1/84. enzyme therapy in gaucher disease type 2: an autopsy case.A Japanese patient with gaucher disease type 2 was treated with enzyme therapy, alglucerase, from 7 to 22 months of age. Whereas hematologic parameters were normalized and hepatosplenomegaly was alleviated, no improvement in neurologic symptoms occurred, and the patient died of respiratory failure at age 22 months. Postmortem examination revealed massive intra-alveolar infiltration of Gaucher cells in lungs and in the central nervous system, i.e., the presence of Gaucher cells in the perivascular Virchow-Robins spaces in the cortex and deep white matter and extensive lamilar necrosis with reactive proliferation of blood vessels and macrophage infiltration of the cerebral cortex. It is suggested that enzyme therapy, with thus far recommended dose, does not prevent long-term respiratory and central nervous system involvement in severe varients of Gaucher disease.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/84. Laminar cortical necrosis in central nervous system lupus: sequential changes in MR images.A 44-year-old woman with systemic lupus erythematosus developed central nervous system lupus presenting with headache, fever, cloudiness of consciousness, and psychotic symptoms. T1-weighted and proton MR images showed laminar high intensity lesions in the parietal and temporal cortex bilaterally. T2-weighted images of the lesions showed low signal intensity. Treatment with corticosteroids alleviated the clinical symptoms within 7 months. The low T2 and high T1 signal abnormalities disappeared in a year and in 5 years, respectively. The laminar cortical lesions on MR images were suggested to represent cortical necrosis associated with central nervous system lupus.- - - - - - - - - - ranking = 3keywords = nervous system (Clic here for more details about this article) |
3/84. Acute intermittent porphyria with central pontine myelinolysis and cortical laminar necrosis.Acute intermittent porphyria (AIP) is an autosomal-dominant disease caused by a deficiency of porphobilinogen (PBG) deaminase. patients with AIP present with neurological syndromes such as autonomic neuropathy, peripheral axonal neuropathy or central nervous system dysfunction. We report serial MRI of a patient with AIP who had cortical and subcortical cerebral changes. A 29-year-old woman with a 6-month history of AIP had an attack with severe hyponatraemia and generalised convulsions, treated with haem arginate and supportive therapy. MRI showed central pontine and extrapontine myelinolysis and cortical laminar necrosis. These are not common in AIP, but are likely to have been caused by rapid correction of hyponatraemia and by vasospasm, which could be induced by AIP.- - - - - - - - - - ranking = 0.5keywords = nervous system (Clic here for more details about this article) |
4/84. Necrotizing sarcoid granulomatosis mimicking an intracranial neoplasm: clinicopathologic features and review of the literature.We present a unique case of biopsy-proven necrotizing sarcoidosis involving the central nervous system (CNS) in a 52-year-old woman. The patient presented with a 3-month history of left-sided headache and sharp, shooting pains on the left side of her face. She also has a previous history of sarcoidosis, histopathologically confirmed on parotid gland biopsy 24 years before. Imaging studies of the present lesion revealed a 1.8 x 1.4-cm mass in the left temporal lobe with signal intensity suggestive of meningioma or low-grade glial neoplasm. Surgical resection was initiated, and intraoperative consultation with frozen sections revealed granulomata. The lesion was biopsied, and surgical intervention was terminated. Permanent sections failed to reveal bacteria, mycobacteria, fungi, or foreign bodies. A diagnosis of necrotizing neurosarcoidosis was rendered. The patient was administered steroid therapy and clinically responded favorably. At the most recent follow-up almost 2 years later, there was no evidence of recurrence or progression. Necrotizing sarcoidosis has been reported most commonly in the lungs and rarely in other organ systems. We report the first histologically proven case involving the CNS as well as a rare example of sarcoidosis and necrotizing sarcoid granulomatosis in the same patient. sarcoidosis and its necrotizing variant should be considered in the differential diagnosis of a granulomatous mass lesion involving the CNS, particularly in the context of a history of systemic disease.- - - - - - - - - - ranking = 0.5keywords = nervous system (Clic here for more details about this article) |
5/84. August 2000: Two cases with necrosis and hemorrhage in the putamen and white matter.The August COM: Acute methanol poisoning is an uncommon, but well-recognized, cause of central nervous system injury. We present two autopsy cases showing the classic neuropathologic injuries in acute methanol poisoning: putamen and white matter necrosis and hemorrhage. In Case 1, putamen hemorrhages were striking; white matter pathology predominated in Case 2. The precise mechanism of methanol toxicity is unclear. Direct toxicity of metabolites, particularly formic acid, as well as ischemic injury and acidosis likely play a role. methanol is readily available in many commercial products, and may be ingested accidentally or intentionally.- - - - - - - - - - ranking = 0.5keywords = nervous system (Clic here for more details about this article) |
