1/18. Isolated central nervous system vasculitis associated with hepatitis c infection.Since its identification in 1989, hepatitis c has been implicated in the pathogenesis of an increasing number of diseases previously believed to be primary or idiopathic. We report 2 rarely seen cases of isolated central nervous system (CNS) vasculitis in patients with hepatitis c infection. Patient 1. A 43-year-old man with 4 day right temporal headache developed a left hemiparesis. Weakness was his only physical finding. Computed tomography (CT) scan demonstrated a large right frontotemporal hemorrhage, and angiography revealed focal dilatations and irregularities of multiple branches of the right middle and anterior cerebral arteries. Cerebral decompression was performed and leptomeningeal biopsies showed granulomatous angiitis. Laboratory results were normal except for elevated liver biochemical tests. Later testing for hepatitis c was positive. His neurological symptoms improved with corticosteroids and cyclophosphamide. Patient 2. A 39 yr old male developed 3 days of left sided weakness, slurred speech and difficulty swallowing fluids. Physical findings were limited to his weakness. magnetic resonance imaging demonstrated a right superior pontine subacute infarct with a small left internal capsule lacunar infarct. angiography revealed multiple areas of focal narrowing with no areas of abrupt vessel cut off. Cerebral spinal fluid showed 71 PMN, 29 RBC, normal glucose, elevated protein (64 mg/dl), no oligoclonal bands, and low myelin basic protein. Other laboratory analyses were normal including liver biochemical tests. However, hepatitis c serology was positive and mixed cryoglobulins were detected. CNS vasculitis was diagnosed and nearly full recovery was achieved with corticosteroids, cyclophosphamide and warfarin.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
2/18. Primary angiitis of the central nervous system as a first presentation in Hodgkin's disease: a case report and review of the literature.OBJECTIVE AND IMPORTANCE: Granulomatous angiitis of the central nervous system is a rare cause of neurological deterioration. It is often diagnosed posthumously, and a high index of suspicion is necessary to make the correct diagnosis on a timely basis. CLINICAL PRESENTATION: A 27-year-old woman presented to the emergency room with complaints of worsening headache, nausea, and vomiting for 10 days, which were preceded by swelling of her tongue. At the examination, she had mild ocular tenderness, but no cranial nerve abnormalities. Radiographic examination revealed a right temporal lobe area with edema, and mild contrast enhancement was noted on computed tomography and magnetic resonance imaging. A similar but smaller region was present in the left frontal lobe. INTERVENTION: Stereotactic biopsy of the left temporal lobe revealed granulomatous angiitis. Further workup revealed Hodgkin's disease in the mediastinum. dexamethasone as well as chemotherapy for Hodgkin's disease was initiated. The patient's symptoms resolved, and she returned to work with her disease in remission. CONCLUSION: Previous reports of central nervous system angiitis have shown an association with sjogren's syndrome, herpes zoster infection, human immunodeficiency virus, and Hodgkin's disease. A review of the literature revealed a total of 12 patients with central nervous system angiitis and Hodgkin's disease. As a group, these patients had very poor outcomes. However, of six patients who presented with central nervous system angiitis and concurrent Hodgkin's disease and who underwent aggressive treatment for both conditions, three had a full recovery, two had a partial recovery, and one died.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
3/18. Primary angiitis of the central nervous system and Alzheimer's disease: clinically and pathologically evident in a single patient.Two years after being successfully treated for biopsy confirmed primary angiitis of the central nervous system (PACNS), a 69-year-old woman presented with cognitive decline. In contrast to her first presentation, her altered mental function developed gradually, was not associated with headache or abnormal cerebrospinal fluid analysis, and did not improve with immunosuppression. Reevaluation of her original brain biopsy not only confirmed the presence of PACNS, but also revealed neuritic plaques and neurofibrillary tangles, suggesting a concurrent diagnosis of Alzheimer's disease. Cerebral angiogram did not suggest vasculitis and magnetic resonance imaging showed generalized cerebral atrophy supporting the diagnosis of Alzheimer's. This case illustrates that Alzheimer's dementia and PACNS can coexist in a single patient and that Alzheimer's disease should be considered when a patient with successfully treated PACNS presents with cognitive decline months or years after initial diagnosis.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
4/18. Primary angiitis of the central nervous system in children: 5 cases.We describe 5 children who meet criteria for primary angiitis of the central nervous system (PACNS). All patients presented with headache and/or focal neurologic deficits and exhibited clinical and/or radiographic evidence of disease progression. Two patients had disease progression prior to combined treatment with cyclophosphamide and corticosteroids; one progressed while receiving intravenous cyclophosphamide and stabilized after a change to daily oral dosing; one progressed after discontinuing therapy after less than 12 months and improved after retreatment; and one progressed on steroid therapy alone but was lost to followup. Children who have frequent or severe headaches or focal neurologic deficits should be carefully evaluated and those meeting criteria for PACNS should be treated aggressively.- - - - - - - - - - ranking = 2keywords = headache (Clic here for more details about this article) |
