conducted from the year of their inception (1966 for Medline and 1988 for EMBASE) to September 2000. The search strategy employed entailed combining these terms: Cryoglobulinemia, Central Nervous System, Hepatitis C, Chronic Hepatitis. Cryoglobulinemia and Central Nervous System were also used as free test words. We analysed articles with case reports and the most frequent articles on the references list. The main pathophysiologic mechanism of cerebral involvement is ischemia (or rarely hemorrhage) due to diffuse or segmental vasculitis of the small cerebral vessels. In these cases a brain MRI usually shows single or multiple increased T2 signals. Furthermore an occasional occlusive vasculopathy without vasculitis was documented histologically. In these patients ischemia could be started or enhanced by the engorgement of the microvasculature by clumps of red cells and by aggregates of cryoglobulins. In the same patients vasculitis and hemorheological abnormalities can affect the clinical picture of the cerebral involvement in mixed cryoglobulinemia. Finally, the detection of HCV in the lesions induces a hypothesis that, in some cases, CNS involvement could be directly related to chronic HCV infection, even in the absence of cryoglobulin production. We describe a 63 year-old woman with acute severe encephalopathy. Laboratory evaluation revealed a high positive test result for rheumatoid factor (3390 U/ml) and hypocomplementemia (C4 less than 1.67 mg/dl). Protein immunofixation electrophoresis demonstrated 5% monoclonal proteins (IgM/k and IgG/k), 3% cryoglobulins were present, HCV antibody and HCV-RNA (type 2a-2c) were positive. Cryoglobulins were never typed, because they disappeared after plasma exchanges. Liver enzymes, renal function and findings on cerebrospinal fluid were normal. Cerebral CT and MRI were also normal. Antinuclear antibodies, anti nDNA antibodies, antiphospholipid antibodies, lupus anticoagulant, ANCA, Lyme disease serology, complete tests for thrombophilia were negative. Bone aspiration was normal. The patient, in coma, was treated with two plasma exchanges. During the first treatment she recovered consciousness. Prednisone (1 mg/Kg/day) and cyclophosphamide (400 mg iv for three days) were added. After a week two plasma exchanges were performed again. Liver enzymes and rheumatoid factor were analysed monthly for six months and than every two months for another six month period up to the present. Liver enzymes were always normal, rheumatoid factor was always at a lower level than the first evaluation (now it's 311 U/ml). At present she is taking Prednisone 5 mg once a day, neurologic symptoms are absent and neurologic examination is normal. We can conclude that: central neurologic involvement may be the clinical presentation of HCV infection and mixed cryoglobulinemia. HCV serologic tests and cryoglobulins should be considered in patient with encephalopathy of non-obvious cause; plasma exchange is the treatment of choice in acute severe forms; in some patients HCV could involve directly CNS, even in the absence of cryoglobulin production.

Responsabilité de la ciclosporine dans la survenue d’un neuro-Behçet ?

Central neurological involvement in Behcet's disease (neuro-Behcet) occurs in 10 to 20% out of the patient. We report a 47-year-old man treated with cyclosporine who presented with neurologic manifestations of Behçet's disease. Relationship between cyclosporine and neuro-Behcet's disease are discussed.

