Clinical Sciences/Health Conditions
Brianna Ritz, MD
Resident Physician
Medstar/Georgetown University
Cheverly, Maryland, United States
Katherine Power, MD
Physician
Medstar/Georgetown University
Washington, District of Columbia, United States
An 86-year old female with COPD and controlled HIV presented to the emergency department with fingertip numbness, weakness, progressive blurry vision, and worsening shortness of breath. Patient was noted to have nasolabial flattening and a pronator drift on examination. A head CT demonstrated chronic bilateral cerebellar infarcts with no evidence of acute infarcts. The patient was treated for the infarcts and admitted for further stroke workup. On admission, additional exam findings included bilateral ptosis, restricted extraocular movement in all directions, and equal but nonreactive pupils. The patient subsequently underwent a lumbar puncture with CSF studies revealing the G1QB antibody. A diagnosis of Miller Fisher Syndrome (MFS) was made, and the patient received 5 days of IVIG prior to discharge.
Discussions: The case highlights a nearly missed presentation of MFS, a rare variant of acute inflammatory demyelinating polyneuropathy (AIDP), confounded by cerebrovascular events. AIDP is the most common cause of flaccid paralysis worldwide. It is imperative AIDP is diagnosed promptly as it can progress to life-threatening respiratory failure. The diagnosis of MFS requires clinical criteria and CSF studies to rule out other similarly presenting neurologic conditions. Nerve conduction studies can be an early indicator of demyelinating polyneuropathy and could clue clinicians into a diagnosis that may be delayed or otherwise misdiagnosed.
Conclusions:
MFS is a rare and atypical variant of AIDP which manifests through a nonspecific constellation of neurologic symptoms, making it difficult to differentiate from cerebrovascular or neuromuscular junction disorders. It is important to promptly diagnose MFS, and in patients with other confounding neurologic conditions, nerve conduction studies can help key clinicians into a diagnosis of a demyelinating condition which could otherwise be overlooked. Physiatrists can also play a key role in recovery of patients with AIDP variants through a comprehensive rehabilitation program.