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11/48. Primary angiitis of the central nervous system: an ante-mortem diagnosis.

    A rare case of primary angiitis of the central nervous system (PACNS) is reported with its clinical and magnetic resonance imaging (MRI) features. A 20-year-old girl presented with headache, projectile vomiting, unsteadiness of gait and urgency of micturition. She had left seventh nerve upper motor neuron type paresis, increased tone in all four limbs, exaggerated deep tendon reflexes, cerebellar signs, and papilloedema. cerebrospinal fluid showed lymphocytosis with elevated protein and normal glucose level. Cerebral computerised tomographic scan and MRI showed bilateral diffuse asymmetric supra- and infra-tentorial lesions (predominantly in the supratentorial and left cerebrum). On MRI, the lesions were hyperintense on T2, and proton density-weighted images and hypointense on T1-weighted images. Based on the clinical findings of raised intracranial tension and MRI features, initial diagnoses of gliomatosis cerebrii, tuberculous meningitis, primary central nervous system lymphoma and chronic viral encephalitis were considered. PACNS was not included in the initial differentials and, an open brain biopsy was advised which established the definitive diagnosis.
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ranking = 1
keywords = nervous system, nerve
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12/48. Intracranial vasculitis and multiple abscesses in a pregnant woman.

    Cerebral vasculitis is an unusual disorder with many causes. Infectious causes of cerebral vasculitis are predominantly bacterial or viral in nature. Purulent bacterial vasculitis is most often a complication of severe bacterial meningitis. The patient is a 25-year-old African American female, 25 weeks pregnant, who presented to the neurology service after a consult and referral from an outside hospital. She had a 1-month history of right sixth nerve palsy. Initial workup included a negative lumber puncture and a noninfused magnetic resonance imaging (MRI). Three days later, the patient developed right-sided migraine headaches and right third nerve palsy. The angiogram revealed diffuse irregularity and narrowing of the petrous, cavernous, and supraclinoid portions of the internal carotid and right middle cerebral arteries. Shortly thereafter, an MRI examination revealed diffuse leptomeningeal enhancement and abscess and a right parietal subdural empyema. Infectious vasculitis secondary to purulent meningitis has a rapidly progressive course and presents with cranial nerve palsy with involvement of the cavernous sinus. Although the association of this disease with pregnancy has not been established, it should be recognized that the early imaging studies may be negative or discordant and follow-up imaging might be necessary.
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ranking = 2.7604501488603E-5
keywords = nerve
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13/48. Postvaricella angiopathy: report of a case with pathologic correlation.

    Varicella is a common childhood illness, and central nervous system complications occur frequently. Delayed angiopathy has been described, although there are few reports of clinicopathologic correlation. A previously well 4-year-old male is presented. He suffered varicella 2 months before presentation with extensive right middle cerebral artery (MCA) territory infarction. cerebral angiography demonstrated an isolated 89% stenosis of the right proximal MCA. He developed cerebral edema refractory to medical treatment and progressed to transtentorial herniation. Right frontal temporoparietal craniotomies were performed with evacuation of infarcted brain tissue. Pathologic studies revealed small vessel vasculitis with lymphocytic infiltration of the vessel wall. Areas of demyelination were present within the white matter. polymerase chain reaction for varicella was negative on brain tissue. Postvaricella angiopathy, although an uncommon complication, may affect both small and large blood vessels, with catastrophic results.
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ranking = 0.16666513308325
keywords = nervous system
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14/48. Tumor-like multiple sclerosis (MS) lesions: neuropathological clues.

    The neuroradiological evidence of a single, large white matter lesion with mass effect, clinically revealed by signs of endocranial hypertension, is highly suspicious for central nervous system neoplasm. In rare cases, a demyelinating disorder can start with atypical features suggestive of a brain tumor; in these cases a brain biopsy is often carried out. We report our experience regarding cases of multiple sclerosis (MS) with atypical tumor-like presentation. None of our patients underwent biopsy. Serial magnetic resonance imaging performed during steroid treatment, together with other paraclinical data, were sufficient for the final diagnosis of MS. These cases are characterized by a severe clinical course and a rapid clinical deterioration, only partially modified by medical treatments. Atypical severe cases, misdiagnosed as MS, can be indeed due to primary CNS vasculitis.
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ranking = 0.16666513308325
keywords = nervous system
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15/48. Recurrent stroke as a manifestation of primary angiitis of the central nervous system in a patient infected with human immunodeficiency virus.

