AN INTERESTING PRESENTATION OF FROIN SYNDROME AS A GUILLAIN-BARRE SYNDROME MIMIC
Haider M, Khalid Y, Chaudhary AJ, Nawaz AB, Akram S, and Kumar J. AN INTERESTING PRESENTATION OF FROIN SYNDROME AS A GUILLAIN-BARRE SYNDROME MIMIC. J Gen Intern Med 2023; 38:S404.
J Gen Intern Med
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.