Acute hemorrhagic leukoencephalitis with atypical features.

Acute hemorrhagic leukoencephalitis (AHL) is a rare demyelinating disease mainly affecting children, characterized by acute onset, progressive course and high mortality. A 62-year-old man was admitted to our Unit for diplopia and ataxia ensuing 2 weeks after the onset of pneumonia. MRI T2-weighted images showed signal hyperintensities in the brainstem. Antibodies against Mycoplasma Pneumoniae and cold agglutinins were found. Two weeks later the patient had a worsening of his conditions: he developed left hemiplegia with motor focal seizures and the day after he was deeply comatose (GCS = 4). A second MRI scan showed extensive hyperintensities involving the whole right hemisphere white matter with a small parietal hemorrhagic area. The clinical and neuroimaging features suggested the diagnosis of AHL, Aciclovir in association with steroid therapy were administered and then plasmapheresis was started. After 30 days of coma, the patient gradually reacquired consciousness and motor functions; anyway a left hemiplegia persisted.

Atypical presentation of varicella-zoster virus encephalitis in an immunocompetent adult.

BACKGROUND: Varicella-zoster virus encephalitis is uncommon, but not rare, in immunocompetent adults. Typically, patients develop stroke with hemiplegia caused by large vessel vasculopathy days to weeks after herpes zoster ophthalmicus. METHOD: A previously healthy 66-year-old man developed obtundation deteriorating to coma within 24 hours. He had lymphocytic meningitis and multiple bilateral edematous and hemorrhagic lesions predominantly in the white matter, and intraventricular and subarachnoid hemorrhage. Treatment with acyclovir and dexamethasone was readily administered. The diagnosis of varicella-zoster virus encephalitis was confirmed by polymerase chain reaction analysis of the cerebrospinal fluid. No zosteriform rash preceded or followed encephalitis. Two years later, the patient is in good health, and no relapse or sign of immunosuppression has been reported. CONCLUSION: This is a case of varicella-zoster virus encephalitis in an immunocompetent patient presenting without typical rash and with clinicoradiologic features of multifocal encephalitis, which characterize immunosuppression.

Immune reconstitution inflammatory syndrome associated with PML in AIDS: a treatable disorder.

An HIV-1-infected patient with progressive multifocal leukoencephalopathy presented clinical deterioration and contrast-enhancing lesions on brain nuclear MR after the initiation of highly active antiretroviral therapy (HAART). Brain biopsy identified an inflammatory reaction compatible with immune reconstitution inflammatory syndrome. Treatment with corticosteroids and transient suppression of HAART led to marked neurologic improvement.

Acute childhood hemiplegia associated with chickenpox and elevated anticardiolipin antibody.

Chickenpox is a common infection of childhood. Central nervous system complications, such as cerebellar ataxia and meningoencephalitis, are estimated to occur in less than 1% of cases of chickenpox. Although transverse myelitis and hemiplegia have also, although rarely, been reported, hemiplegia associated with chickenpox and elevated anticardiolipin antibody has not been reported in the literature. We report the case of a 2.5-year-old boy who developed a right hemiplegia 3 weeks after the onset of a primary varicella infection. The serum level of anticardiolipin antibody IgG was markedly elevated. Brain magnetic resonance imaging (MRI) revealed an infarction involving the left globus pallidus, caudate nucleus, and posterior leg of the internal capsule. Laboratory studies ruled out all known causes of stroke. Neurologic signs and symptoms slowly improved with supportive treatment. Varicella infection should be considered one of the possible causes of acute ischemic strokes in children. Usually, two or more risk factors are detected in children with ischemic strokes, and we suggest that all possible causes of strokes be investigated in children with ischemic strokes.

[Rasmussen syndrome]. / Síndrome de Rasmussen.

INTRODUCTION: Rasmussen's encephalitis is a neurological disorder probably of immunological origin, characterized by intractable epilepsy, neurological deterioration and the presence of antibodies against R3 glutamate receptors. OBJECTIVE: To present the experience of an Argentinian Paediatric Neurology Service during the past 7 years. MATERIAL AND METHODS: We analyzed the clinical histories of 12 patients with RS as defined by the diagnostic criteria proposed by Hart et al. We evaluated the following parameters: neurological examination, neuropsychological assessment, types of crises, age of onset, frequency, distribution, electroencephalogram, video EEG, evoked potentials, neuro-radiological studies; CT and cerebral MR using gadolinium, SPECT, lumbar puncture and cerebral biopsy. RESULTS: The crises were of simple partial motor type in 12 cases, secondarily generalized partial crises in 7 cases, complex partial crises in 4 cases and partial and generalized motor states in 8 cases. RESULTS. The ictal EEGs during the continuous partial crises showed contralateral focal slow activity of the affected side of the body in 30% of cases. The neuro-radiological studies (CT and MR) showed signs of cerebral hemiatrophy in all 12 cases, hyper-intense images in the T2 sequences of the fronto-temporo-parietal regions in 5 cases, an asymmetric megacisterna magna in 1 case and hypoplasia of the cerebellar vermis in another case. CONCLUSIONS: The continuous partial crises showed little correlation with the electro-encephalography. Following administration of i.v. gammaglobulin there was a transient halt in progression of the neurological deterioration and control of the crises in 55% of the cases. Surgery continued to be the treatment recommended for control of the epileptic crises.

