Fatal encephalitis due to BK virus in a patient with common variable immunodeficiency: a case report.

Encephalitis due to BK virus is a rare condition. Here, we describe a young male patient with common variable immunodeficiency who developed fatal encephalitis due to BK virus. The patient presented initially with ocular symptoms that were followed by behavioral changes and spastic quadriparesis. Diagnosis was made by the compatible clinical findings and detection of viral DNA by polymerase chain reaction in the cerebrospinal fluid. To the best of our knowledge, this is the first report of BK virus encephalitis in a patient with common variable immunodeficiency. We suggest that BK virus should be suspected in cases of encephalitis; particularly in patients with immunodeficiency.

Pituitary abscess presenting two years after the diagnosis of a pituitary lesion in a patient with panhypopituitarism.

Pituitary abscess is a rare condition. Here, we present the case of a young male patient who was initially found to have a pituitary lesion following the diagnosis of panhypopituitarism. Two years later, he presented with severe headache and was subsequently diagnosed intraoperatively with pituitary abscess. At a follow-up of 6 years after surgery, the patient was continuing to do very well. We discuss the differential diagnosis and demonstrate the evolution of the pituitary lesion on magnetic resonance imaging at four different time points: at the time of the detection of the initial lesion; two years later at the time of the diagnosis of the pituitary abscess; at 7 weeks post operatively; and finally after six years from the pituitary surgery.

Effect of disease-modifying therapies on brain volume in relapsing-remitting multiple sclerosis: results of a five-year brain MRI study.

OBJECTIVE: To compare the long-term effect of disease-modifying therapies (DMT) on brain volume loss in relapsing-remitting MS (RRMS) patients. METHODS: We conducted a study to examine the effect of daily glatiramer acetate (GA), weekly low dose interferon beta (LD-IFNB), and high-dose high-frequency interferon beta disease (HD-IFNB) on brain volume loss over 5 years in RRMS patients. All patients were previously treatment naïve, had disease duration ≤5 years at the time of initiating DMT, and subsequently received the same DMT for 5 years continuously. The percentage change in brain volume (PCBV) was measured using fully automated software. MRI analysis was performed blinded to treatment allocation. RESULTS: The adjusted PCBV from baseline to year 5 was -2.27% in GA, -2.62% in LD-IFNB, and -3.21% in the HD-IFNB groups (-2.27 vs -2.62, p=0.0036; -2.27 vs -3.21, p<0.0001; -2.62 vs -3.21, p<0.0001). These data remained unchanged from year 1 to year 5, after adjusting for pseudoatrophy in the first year. A group of RRMS patients that remained untreated for a period ranging from 8 to 24 months, served as controls. All treatment groups were significantly better than the rate of projected brain volume loss in the untreated group over 5 years (p<0.0001). CONCLUSIONS: Global brain volume loss is a dynamic process even in relatively early RRMS patients that occurs despite intervention with therapy. However, all DMT significantly reduced the loss of brain volume compared to no treatment. The GA-treated group experienced the least reduction in brain volume over 5 years, compared to the LD-IFNB and HD-IFNB treated groups. These differences could be partly related to the immunologic consequences of GA therapy in RRMS.

Mondini malformation associated with diastematomyelia and presenting with recurrent meningitis.

The authors report the case of 5-year-old girl who presented with 4 episodes of recurrent meningitis. Her initial workup revealed a lumbosacral dermoid sinus associated with diastematomyelia and a tethered cord. Therefore, a surgical repair to correct the anomaly was performed. However, another episode of meningitis occurred after surgery, and a subsequent temporal bone scan revealed the presence of left Mondini dysplasia. To the authors' knowledge, this is the first report of Mondini dysplasia in association with diastematomyelia.

Progressive multifocal leukoencephalopathy and relapsing-remitting multiple sclerosis: a comparative study.

OBJECTIVE: To identify clinical and magnetic resonance imaging (MRI) features that distinguish progressive multifocal leukoencephalopathy (PML) from relapsing-remitting multiple sclerosis (RRMS). DESIGN: Retrospective medical record review. SETTING: Two urban teaching hospitals in Detroit, Michigan. Patients Forty-five confirmed PML cases and 100 patients with RRMS. MAIN OUTCOME MEASURES: Clinical and MRI features distinguishing PML from RRMS. RESULTS: Overall, monosymptomatic presentations were more common in multiple sclerosis (MS) than PML (85% vs 47%; P < .01). However, patients with PML presented more often with hemiparesis (24% vs 5%; P = .001) and altered mentation (19% vs 0%; P < .0001), whereas brainstem (2% vs 18%; P = .007) presentations were more common in patients with RRMS. Spinal cord and optic neuritis presentations were seen in 18% and 33% of patients with RRMS, respectively, but not in patients with PML (m < .0001). Brain MRI scans, available in 35 (78%) PML cases, revealed 7 lesion types. Large, confluent T2-weighted lesions (74% vs 2%; P < .0001) and deep gray matter lesions (31% vs 7%; P < .001) were more frequent in patients with PML than patients with RRMS. Crescentic cerebellar lesions (23% vs 0%; P < .001) were seen only in patients with PML. Gadolinium-enhancing (23%), transcallosal (9%), and periventricular (9%) lesions were noted in patients with PML. Brain magnetization transfer ratio (MTR) was low in both PML and MS lesions. However, normal-appearing brain tissue MTR in PML was higher than normal-appearing brain tissue MTR in RRMS (44.15% vs 41.04%; P = .002), suggesting that PML may be relatively more focal than MS. CONCLUSIONS: There appear to be differences between the clinical and MRI characteristics of PML and RRMS, which may help distinguish new MS activity from PML. Magnetization transfer ratio studies may provide additional clues in improving early detection of PML in patients with preexisting MS and warrant further investigation.