Étude de 22 observations de neuroBehçet dans une série de 170 maladies de Behçet

To present neurological involvement in Behçet's disease, its prognosis and treatment. Our study was retrospective and was done at Hotel-Dieu Hospital of Beirut between 1980 and 2005. All these patients fulfilled the International Study Group criteria for diagnosis of Behçet's disease. Neurological involvement was observed in 13% (22/170) of our patients and was more frequent in men (sex-ratio: 1,75). The mean age of onset for Behçet's disease and NeuroBehçet's syndrome was 26+/-6 and 30+/-8 years respectively. Central nervous system involvement was found in 21 patients and peripheral nervous system involvement in one. Meningoencephalitis and/or transverse myelitis were found in 57% (12/21) of cases (in association with brainstem syndrome in 2 of these cases), brainstem syndrome without meningoencephalitis in 5 cases, tumor-like syndrome in 2 cases, repetitive ischemic attacks in 1 case and cerebral venous thrombosis in one. Focal deficits were the major presenting signs (16 cases) and external oculomotor nerve paralysis was observed in 4 patients. In meningoencephalitis, the cerebrospinal fluid findings were lymphocytic pleocytosis and elevated protein level. CT Scan, performed in 6 patients, was normal in 33% of cases. MRI, performed in 9 patients, was abnormal in 6 and showed abnormal signals distributed over the brainstem and the thalamus in 4, a tumor-like lesion and thrombosis of the left lateral sinus one each. Corticosteroids were usually efficacious but, when used alone, relapse was observed in 31% of patients. One patient who had brainstem syndrome died within 18 months because of a delayed corticosteroid treatment. Within central neurological involvement in Behçet's disease, we can individualize 4 clinical aspects: meningoencephalitis (and/or myelitis), brainstem syndrome, tumor-like features and cerebral venous thrombosis. Abnormalities, observed on CT Scan and MRI, by their brainstem localization and their multiplicity, should evoke the diagnosis. Corticosteroids, when prescribed early, are useful and are associated with better prognosis; their association to immunosuppressant agents should be considered in the parenchymatous forms.

Infection à virus Chikungunya : revue générale par temps d'épidémie

The Chikungunya virus is an alpha arbovirus, first identified in 1953, transmitted by Aedes, mosquitoes, responsible for a little documented uncommon acute specifically tropical disease. Its main symptoms are fever, a rash, and debilitating arthralgia. An unprecedented Chikungunya epidemic is ongoing on the Reunion Island (775,000 inhabitants) with over 244,000 reported and 205 deaths (directly or indirectly linked) as of April 20 2006. Aedes albopictus, long present on the island, is the assumed vector. It had already been identified as the vector for type 2 Dengue fever in 1997–1978 (200,000 cases) for type 1 Dengue fever in 2004 (300 cases). After the Grande Comore Island epidemic, the first cases were reported in the Reunion Island in March 2005. The epidemic was a surprise because of its unexpected emergence, its magnitude, and clinical cases rarely or never described before: severe forms, central neurological involvement, hepatic cytolyse, severe lymphopenia, severe dermatological involvement, deaths, and neonatal infections. This is the first manifestation of the intrusion CHK virus on the island, which benefits from a sub-tropical climate, but also of an occidental healthcare environment, with a non-immune population. This is also the first time that a Chikungunya epidemic is described in this part of the world.

Central Neurologic Involvement in Mycosis Fungoides

Neurologic involvement in mycosis fungoides is rare. Isolated case reports in the literature suggest the pattern and the natural history for such occurrences, while a literature summary can provide direction on diagnosis and management. Although case series may confirm such information, cohort data are required to establish an overall risk of occurrence and to evaluate possible predictive factors. We presented a case of central nervous system involvement in mycosis fungoides from Haifa, Israel and tabulated a series of nine cases from Canada. To estimate the risk of neurologic involvement, a cohort of 680 consecutive patients with newly diagnosed mycosis fungoides, of which the nine cases of neurologic involvement emerged during follow up, was analyzed using the Kaplan-Meier method. The actuarial risk of developing neurologic involvement was related to the baseline tumor-node-metastasis-blood classification factors. The pattern of disease in these 10 additional cases confirms the overall pattern in the approximately 40 patients described in the literature. The main symptoms are fluctuating higher cognitive functions and cranial nerve dysfunction, with fairly rapid clinical onset of symptoms. Most cases of central neurologic involvement with mycosis fungoides emerge within a setting of advanced disease. In patients with newly diagnosed mycosis fungoides, the greatest risk of developing neurologic involvement is within the first several years after diagnosis and is associated with the initial stage of disease. Patients with two or more of the T3-4, N3, M1, and B1 classification factors have a one in six chance of developing central neurologic involvement, while there is about a one in a hundred chance for the corresponding control group. Neurologic involvement with mycosis fungoides is indeed rare, but it is associated with a more advanced stage at diagnosis and with other visceral disease that can precede it. Although the role of low-dose prophylactic cranial radiation is uncertain, overt neurologic involvement requires urgent palliative treatment.