Lateralization of T-lymphocyte responses in patients with stroke. Effect of sympathetic dysfunction?

BACKGROUND AND PURPOSE: A number of clinical observations indicate that stroke affects the course of immune-mediated diseases by lateralization of the disease manifestations, such as arthritis. The purpose of this study was to assess the impact of early stroke on lateralization of immune responsiveness. METHODS: The delayed-type hypersensitivity (DTH) reaction to purified protein derivative was used as an in vivo measure of antigen-specific T-lymphocyte reactivity. Assessment of axon reflex vasodilation was simultaneously used to test for cutaneous sympathetic activity. RESULTS: There were no significant differences with regard to lateralization of DTH reactivity when all stroke patients were tested. However, patients with minor stroke displayed a significant (P < .001) decrease of DTH reaction on the paretic side compared with the contralateral side. In contrast, patients with major stroke showed a significant increase (P = .022) of DTH reaction on the paretic side. Patients with left hemiparesis had a significantly greater (P = .045) DTH response on the affected side than patients with a right hemiparesis. In addition, only the patients with motor deficit but not with sensory deficit or aphasia displayed side differences in DTH responses. When electrically evoked axon reflexes were studied in relation to DTH reactions, a significant correlation (r = .64; P < .001) was found between side asymmetries of DTH responses and side asymmetries of axon reflexes in an innervated skin area. No similar relation was present in skin areas where cutaneous sympathetic activity had been blocked by regional anesthesia. CONCLUSIONS: Early stroke lateralizes T-cell-mediated cutaneous inflammation. This effect depends on (1) the localization of the brain lesion, (2) the clinical course of the disease, and (3) the presence of motor deficit and may be mediated by (4) alteration of the cutaneous sympathetic nerve traffic.

Fetal stroke associated with elevated maternal anticardiolipin antibodies.

Middle cerebral artery infarction explains some cases of congenital hemiparesis with or without neonatal stroke. The etiology of the stroke is often obscure. We describe two infants with imaging evidence of middle cerebral artery infarction whose mothers had elevated anticardiolipin antibody levels after delivery. We speculate that these antibodies may have been responsible for intrauterine thromboembolic stroke.

Unilateral bullous pemphigoid in a hemiplegic patient.

A 78-year-old man with a long-standing left-sided hemiplegia presented with bullous pemphigoid which affected his paralysed side only. Although the rash was unilateral, direct immunofluorescence demonstrated IgG antibody at the dermo-epidermal junction on both sides of the body. Indirect immunofluorescence was also positive. A suction-blister test showed increased skin fragility on the affected side.

Anticardiolipin antibody associated ischaemic strokes in elderly patients without systemic lupus erythematosus.

We describe four elderly stroke patients--two men and two women--who had significantly elevated levels of anticardiolipin antibody in the absence of systemic lupus erythematosus or other connective-tissue disease.

Demonstration of antibody and cellular immune response to brain extract in West and Lennox-Gastaut syndromes.

We investigated humoral and cellular immune response to brain tissues in 15 patients with West syndrome, in 9 patients with Lennox-Gastaut syndrome and in 20 healthy children. High levels of a precipitating antibody to a saline extract of brain tissue were detected in all patients; leucocyte migration inhibition test with the same antigen was found to be positive in most of them. The role of this autoimmune response in the pathogenesis of West and Lennox-Gastaut syndromes remains to the elucidated.

Immunological disturbance in West and Lennox-Gastaut syndromes.

Cell-mediated and humoral immunity were investigated in 18 patients with West syndrome, 12 with Lennox-Gastaut syndrome and 19 healthy controls. The study included determination of T and B peripheral blood lymphocytes, serum levels of IgG, IgA and IgM, skin sensibilization with DNCB, intracutaneous PHA, leucocyte migration inhibition test and lymphocyte blastic transformation in the presence of PHA. Cell-mediated deficiency was detected in 28 children whereas low levels of immunoglobulins were observed only in 6 children. Immunological disturbances were more prominent in children with West syndrome.

Immunologic mechanisms in multiple sclerosis. Exacerbation by type A hepatitis and skin test antigens.

A 29-year-old man with quiescent multiple sclerosis had hemiparesis during the prodrome of serologically confirmed type A hepatitis. After neurological symptoms and acute hepatitis had abated, hemiparesis again developed when skin tests were applied. While he was receiving prednisone, skin test inflammation and neurological signs cleared. One year later, lymphocyte stimulation, which had been noticeably elevated during his exacerbations, returned to low normal. Both hepatitis A and skin test antigens produced immunostimulation that resulted in exacerbation of multiple sclerosis.