Neuro-Sweet disease: clinical manifestations and criteria for diagnosis.

BACKGROUND: Sweet disease, also known as acute febrile neutrophilic dermatosis, is a multisystem inflammatory disorder characterized by painful erythematous plaques and aseptic neutrophilic infiltration of various organs. Skin biopsies typically demonstrate dermal infiltration with neutrophils in the absence of vasculitis. Sweet disease responds to systemic corticosteroids. The CNS can also be involved. METHODS: The authors performed a survey on neuro-Sweet disease (NSD) in Japan and obtained detailed information about 16 cases. They analyzed 42 cases, including 26 cases documented in the literature, and assessed clinical and laboratory criteria for the diagnosis. RESULTS: Thirteen cases also fulfilled the criteria for the diagnosis of Behcet disease. The clinical features of 27 cases, which the authors classified as probable NSD, are as follows: 1) both sexes are almost evenly affected; 2) people of ages 30 to 70 years are affected; 3) encephalitis and meningitis are common neurologic manifestations; 4) any region of the CNS can be involved, resulting in a variety of neurologic symptoms; 5) there is a strong human leukocyte antigen-Cw1 association; 6) systemic corticosteroids are highly effective for most of the neurologic manifestations, although recurrences are not infrequent. CONCLUSIONS: Neuro-Sweet disease is a distinct entity that may account for some cases of idiopathic encephalomeningitis.

Non-traumatic recurrent dissection and its spontaneous repair in the circle of Willis: report of two autopsy cases.

Post-mortem examinations of the circle of Willis in two cases of subarachnoid hemorrhage disclosed a wide spectrum of vasculopathy ranging from a minimal tear between the intima and media, and between the media and adventitia, to complete transmural disruption leading to the formation of pseudoaneurysms. The presence of coexistence of the focal lesions with complete replacement of the entire arterial wall with thick fibrous connective tissues and the vasculopathy was suggestive of the spontaneous repair of recurrent non-traumatic dissection of intracranial arteries. The patients were 58-year-old and 43-year-old females. There was no history of injury to the head or neck in either case. They were hypertensive, but the degree of atherosclerotic changes in the circle of Willis was compatible with age. There was no histological evidence of vasculitis. The role of hypertension and medial mucoid degeneration in the genesis of non-traumatic dissection of intracranial arteries was discussed.

Anaplastic ganglioglioma with sarcomatous component: an immunohistochemical study and molecular analysis of p53 tumor suppressor gene.

The present case report describes a case of ganglioglioma with a distinct sarcomatous component in the left temporal lobe of a 59-year-old Japanese man. Neoplastic neuroglial tissue contained both benign and anaplastic glial components with a MIB-1 labeling index of 0.1% and 12.0%, respectively. Sarcomatous tissue adjacent to the anaplastic glial tissue was dominated by pleomorphic fibroblastic cells with a MIB-1 labeling index of 10.8%. They were immunoreactive for smooth muscle actin, type IV collagen, and alpha 1 antitrypsin, but not for desmin and CD34. Interestingly, some of the sarcomatous cells were double-positive for smooth muscle actin and GFAP. The p53 protein had accumulated in the anaplastic astrocytes and sarcomatous cells, but direct DNA sequencing of PCR products failed to detect any mutation in the p53 gene (from exon 4 to exon 10).

A case of angioglioma composed of astrocytoma with a papillary growth pattern: immunohistochemical and ultrastructural studies.

We report a case of a large cystic astrocytoma associated with arteriovenous malformation in the right cerebral hemisphere of a 16-year-old boy. Neuroimaging showed large abnormal vessels with flow voids and arteriovenous shunt around the cystic lesion. Histologically, the cyst wall was formed by abnormal vasculature and clusters of glial cells forming a papillary growth pattern. The abnormal vasculature consisted of dilated vein-like vessels and medium-sized arteries with incomplete media, and was diagnosed as an arteriovenous malformation. Immunohistochemically, glial fibrillary acidic protein (GFAP) decorated both the perikaryon and the processes of the glial tumor cells. They were negative for epithelial membrane antigen (EMA), cytokeratin, and S-100 protein. Ultrastructurally, the tumor cells were rich in intermediate filaments, and neither cilia, microvilli, nor ependymal rosettes were verified. Based on these morphological features and the low MIB-1 labeling index of 0.8%, the glial tumor was diagnosed as astrocytoma, Grade II, according to the World Health Organization (WHO) tumor classification. An association of glioma with various types of vascular anomalies has been designated as angioglioma. A unique feature of the present case, however, is a papillary growth pattern, which is not listed in the current WHO classification of brain tumors. The recognition of the occurrence of such cases would be important in differential diagnosis of papillary ependymoma and choroid plexus papilloma.