Vascular complications of fungal meningitis attributed to injections of contaminated methylprednisolone acetate.

IMPORTANCE: Fungal meningitis due to injections of contaminated methylprednisolone acetate can present with vascular sequelae in immunocompetent individuals. This is particularly germane to neurologists because better recognition of the clinical characteristics of patients with fungal meningitis and ischemic stroke will provide more timely and efficient care. OBSERVATIONS: In a case series, 3 patients presented to Vanderbilt University Medical Center in Nashville, Tennessee, with acute ischemic stroke and later received a diagnosis of fungal meningitis attributed to epidural injections of contaminated methylprednisolone. Of these 3 patients, 2 were women, and the mean age for all 3 was 75.3 years. Their medical records and imaging scans were reviewed. All 3 patients presented with acute ischemic strokes and had a history of epidural spinal injections of methylprednisolone for low back pain. All 3 patients had 1 or more traditional risk factors for stroke. There were differing vascular patterns of presentation: 2 patients presented with small-vessel (lacunar) infarctions, whereas 1 patient presented with a large-vessel infarct. Of these 3 patients, 2 died and underwent an autopsy, which revealed Exserohilum rostratum as the presumed cause of death. For 2 cases, fever and meningeal signs were absent at presentation. CONCLUSIONS AND RELEVANCE: Patients with fungal meningitis may present with ischemic stroke detected on initial imaging scans. A definitive diagnosis should not delay early antifungal treatment.

Leptomeningeal metastasis from occult signet-ring cell colon adenocarcinoma presenting with isolated headache.

We report a patient with a rare primary signet-ring cell carcinoma of the transverse colon with secondary leptomeningeal carcinomatosis. The only presenting and persistent symptom was worsening headache for 6 weeks until death. There were no other neurological, constitutional, or gastrointestinal symptoms. The primary tumor was detected by CT scan and confirmed by endoscopy, and the final diagnosis was eventually made from cerebrospinal fluid and tissue cytology. This report confirms the dismal prognosis of leptomeningeal carcinomatosis, especially when the primary malignancy is asymptomatic.

Adult-onset leukoencephalopathy with neuroaxonal spheroids and pigmented glia: report of five cases and a new mutation.

The objective of this work is to report on a series of five patients with adult-onset leukoencephalopathy with neuroaxonal spheroids and pigmented glia (ALSP). ALSP is a rare adult-onset leukodystrophy, which encompasses hereditary diffuse leukoencephalopathy with axonal spheroids and pigmentary orthochromatic leukodystrophy. This was a retrospective chart review and literature review. Five previously healthy women presented with a rapidly progressive neurological disorder at ages 39, 37, 40, 30, and 47, respectively. All five individuals were initially diagnosed as suffering from multiple sclerosis. The clinical courses of the five patients were dominated by progressive spastic quadriparesis (patient 5, newly diagnosed, has paraparesis at this time) and dementia. Brain magnetic resonance imaging (MRI) showed diffuse cerebral atrophy, corpus callosal atrophy, and diffuse T2 hyperintensities in the subcortical and periventricular white matter with no gadolinium enhancing lesions. Three patients showed involvement of pyramidal tracts from motor cortex to the brainstem. Cerebrospinal fluid was normal in all cases. Diagnosis of ALSP was established by biopsy (two cases) and autopsy (two cases). Histopathology showed the presence of neuroaxonal spheroids in all four cases and pigmented glia in three. In the fifth case, diagnosis was established by genetic analysis alone that showed a disease-causing mutation in the colony-stimulating factor 1 receptor (CSF1R) gene. Genetic analysis was done in three patients with available DNA, and identified the disease-causing mutation in all three, including a novel mutation F828S. ALSP may be suspected in adults with rapid to subacute progression of neurological disease when (1) MRI shows corpus callosal atrophy on a background of generalized brain atrophy and diffuse white matter disease without postcontrast enhancement, (2) CSF studies are normal, and (3) studies for systemic inflammatory diseases and specific leukodystrophies are normal. Diagnosis may be made without histopathological evidence when a disease-causing mutation is demonstrated in the CSF1R gene.