Caleb Ward MB BChir, Children’s National Medical Center

The Case
A 13yo F presents with worsening headache, an abnormal MRI, and eventually, abnormal but nonspecific CSF findings. This case asked readers to determine how urgent the patient’s workup and treatment should be given her relatively chronic symptoms but new diagnostic results.

Here’s How You Answered Our Questions:

CSF results:
OP 55, glucose 19, protein 117, 4 WBCs, 1 RBC

Discussion:
Despite the polls indicating a split between providers on willingness to perform an LP in the ED, most of the members of our live discussion group were in favor. Dewesh Agrawal reminded us that hydrocephalus in the absence of obstruction (i.e. communicating hydrocephalus) is not a contraindication to performing an LP.

Our group spent quite a bit of time discussing the urgency (or lack thereof) of giving antimicrobial therapy. The elevated protein, low glucose, and elevated opening pressure of the CSF did raise concern for CSF infection, but the lack of a pleocytosis and chronicity of symptoms made many of our providers less enthusiastic about starting antibiotics in the ED.

Our teaching attending Alyssa Abo posted a broad differential for the diffuse leptomeningeal enhancement seen on MRI.

Denouement:
The patient was admitted to the PICU for frequent neuro observations overnight. Given the absence of a pleocytosis, empiric antimicrobials were withheld. Her infectious disease workup including: cryptococcal antigen, tuberculosis (quantiferon and PPD), HSV, enterovirus, Lyme PCR and antibodies were all negative. Her CSF culture was sterile. Rheumatology was consulted with concern for neurosarcoidosis, but serum and CSF ACE levels were normal. Oncology was involved from the time of admission, with suspicion for a CNS lymphoma, diffuse leptomeningeal glionueuronal tumor or diffuse leptomeningeal dissemination of unknown primary or histiocytic disease. Her LDH was elevated, and beta HCG, AFP CSF, and uric acid were all normal. Malignant cells were not obtained from the CSF.

Her clinical condition deteriorated with progression of her headaches and repeat imaging showing progressive dilation of her ventricles. She was treated with Acetazolamide and on Day 10 of admission she underwent a biopsy of the meninges and brain and placement of a VP shunt. She subsequently required externalization of the shunt for episodes of headache with associated bradycardia. The shunt has since been re-internalized. Pathology from the biopsy was consistent with an anaplastic large cell medulloblastoma. A PET scan was completed, showing areas of concern in the bone marrow space. One month post admission she remains inpatient undergoing chemotherapy with cyclophosphamide and being prepared for stem cell harvest.

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