No abstract

A 23-year-old right-hand-dominant woman presented with 3 weeks of progressive difficulty recognizing faces, including her own, subsequently developing psychosis. Noncontrast CT head and MRI brain with contrast revealed a nonenhancing lesion of the right posterior temporal lobe and fusiform gyrus, with corresponding hypermetabolism on FDG-PET/CT brain (figure). EEG demonstrated right posterior temporal slowing. CSF and serum anti-NMDA receptor (NMDAR) antibodies were positive, with CT abdomen/ pelvis revealing ovarian teratoma. Teratoma removal, plasmapheresis, and 1 cycle of rituximab yielded symptom resolution over 1 month. Anti-NMDAR encephalitis rarely presents with prosopagnosia, 1 and in this case, is likely secondary to right fusiform gyrus dysfunction. 2 Author contributions Grayson Beecher performed the patient's clinical assessment, wrote the manuscript, and created the figure. Amanda Nicole Wagner assisted in writing the manuscript and figure legend.

Bing-Neel Syndrome Mimicking Lower Motor Neuron Predominant Amyotrophic Lateral Sclerosis Waldenström macroglobulinemia (WM) is a rare hematological malignancy, defined as a lymphoplasmacytic lymphoma (LPL) with immunoglobulin M (IgM) paraprotein. 1 Rarely, LPL will infiltrate the central nervous system (CNS), an entity termed Bing-Neel syndrome (BNS). We describe an atypical BNS case, with a lower motor neuron-predominant presentation mimicking amyotrophic lateral sclerosis (ALS). A 74-year-old male with WM and type I cryoglobulinemia, in remission following bendamustine chemotherapy 4 years prior, and type 2 diabetes mellitus complicated by retinopathy and nephropathy was referred to our center with 24 weeks of slowly progressive, painless lower greater than upper extremity weakness, with subsequent diffuse muscle atrophy and fasciculation, without bulbar involvement. On examination (week 24), facial, palatal, and tongue musculature was preserved, and jaw jerk absent. Atrophy and fasciculations were present throughout all extremities and thoracic and lumbar paraspinals. Tone was reduced in lower extremities. Upper extremities were symmetrically weak proximally and distally (4−/5), as were lower extremities (3/5), save for left iliopsoas (2/5). He was areflexic, with downgoing plantars. There was unchanged, long-standing sensory loss to pinprick to mid-shin bilaterally and reduced vibratory sensation to the ankles, attributed to diabetic polyneuropathy. Prior investigations by the primary care physician (week 16), including complete blood count and differential, electrolytes, magnesium, calcium, creatinine, urea, creatine kinase, C-reactive protein, liver enzymes, and bilirubin, were normal. Serum protein electrophoresis (SPEP) was normal but had previously identified an IgM kappa peak (7 g/L) with the initial WM presentation that resolved with treatment. Contrast computed tomography (CT) of the head, chest, abdomen, and pelvis was unremarkable. Prereferral, outpatient nerve conduction studies (week 20, where clinical findings and pattern of weakness were similar to week 24, save for increased strength in the upper and lower extremities at 4+/5 and 4−/5, respectively) demonstrated axonal sensorimotor polyneuropathy. F-response was tested only in left tibial nerve and showed mildly prolonged latency. Electromyography demonstrated active denervation potentials (predominantly fibrillation potentials and/or positive sharp waves with fewer fasciculations) and chronic neurogenic changes in bilateral tibialis anterior, right gastrocnemius, bilateral vastus medialis, and left first dorsal interosseous. Active denervation potentials only without chronic neurogenic changes were observed in left gastrocnemius, deltoid, and triceps. Electromyography of tongue was normal, while thoracic paraspinal muscles demonstrated pervasive motor unit action potentials from incomplete relaxation. The rapidly progressive weakness, physical examination, and electrodiagnostic findings gave consideration to the diagnosis of lower motor neuro...

A 23-year-old right-hand-dominant woman presented with 3 weeks of progressive difficulty recognizing faces, including her own, subsequently developing psychosis. Noncontrast CThead and MRI brain with contrast revealed a nonenhancing lesion of the right posterior temporal lobe and fusiform gyrus, with corresponding hypermetabolism on FDG-PET/CT brain (figure). EEG demonstrated right posterior temporal slowing. CSF and serum anti–NMDA receptor (NMDAR) antibodies were positive, with CT abdomen/pelvis revealing ovarian teratoma. Teratoma removal, plasmapheresis, and 1 cycle of rituximab yielded symptom resolution over 1 month. Anti-NMDAR encephalitis rarely presents with prosopagnosia,1 and in this case, is likely secondary to right fusiform gyrus dysfunction.2