Steven P. Braff

Treatment of steroid-unresponsive tumefactive demyelinating disease with plasma exchange

The authors describe a patient with an isolated, gadolinium-enhancing, biopsy-proven focus of tumefactive demyelination. There was marked clinical improvement with plasma exchange after failure of high-dose i.v. corticosteroids. The post-treatment clinical course correlated with decreasing enhancement and lesion size on MRI. This patients rapid clinical and MRI response suggests that plasma exchange may be beneficial in this disorder, and could perhaps serve as a diagnostic tool to avoid the need for brain biopsy.

Tumefactive multiple sclerosis plaque

We present a case of a large solitary demyelinating plaque in the brain masquerading as tumour. A 34 year old healthy woman presented with sudden onset of left hemiparesis. Subsequent MRI of the brain showed a single large 2.5 cm right sided enhancing lesion within the white matter of the parietal lobe …

Idiopathic Lymphocytic Cerebellitis

Accessible online at: www.karger.com/journals/pne A 16-year-old girl presented with a 3-month history of worsening right temporal and retroauricular headaches. She had previously been treated for otitis media with antibiotics, but her headache and pain continued to increase. Later, she developed an ataxic gait, increasing somnolence, vomiting and diplopia that prompted admission to hospital. On examination, she was also noticed to have right third-division trigeminal neuralgic pain and rightsided dysmetria on finger to nose testing.

JC Virus PCR Detection Is Not Infallible: A Fulminant Case of Progressive Multifocal Leukoencephalopathy with False-Negative Cerebrospinal Fluid Studies despite Progressive Clinical Course and Radiological Findings

We describe a case with a false-negative PCR-based analysis for JC virus in cerebrospinal fluid (CSF) in a patient with clinical and radiological findings suggestive of progressive multifocal leukoencephalopathy (PML) who was on chronic immunosuppressive therapy for rheumatoid arthritis. Our patient developed rapidly progressive global decline with clinical and radiographic findings suggestive of PML, but JC virus PCR in CSF was negative. The patient passed away 3 months from the onset of her neurological symptoms. Autopsy confirmed the diagnosis of PML with presence of JC-polyoma virus by immunohistochemical staining. This case highlights the potential of false-negative JC virus PCR in CSF when radiographic and clinical features are suggestive of “possible PML.” We review the plausible causes of potential false-negative CSF results and suggest that when the clinical presentation is suspicious for PML repeat CSF analysis utilizing ultrasensitive PCR assay and subsequent brain biopsy should be considered if CSF remains negative. Additionally, appropriate exclusion of other neurologic conditions is essential.

Torsion of a Lumbar Nerve Root Schwannoma

We report a very rare case of a lumbar nerve root schwannoma presenting with torsion and infarction. The patient was a 16-year-old male presenting with severe low back pain and urinary retention following an aggressive game of hockey. Subsequent MRI of the lumbar spine revealed a nonenhancing lumbar intradural lesion at the level of L3. The patient was taken to the operating room where he underwent a bilateral L2 and L3 laminectomy and gross total resection of an intradural nerve root tumor, which appeared to have undergone torsion and infarction. Subsequent histopathological examination of the surgical specimen verified the diagnosis of infarcted schwannoma. This is a unique case of lumbar nerve root schwannoma, with atypical MRI findings, presenting with infarction due to torsion of the involved nerve root.

Giant cavernous angioma.

Accessible online at: www.karger.com/journals/pne The patient is an 18-month-old female who presented with a staring episode followed by loss of consciousness lasting 1 min. Physical examination showed a slight asymmetry of movement of the extremities. Magnetic resonance imaging revealed a 2.5 ! 2.2 ! 2.5-cm enhancing lesion with surrounding edema and mass effect (fig. 1). No blooming artifact was discernible on the gradient echo sequence (not shown). These radiological features were suggestive of a brain tumor. The lesion was removed, and pathology demonstrated multiple thin-walled thrombosed vessels with microcalcifications and no intervening brain parenchyma, consistent with cavernous angioma.

Hypothalamic-Opticochiasmatic Gliomas Mimicking Craniopharyngiomas

The differential diagnosis of suprasellar masses in the pediatric age group includes craniopharyngiomas and hypothalamic-opticochiasmatic gliomas (HOCGs). These tumors frequently display unique features on computed tomography and magnetic resonance imaging. We review two cases of pediatric suprasellar HOCGs with preoperative imaging characteristics resembling those of craniopharyngioma. HOCGs mimicking craniopharyngiomas represent a diagnostic and operative challenge to the pediatric neurosurgeon. Although an accurate leading pathologic diagnosis can frequently be made with preoperative neuroimaging, the neurosurgeon must be prepared for discovery of another tumor type and have plans to proceed accordingly.