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Background: Familiality in brain tumors is not definitively substantiated. Methods: We used the Utah Population Data Base (UPDB), a genealogy representing the Utah pioneers and their descendants, record-linked to statewide cancer records, to describe the familial nature of primary brain cancer. We examined the familial clustering of primary brain tumors, including subgroups defined by histologic type and age at diagnosis. The UPDB includes 1,401 primary brain tumor cases defined as astrocytoma or glioblastoma, all with at least three generations of genealogy data. We tested the hypothesis of excess relatedness of brain tumor cases using the Genealogical Index of Familiality method. We estimated relative risks for brain tumors in relatives using rates of brain tumors estimated internally. Results: Significant excess relatedness was observed for astrocytomas and glioblastomas considered as a group (n 1,401), for astrocytomas considered separately (n 744), but not for glioblastomas considered separately (n 658). Significantly increased risks to first- and seconddegree relatives for astrocytomas were identified for relatives of astrocytomas considered separately. Significantly increased risks to first-degree relatives, but not second degree, were observed for astrocytoma and glioblastoma cases considered together, and for glioblastoma cases considered separately. Conclusions: This study provides strong evidence for a familial contribution to primary brain cancer risk. There is evidence that this familial aspect includes not only shared environment, but also a heritable component. Extended high-risk brain tumor pedigrees identified in the UPDB may provide the opportunity to identify predisposition genes responsible for familial brain tumors. Neurology ® 2008;71:1015–1020

A 67-year-old man sequentially developed anti-Ma2-associated paraneoplastic encephalitis (PNE) and contralateral herpes simplex encephalitis (HSE). Brain biopsy 1 month before HSE revealed extensive infiltrates of T cells, B cells, and plasma cells. Most T cells expressed the cytotoxic granule-associated protein TIA-1 and the membranolytic protein granzyme-B. Although recovery was thought to be unlikely, treatment of the PNE with corticosteroids and resection of the associated lung cancer resulted in dramatic improvement for 21 months.

Capecitabine is used to treat advanced breast and gastrointestinal malignancies. A single case of encephalopathy and three cases of peripheral neuropathy are the only neurotoxicities reported. The authors report five additional cases of capecitabine-induced multifocal leukoencephalopathy.

We report MRI changes associated with chronic active varicella zoster virus (VZV) ganglionitis. A 28-year-old woman with metastatic breast cancer was treated with intrathecal methotrexate for recurrent neoplastic meningitis. Six weeks earlier, she had completed total brain irradiation for dural metastases. In late January 2003, she developed left-sided low back pain. Neurologic examination 3 weeks later was normal. On February 21, 2003, she developed L2/3 distribution zoster and was treated with famciclovir, 500 mg three times daily for 12 days, with improvement of pain and rash. On March 12, 2003, she developed severe pain radiating into the left hip and groin, which was worse with walking. Neurologic examination revealed mild iliopsoas weakness bilaterally and reduced left patellar reflex. Sensation was normal, and straight leg raising was negative. A lumbar spine MRI (1.5-T scanner) with contrast revealed enhancement of the left L2 ganglion that extended laterally into the nerve roots and psoas muscle (figure). The CSF contained 127 cells/mL, 30% malignant. PCR did not detect amplifiable VZV or herpes simplex virus (HSV) DNA in CSF. Enzyme immunoassay1 revealed immunoglobulin (Ig) M and IgG antibody to VZV but not to HSV in CSF; the serum-to-CSF ratio of anti-VZV IgG was 25 compared with 487 for …

Perineural lumbosacral plexus infiltration is an unusual manifestation of systemic lymphoma that is difficult to diagnose using conventional imaging techniques. We report a case of lymphomatous lumbosacral plexus infiltration that was occult on lumbar MR and abdominopelvic CT imaging. High-resolution MR neurography (MRN) demonstrated a diffusely infiltrating lumbar plexus lesion, suggesting neurolymphomatosis (NL), subsequently confirmed at autopsy. MRN is a sensitive modality for characterizing plexus and peripheral nerve abnormalities1,2⇓ and may be diagnostic after inconclusive conventional MRI. We review NL and demonstrate the diagnostic utility of MRN. A 69-year-old man presented with stage IV thoracic B-cell lymphoma. Four cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) followed by cyclophosphamide produced a full response. One month after completing chemotherapy, he presented again with nocturnal right leg pain exacerbated by lying supine or by extended periods of sitting. The pain became constant and intensified in severity, and examination revealed new bilateral pedal edema. There was severe weakness of the right psoas, quadriceps, and gluteal muscles with moderate involvement of adductor and hamstring muscles. Distal leg …

Objective: To report a series of HIV-infected patients with intracranial tumors not known to be associated with immunodeficiency.Background: The spectrum of HIV-associated diseases is changing with improved treatments and prolonged patient survival. Although primary central nervous system lymphoma (PCNSL) and toxoplasmosis continue to be the most common intracranial lesions in HIV-infected patients, the recognition of other pathologic entities is increasingly important.Methods: The clinical characteristics and outcome of eight HIV-infected patients with nine intracranial neoplasms other than PCNSL are reported. In addition, all available pathologic specimens were tested for evidence of either HIV or Epstein-Barr virus (EBV) infection. An additional 28 patients reported in the literature are summarized.Results: Five of eight patients had a glioblastoma multiforme; other tumors included an anaplastic ependymoma, a low-grade glioma, a subependymoma, and a leiomyosarcoma. More than half of the patients developed their tumor ≥6 years after the diagnosis of HIV infection. Patient prognosis and survival was best predicted by tumor histology. Treatment response and outcome did not appear to be influenced by HIV infection. Only the leiomyosarcoma demonstrated evidence of latent EBV infection.Conclusions: HIV-infected patients are at risk for intracranial neoplasms other than PCNSL, and benefit from aggressive tumor-specific therapy. It is possible that gliomas are occurring at a higher rate than in the general population. There was no evidence of HIV or EBV infection in any glial tumor.