Abstract Details

Title Not an Ordinary Dry Spinal Tap: Froin's Syndrome in an Unusual Case of Neurosarcoidosis

Topic Critical Care/Emergency Neurology/Trauma

Presentation(s) P05 - (-)

Poster/Presentation
Number 207

Objective

Background BACKGROUND: In 1903, Froin first described the syndrome bearing his name marked by cerebrospinal fluid xanthochromia and hypercoagulability due to an increased protein content. This was noted in loculated areas of the subarachnoid space, sequestered from cerebrospinal fluid circulation by an obstructing inflammatory or neoplastic lesion.

Design/Methods DESIGN/METHODS: Case Report. A 43-year-old man presented with a 4 week history of limb weakness, severe back and radicular leg pain, urinary retention and headache. Fever, night sweats and weight loss were reported. Limb flaccidity was followed by spasticity among other pyramidal signs and he developed bilateral Horner's syndrome. Intraorbital bullet fragments precluded MRI neuroaxis imaging and initial noncontrast CT scanning only showed possible mild hydrocephalus. Extensive infectious, inflammatory, toxic, metabolic, neoplastic/paraneoplastic and autoimmune studies were unremarkable except for CSF findings. There were 4 dry lumbar spinal taps before success with a C1-C2 level tap with CSF showing RBC: 0mm/3, WBC: 162mm/3 (Neutrophils-40%, Lymphocytes-60%), protein: 22mg/dL, glucose: 67mg/dL; and cytology: negative. Contrast CT scans of the spine revealed ill-defined extradural versus intramedullary enhancing lesions at the cervicothoracic and the lumbosacral levels. A biopsy attempt with a L4-L5 laminectomy was without histopathological yield but an intraspinal canal filled with a gelatinous material encasing the nerve roots was seen with no CSF present. The CSF protein was found to be 256mg/dL when an external ventricular drain was placed for worsening hydrocephalus. The patient eventually succumbed to a subarachnoid hemorrhage.

Results RESULTS: Postmortem neuropathology revealed sarcoid granulomas extensively affecting dural and perivascular tissues. There were large obstructing subarachnoid granulomatous lesions consistent with neurosarcoidosis.

Conclusions CONCLUSIONS: In patients with inflammatory or neoplastic neurological conditions, Froin's syndrome must be considered in the differential for a repeatedly dry spinal tap.

Authors/Disclosures
John A. Morren, MD, FAAN (Cleveland Clinic) PRESENTER	Dr. Morren has received publishing royalties from a publication relating to health care.
Efrain D. Salgado, MD (Cleveland Clinic Florida)	Dr. Salgado has nothing to disclose.
Brian C. Healy	The institution of Mr. Healy has received research support from Analysis Group. The institution of Mr. Healy has received research support from Bristol-Myers Squibb. The institution of Mr. Healy has received research support from Verily Life Sciences. The institution of Mr. Healy has received research support from Novartis. The institution of Mr. Healy has received research support from Merck Serono. The institution of Mr. Healy has received research support from Genzyme.

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