Long-term study of brain 1H-MRS study in multiple sclerosis: effect of glatiramer acetate therapy on axonal metabolic function and feasibility of long-Term H-MRS monitoring in multiple sclerosis.

Glatiramer acetate (GA) has several putative mechanisms of action with the potential of limiting sublethal axonal injury in the central nervous system (CNS). Brain proton magnetic resonance spectroscopy ((1)H-MRS) allows in vivo examination of axonal integrity by quantifying the neuronal marker N-acetylaspartate (NAA), often expressed as a ratio to creatine (Cr). We showed that treatment with GA led to improvement in NAA/Cr over a 2-year period. We now report the results of this ongoing study after 4 years of annual brain (1)H-MRS examinations. Compared to baseline, at year 4, patients receiving continuous GA therapy showed a 12.7% increase in NAA/Cr and (P= .03) in the multivoxel brain volume of interest (VOI) studied and by 9.6% (P= .04) in the normal-appearing white matter within the VOI. Three patients in the control group who began therapy with GA during the course of the study showed similar increases in NAA/Cr after the first year of therapy. These data support the long-term effect of GA on maintaining axonal metabolic function and protection from sublethal injury as well as the feasibility of employing brain (1)H-MRS in long-term investigative studies in MS.

Acute transverse myelitis with normal brain MRI : long-term risk of MS.

OBJECTIVE: To investigate the long-term risk of developing MS in patients presenting with acute transverse myelitis (ATM) and normal brain MRI scans at onset. METHODS: We studied 58 ATM patients with normal brain MRI at presentation for up to 5 years with serial neurologic and imaging studies. All patients underwent CSF analysis at onset which was defined positive if two or more IgG oligoclonal bands and/or elevated IgG index were present. Brain and spinal cord MRI scans were obtained every 6 months for the first 2 years, and annually thereafter unless the patient experienced a second neurologic attack different from the initial episode to confirm CDMS or there was demonstration of MRI lesions confirming dissemination in time and space to fulfill McDonald imaging criteria to diagnose MS. RESULTS: Seventeen of 58 (29%) patients developed MS of which 7 (41%) patients developed CDMS and 10 (59%) developed MS using McDonald Imaging Criteria. Mean time to CDMS by a second clinical attack was 11. 1 months compared to 19. 2 months by MRI lesions (P = 0. 03). None of the patients developed MS after 24 months of onset. All 17 patients who developed MS had positive CSF although 15 patients who had positive CSF did not develop MS during the 5 years of follow-up. CONCLUSIONS: The majority of patients with ATM and normal brain MRI do not develop MS after 5 years of follow-up confirming the relatively low risk compared to patients with abnormal brain MRI scans. CSF is helpful in distinguishing patients more likely to develop MS. Compared to clinical attacks, serial imaging may not lead to an earlier diagnosis in ATM patients with normal brain MRI.

Leptomeningeal metastasis in a patient with squamous cell carcinoma of the uterine cervix: Report of a case and review of the literature.

Leptomeningeal spread of advanced squamous cell carcinoma (SCC) of the uterine cervix is unusual. To our knowledge, very few cases were reported in the English literature. We report a case of a 39-yr-old woman who presented with leptomeningeal metastasis from a stage IIb SCC of uterine cervix. The diagnosis was made by cytomorphological examination of smears obtained from cerebrospinal fluid. The cytomorphological features are demonstrated.

Imaging of neurologic disorders associated with pregnancy and the postpartum period.

Diverse pathologic conditions affect the central nervous system (CNS) and pituitary gland during pregnancy and the puerperium. Some are specific to the physiologic process of reproduction (eg, eclampsia, postpartum cerebral angiopathy, Sheehan syndrome, lymphocytic adenohypophysitis). Others are nonspecific but occur more often in pregnant women (eg, cerebral infarction, dural venous thrombosis, pituitary apoplexy). Recognition of the characteristic imaging findings in eclampsia, for example, may allow exclusion of other disorders. Even when imaging changes are nonspecific, knowledge of those entities associated with pregnancy and awareness of the increased likelihood of certain diseases in pregnancy will allow a more informed differential diagnosis. Differentiation of primary nonaneurysmal subarachnoid hemorrhage (SAH) from aneurysmal SAH is an example. Moreover, earlier use of imaging will result in fewer delayed diagnoses. For example, magnetic resonance venography allows early diagnosis of cerebral venous thrombosis. Even when the imaging changes are less specific, knowledge of likely possibilities will lead to more appropriate earlier use of imaging. For example, the stimulatory effects of pregnancy on prolactinoma, meningioma, hemangioblastoma, vestibular schwannoma, and metastatic tumors such as breast cancer and choriocarcinoma suggest the early use of CNS imaging to avoid the consequences of a delayed diagnosis.

Retroperitoneal malignant peripheral nerve sheath tumor: evaluation with serial FDG-PET.

Retroperitoneal malignant peripheral nerve sheath tumor (MPNST), a rare type of neurogenic tumor, was diagnosed in a 14-year-old girl with a history of neurofibromatosis type 1 (NF1). Immunochemistry demonstrated spindle cells positive for S-100 protein. The patient had multiple tumor recurrences and she was evaluated with serial F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET). A tumor in the right iliac wing showed increased FDG uptake on PET. FDG-PET played an important role in therapy planning and subsequent follow up. This case emphasizes the important role FDG-PET could play in the staging, restaging, and posttherapy follow up of MPNST.