AN INTERESTING PRESENTATION OF FROIN SYNDROME AS A GUILLAIN-BARRE SYNDROME MIMIC

Document Type

Conference Proceeding

Publication Date

6-23-2023

Publication Title

J Gen Intern Med

Abstract

CASE: A 66-year-old female with a past medical history significant for migraines & invasive lobular breast cancer status post bilateral mastectomy, chemotherapy & radiotherapy presented with worsening migraine headaches, nausea, projectile vomiting & progressive bilateral lower extremity weakness for 3 weeks. The patient was recently treated with Azithromycin for Campylobacter gastroenteritis four weeks ago. The patient was hemodynamically stable. Physical exam was significant for irritability, decreased power (3/5) & absent reflexes bilaterally in the lower extremities. Lab work was remarkable for mild leukocytosis. Brain imaging (CT & MRI) revealed disproportionate lateral ventriculomegaly with obstructive hydrocephalus. A CT abdomen pelvis & MRI thoracolumbar spine were unremarkable. Lumbar puncture (LP) revealed an elevated opening pressure of 38 mmHg, glucose:41 & total protein: 295 mg/dl(elevated). Cerebrospinal fluid(CSF) cytology revealed xanthochromia, elevated RBCs >3000 & WBC: 38 (0-10) with 44% lymphocytes, 2% neutrophils & malignant cells morphologically resembling invasive lobular carcinoma. The patient was initially started on broad-spectrum antibiotics & later discontinued when infectious etiologies were ruled out. Given mild symptoms & low concern for GBS, intravenous immunoglobulin & plasmapheresis were not done. The patient was started on steroids. However, given the inadequate response to treatment patient was referred to a high-level care facility where the patient was admitted to the intensive care unit. Neurology, neurosurgery, & infectious disease services were on board & repeat workup was consistent. The patient received five days of high-dose steroids.

IMPACT/DISCUSSION: Differential diagnosis in such cases should be broad including metastatic disease, infectious processes, Guillain- Barre syndrome (GBS), & rare Froin syndrome. Symptoms of Froin syndrome include back pain, lower extremity neurological symptoms (sensory deficit, weakness, brisk/absent reflexes), & symptoms of elevated intracranial pressure (headache, irritability, nausea & vomiting). GBS was a differential in our case given the recent history of diarrhea and the patient's presentation with progressive weakness, & areflexia. However, in our patient, neurological symptoms did not progress beyond the lower extremities and elevated ICP pressures were more consistent with a diagnosis of Froin syndrome. Treatment of Froin syndrome is focussed on conservative management with the treatment of the underlying cause.

CONCLUSION: Froin syndrome, first described by Georges Frion in 1910, is a rare condition with xanthochromia, elevated protein levels & hypercoagulability in CSF. Elevated protein levels lead to stagnation leading to the activation of coagulation factors. It can be secondary to obstructive, infectious, or neoplastic processes.

Volume

38

First Page

S404

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