6/84. Disseminated acanthamoebiasis presenting as lobular panniculitis with necrotizing vasculitis in a patient with AIDS.BACKGROUND: Disseminated acanthamoebiasis is a rare entity, almost exclusively occurring in the immunocompromised host. methods: We report an unusual case of a 35-year-old female with recurrent sinusitis and multiple skin nodules demonstrating a necrotizing panniculitis, shown to be due to disseminated acanthamoebiasis. RESULTS: Histologic sections showed a neutrophilic lobular panniculitis with 20- to 30-microm trophozoites consistent with acanthamoeba species. CONCLUSIONS: A review the literature shows that the histopathological presentation of acanthamoebiasis often eludes initial diagnostic attempts and that central nervous system (CNS) involvement is frequent and ultimately fatal. When amoebiasis is suspected, knowledge of the trophozoite and cyst forms may be helpful in distinguishing acanthamoeba species from entamoeba histolytica.- - - - - - - - - - ranking = 0.5keywords = nervous system (Clic here for more details about this article) |
7/84. bone marrow necrosis and refractory hellp syndrome in a patient with catastrophic antiphospholipid antibody syndrome.We describe a 22-year-old woman who developed extensive and fatal bone marrow necrosis along with involvement of liver, lung, and central nervous system during pregnancy in the background of very high titers of antiphospholipid and anti-beta2 glycoprotein antibodies. This case illustrates a rarely recognized, potentially fatal complication of aPL in the setting of pregnancy.- - - - - - - - - - ranking = 0.5keywords = nervous system (Clic here for more details about this article) |
8/84. Fatal hemorrhage in stented esophageal carcinoma: tumor necrosis of the aorta.We report a case of fatal hematemesis in a patient with an esophageal stent for palliation of malignant dysphagia. Post-mortem examination showed tumor thrombi in the vasa vasorum of the aorta causing aortic wall necrosis and perforation. Massive delayed hemorrhage is reported as a complication of metallic esophageal endoprostheses but in the majority of cases there have been no post-mortem examinations. In this case the post-mortem examination demonstrated that the cause of death was not stent-related. We feel that this case report adds important information to the current literature regarding the delayed complications seen in patients with esophageal carcinoma who have been palliated with metallic stents.- - - - - - - - - - ranking = 0.085529367229676keywords = metal (Clic here for more details about this article) |
9/84. radiation myelitis in a 5-year-old girl.Myelopathy is an uncommon complication of radiotherapy, particularly in the pediatric age group. A 5-year-old girl with acute lymphoblastic leukemia developed a severe but transient radiculopathy after intrathecal administration of methotrexate and cytarabine for an isolated central nervous system relapse. Chemotherapy was then given through an intraventricular catheter. Owing to a second central nervous system recurrence, she was treated with craniospinal radiation. The whole brain down to the level of C2 received a dose of 2400 cGy. Two months after completion of radiation, the child developed a progressive tetraparesis, and magnetic resonance imaging revealed an enhancing lesion involving the medulla and upper cervical cord. A biopsy was consistent with a treatment-related necrotizing leukoencephalopathy. This case suggests that patients who develop neurologic dysfunction when treated with methotrexate can also be particularly susceptible to radiation-related injury.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
10/84. Metastatic epithelioid sarcoma to the brain: palisaded necrosis mimicking glioblastoma multiforme.Epithelioid sarcomas are rare, morphologically distinct tumors that have a propensity to arise in the extremities. brain metastasis from epithelioid sarcoma are a relatively rare occurrence. We report a case of brain metastasis in a 50-year-old man who was previously diagnosed with an epithelioid sarcoma arising in the elbow. Before the diagnosis of brain metastasis, he had developed an axillary lymph node metastasis. He presented with neurologic symptoms of progressively worsening headache and loss of vision on the right side. He underwent gross total resection of an occipital lobe mass. Histologically, the tumor was focally characterized by prominent perinecrotic pseudopalisading and demonstrated immunoreactivity with antibodies to cytokeratin AE1/3 and CAM5.2; the tumor did not stain with glial fibrillary acidic protein antibody. The literature is reviewed and the morphologic distinction between metastatic epithelioid sarcoma and other central nervous system neoplasms is discussed.- - - - - - - - - - ranking = 0.5keywords = nervous system (Clic here for more details about this article) |
| | Next -> |