5/18. Isolated angiitis of the CNS in children.OBJECTIVE: To clarify the clinical features and pathologic manifestations of isolated angiitis of the CNS (IACNS) in children. methods: The authors report two new cases and summarize the literature of childhood IACNS confirmed by pathology. RESULTS: IACNS affecting small vessels (n = 5). neurologic manifestations included headaches, focal seizures, and progressive, behavioral, or multifocal neurologic impairment. MRI showed multifocal, T2-hyperintense, cerebral lesions without mass effect or tumor-like lesions. CSF, erythrocyte sedimentation rate, and cerebral angiograms were often normal.CNS biopsy disclosed a nongranulomatous vasculitis. Children were treated with prednisone alone or combined with cyclophosphamide. One child died. Four children had a favorable outcome. IACNS affecting large and medium arteries (n =5). Three children presented with acute ischemic stroke or TIA. Brain CT showed ischemic infarcts. Two children presented with subarachnoid hemorrhage. In this group, CSF, erythrocyte sedimentation rate, and angiograms were often abnormal. No patient received immunosuppressive therapy. Five children died. autopsy showed granulomatous IACNS (n =5). CONCLUSIONS: Clinical and radiologic features correlate with the size of affected vessels. prognosis differs between groups. Potential markers of poor outcome are acute stroke presentation secondary to large and medium-sized artery involvement, granulomatous angiitis, and delayed institution of immunosuppressive therapy.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
6/18. 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.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
7/18. 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.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
8/18. Mycotic cerebral vasculitis in a paediatric cardiac transplant patient excludes misadventure.We present the case of a 10-year-old girl with cardiomyopathy who received a heart transplant. Due to organ rejection, the dosage of immunosuppressive agents was increased postoperatively. The patient complained of intermittent headaches in the following days and developed a haemorrhagic necrosis of the left thalamus. A week later, an oral dose of cyclosporin A was accidentally given intravenously, and 2 weeks later a recurrent subarachnoid haemorrhage of unknown origin was diagnosed. The clinical course was then characterised by progressive deterioration resulting in coma, fluctuating brain stem symptoms and the development of a massive cerebral oedema with subsequent brain death. A coroner's autopsy was instigated to investigate a claim of medical misadventure. Neuropathological investigations found a focal infiltration of fungal hyphae in the left posterior cerebral artery resulting in necrosis of the vascular wall and thus explaining the source of the recurrent subarachnoid haemorrhage which eventually resulted in the girl's death. Medical misadventure due to the administration of cyclosporin was not directly responsible for the death of this patient. This case illustrates that it is of paramount importance to copiously sample and investigate the basal cerebral arteries in cases of subarachnoid haemorrhage of unknown origin, in particular in a medico-legal context.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
9/18. Late-developing cerebral arteropathy after pyogenic meningitis.BACKGROUND: Although vasculopathy is a recognized complication during acute meningitis, to our knowledge, no previous reports have been published of this phenomenon developing months after successful treatment. OBJECTIVE: To report a unique case of a late-developing vasculopathy after pyogenic meningitis in an adult. REPORT OF A CASE: A 51-year-old woman was seen with severe headache 2 months after treatment of haemophilus influenzae type C meningitis. Initial arteriography showed no abnormality; a second arteriogram showed progressive multifocal intracranial stenosis affecting mainly the internal carotid arteries. Findings from pathologic examination disclosed diffuse collagenosis consistent with chronic vascular injury from meningitis. The arterial lesions stabilized, and the patient remained asymptomatic. CONCLUSION: Progressive intracranial arterial stenosis can evolve months after meningitis and should be added to the list of recognized vascular complications.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
10/18. Postpartum cerebral angiopathy: an important diagnostic consideration in the postpartum period.A 32-year-old woman, gravida 4, para 2, presented with a severe headache 5 days after she had a vaginal delivery with epidural anesthesia. Nine days later she had neurologic symptoms develop. magnetic resonance imaging and cerebral angiogram were abnormal with evidence of a vasculitis consistent with postpartum cerebral angiopathy. The patient received both methylprednisolone and cyclophosphamide pulses with improvement of her symptoms. This is an important diagnosis for obstetricians to consider because it may result in serious neurologic damage if it is not diagnosed and treated early.- - - - - - - - - - ranking = 1keywords = headache (Clic here for more details about this article) |
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