without sensory or bulbar signs. Repeat CSF studies showed high protein (98.9), glucose (85), and 1 WBC, with albuminocytological dissociation confirming GBS. She received a 5-day course of intravenous immunoglobulin with significant improvement in weakness, and she was ambulating using a roller walker with minimal assistance. Deep tendon reflexes remained negative. Discussion: Facial paresis is an infrequent presentation at onset, occurring in <1% of GBS patients. Back pain may be an early symptom
of GBS, and the intensity does not correlate with prognosis. Pain, dysautonomia, ataxia, dysphagia, ophthalmoplegia, bulbar palsy, and facial weakness are some atypical clinical features that must increase the clinical suspicion for GBS. Autonomic dysfunction can vary widely from hypertension and urinary retention (as described in this patient) to cardiac arrest. Other common autonomic symptoms include ileus, hypotension, fever, tachycardia, and bradycardia. SIADH and PRES have also been described in rare situations.
PECULIAR ASSOCIATION OF TWO UNCOMMON NEUROLOGICAL DISORDERS— NEUROMYELITIS OPTICA AND MYASTHENIA GRAVIS Sriveni Tangellapelli, Raja Saravanan, Srujani Karra, Rineetha Tandra, Karthik Reddy, Department of Medicine, Baton Rouge General Medical Center, Baton Rouge, LA.
Introduction: Neuromyelitis Optica (NMO) is a rare inflammatory disorder of the central nervous system producing antibodies against Aquaporin-4 (AQP4) that results in florid demyelination, predominantly targeting optic nerves and the spinal cord. Although both conditions are uncommon, the association of NMO and Myasthenia Gravis (MG) occurs much more frequently than by chance. Case: A 72-year-old female with a recent diagnosis of MG and a prior history of stroke presented with sudden progressive weakness on the left side. Computed Tomography (CT) of the head showed no acute process with unchanged chronic changes. However, an Magnetic Resonance Image (MRI) of the head without contrast revealed a new T2/FLAIR hyperintense signal in the right upper cervical spinal cord and medulla, and mild diffuse pachymeningeal enhancement, which is unusual from a stroke standpoint. MRI of the cervical spine showed an extension of this hyperintense signal to the level of C4-C5. Concomitant CT angiogram of the head for vasculitis was negative. In the next 24 hours, symptoms got worse with profound weakness in the limbs, neck flexors, facial muscles, and ptosis
concerning myasthenia gravis exacerbation, and the patient was started on Intravenous Immunoglobin. Despite this, she had progressive worsening of motor strength in extremities associated with brisk reflexes that did not fit the MG. The cerebrospinal fluid (CSF) results showed lymphocytic pleocytosis with elevated protein, which, along with enhancing lesions on MRI, could be concerning for malignancy or autoimmune disorders like sarcoidosis and NMO. The workup for metastatic disease was negative. AQP4 antibody titer was 1: 1000 (usually less than 1: 10) and CSF myelin essential protein >160 ng/mL (0-5.6 ng/mL normal range), meeting diagnostic criteria for Neuromyelitis Optica. She improved partially with high dose steroids and plasmapheresis with plans for outpatient rituximab. Discussion: Although NMO is a rare condition, it appears to be frequently associated with MG. Therefore, maintaining a high clinical suspension and checking for AQ P4 antibodies in MG is beneficial, especially when physical exam and concomitant MRI findings could meet the diagnostic criteria of NMO. This expedites the time for diagnosis and aids in initiating effective treatment.
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