Subacute aseptic meningitis as neurological manifestation of primary Sj ögren's syndrome

Sjögren's syndrome (SS) is an autoimmune disease characterized by inflammatory infiltration and secondary chronic dysfunction of exocrine glands. Systemic (extraglandular) manifestations of the disease occur in one-third of the patients, including a wide spectrum of peripheral and central neurological disorders. We report a case of subacute afebrile aseptic meningitis (AM) as neurological manifestation of primary SS. The neurological symptomatology presented gradual onset and progression, including diplopia, mild headache, pain and stiffness of the neck. The clinical examination pointed out xerostomia and xerophthalmia. Diagnosis of SS was confirmed by Schirmer's tear test and histopathology of the labial salivary glands. The neurological involvement was highlighted by gadolinium-enhanced magnetic resonance imaging of the brain which displayed an increased diffuse leptomeningeal enhancement. Cerebrospinal fluid (CSF) analysis showed moderate pleocytosis with prevalence of polymorphonuclear leukocytes and increased protein level but no evidence of Ig intrathecal synthesis. A cycle of intravenous steroid therapy led to a complete disappearance of the neurological symptomatology and to normalization of the CSF inflammatory pattern. Given the unusual presentation of this case of AM, which resembled the characteristics of a chronic meningitis rather than those of an acute form, in patients affected by SS we must stress the importance of cephalic symptoms such as headaches and cervical stiffness (even if mild and without fever) as possible signs of central neurological involvement of the disease.

Hemophagocytic Syndrome and Adult Still's Disease Associated with Meningoencephalitis and Unconsciousness.

We describe a 19-year-old woman with hemophagocytic syndrome and adult Still's disease who showed rare features of central neurological involvement, including cerebellar symptoms and the sudden onset of unconsciousness with pleocytosis in the cerebrospinal fluid during the early course of the illness. As this patient's serum showed a high level of interferon-gamma and soluble interleukin 2 receptor, this might play a pathologic role in the development of central nervous system symptoms. Intensive treatment consisting of methylprednisolone pulse therapy followed by the oral administration of methylprednisolone and cyclosporine, as well as plasma exchange, was found to achieve good results.

Sicca syndrome and anti-SSA/Ro antibodies in patients with suspected or definite multiple sclerosis.

Thirty-eight consecutive patients with primary Sjögren's syndrome (SS) and 36 with suspected (15 cases) or probable to definite (21 cases) multiple sclerosis (MS) underwent thorough neuropsychiatric and rheumatological examinations in order to detect either the presence of central neurologic involvement in SS or the presence of clinical and immunologic abnormalities typical of SS in MS. The only neuropsychiatric abnormality we found in patients first recorded for primary SS was major depression in 5 cases. On the other hand, one patient with suspected MS because of mild gait ataxia and right hemiparesis fulfilled the diagnostic criteria for SS. As for serologic tests, 6 out of 21 patients with probable to definite MS and 1 out of 15 with suspected MS had low-titre antinuclear antibodies. One patient with definite MS had also circulating anti-SSA/Ro antibodies, detected by gel double diffusion, but did not develop sicca symptoms after a 2-year follow-up. In the present study, the association between SS and MS was considerably weaker than that recently reported in other studies, and might be regarded as a chance finding.

Speech symptoms associated with early signs of Shy Drager syndrome.

Speech disturbances reflecting impaired laryngeal control were found in ten patients whose autonomic dysfunction was associated with central neurological disease (Shy Drager Syndrome). In contrast, ten patients with progressive autonomic failure without evidence of CNS involvement had no difficulties on speech function tasks in comparison with normal controls. Presence of speech symptoms may aid in the clinical differentiation between patients with pure autonomic dysfunction and those with central neurological involvement.