    CONTEXT: Cerebral vasculitis in patients infected with human immunodeficiency virus (hiv) is usually related to additional or secondary infectious agents other than neoplastic diseases or hiv itself. OBJECTIVE: To describe a 31-year-old patient infected with hiv who presented with 2 recurrent, acute episodes of neurologic impairment in a 5-month period. DESIGN: Comparison of clinical and histologic data between the present case and previously published cases. SETTING: Community hospital. PATIENT: A 31-year-old, hiv-infected patient with recurrent strokes and chronic lymphocytic meningitis. INTERVENTION: After ruling out cardiac embolisms and coagulation disorders, the presence of central nervous system vasculitis, probably secondary to an infectious process, was suspected based on the clinical examination and cerebrospinal fluid abnormalities. RESULTS: Necropsy findings suggest the diagnosis of primary angiitis of the central nervous system, and the only infectious agent that could be found was hiv. CONCLUSIONS: Histologic studies were compatible with a diagnosis of primary angiitis of the central nervous system, but the pathogenic role of hiv in the genesis of the vasculitic process cannot be elucidated.
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ranking = 1.1666559315828
keywords = nervous system
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16/48. Granulomatous angiitis of the central nervous system associated with herpes zoster.

    Granulomatous angiitis of central nervous system (CNS) is a rare inflammatory disease of blood vessels mostly confined to CNS. We describe a case which presented with right sided hemiplegia with aphasia, after herpes zoster ophthalmicus. CT scan and MRI brain showed a large left sided infarct in the left middle cerebral artery (MCA) territory. MRI angiography revealed narrowing and thinning of left internal carotid artery (ICA) and to a lesser extent, left MCA suggestive of granulomatous vasculitis. Herpes zoster is often associated with major CNS involvement and a vascular etiology was previously postulated. Recent pathological reports suggest that cerebral angiitis secondary to herpes virus infection may be more common than realised.
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ranking = 0.83332566541625
keywords = nervous system
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17/48. Follow-up examinations by transcranial Doppler ultrasound in primary angiitis of the central nervous system.

    BACKGROUND: Primary angiitis of the central nervous system (PACNS) is a rare disease. The definite diagnosis is made upon proof of mononuclear inflammation of the vessel wall on brain biopsy. The diagnosis can also be established on clinical grounds, typical findings on intra-arterial angiography and other investigatory grounds excluding other diseases. Therapy comprises an aggressive immunosuppressive approach. Close monitoring of the patients is mandatory. Transcranial Doppler ultrasound (TCD) has not yet been used to follow up the vasculitic lesions in PACNS. CASE: We report on a 32-year-old female with massive cerebral infarctions secondary to multiple large-vessel stenoses because of probable PACNS. The patient was followed closely by means of TCD. During therapy the cerebral blood flow velocities normalized as displayed by TCD. Clinical improvement followed several days after normalization of cerebral blood flow. CONCLUSIONS: TCD is a valuable noninvasive bedside tool to monitor cerebral blood flow velocities and therapy response in patients with cerebral vasculitis, if large arteries are involved.
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ranking = 0.83332566541625
keywords = nervous system
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18/48. Primary angiitis of the central nervous system and silent cortical hemorrhages.

    Primary angiitis of the CNS is histopathologically characterized by ischemic lesions and small petechial hemorrhages. Unlike CT or conventional MR imaging, gradient-echo MR imaging depicts these chronic petechial hemorrhages. We herein report the case of biopsy-proved primary angiitis of the CNS in a 42-year-old man; whom gradient-echo MR imaging revealed multiple petechial hemorrhages in the cortical-subcortical brain regions. The identification of petechial hemorrhages by gradient-echo MR imaging promises to be a valuable surrogate marker supporting the diagnosis of primary angiitis of the CNS.
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ranking = 0.666660532333
keywords = nervous system
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19/48. Acute encephalopathy with parvovirus B19 infection in sickle cell disease.

    A 13 year old girl with haemoglobin Sbeta( )thalassaemia developed simultaneous aplastic crisis and encephalopathy associated with parvovirus B19 (PB19) infection. brain magnetic resonance imaging findings were consistent with central nervous system (CNS) vasculitis and her symptoms resolved with steroid therapy. Thus, PB19 induced CNS hypersensitivity vasculitis must be considered in the differential diagnosis of encephalopathy.
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ranking = 0.16666513308325
keywords = nervous system
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20/48. vasculitis in a dog resembling isolated angiitis of the central nervous system in humans.

    An 1-year-old dog succumbed to a seizure disorder of 18 days duration. At necropsy, an area of hemorrhage and discolored parenchyma was identified in the left pyriform lobe of the brain. Microscopic examination revealed a localized, necrotizing vasculitis with associated cerebral necrosis. vasculitis was not present in other organs. This presentation is consistent with isolated central nervous system (CNS) angiitis, a rare form of vasculitis in humans.
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ranking = 0.83332566541625
keywords = nervous system
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