A Clinical Approach to Disease of Peripheral Nerve.

Peripheral neuropathy is one of the most prevalent neurologic conditions encountered by neurologists and nonneurologists. Geriatricians and primary care physicians often face the task of screening patients for early neuropathy when they have underlying conditions such as diabetes mellitus and evaluating patients who report new symptoms that suggest neuropathy. An understanding about different forms of neuropathies based on anatomic pattern and type of nerve fiber involvement and ability to perform basic neurologic examination reliably can help determine how to pursue further investigations and identify those patients who are likely to benefit from early specialist referral.

Immune Myopathy With Perimysial Pathology Associated With Interstitial Lung Disease and Anti-EJ Antibodies.

OBJECTIVES: We report a case of immune myopathy with perimysial pathology associated with anti-glycyl-transfer RNA synthetase (anti-EJ) antibody and an excellent treatment response. METHODS: Chart review. RESULTS: A 36-year-old woman presented with 3 months of fatigue, weight loss, progressive weakness in a scapuloperoneal distribution, and dysphagia. Nerve conduction studies, electromyography, and ultrasound suggested an irritable myopathy. She had marked elevations of creatine kinase and positive anti-glycyl-transfer RNA synthetase (anti-EJ) antibodies. A left biceps muscle biopsy revealed inflammation of the perimysium and surrounding perimysial blood vessels with focal fragmentation of the perimysium. Further evaluation revealed interstitial lung disease. Treatment with prednisone and mycophenolate mofetil led to marked clinical improvement of her symptoms. CONCLUSIONS: Our case adds to the growing spectrum of inflammatory myopathies and highlights the importance of performing a comprehensive, multisystem workup.

Median nerve penetration by a persistent median artery and vein mimicking carpal tunnel syndrome.

INTRODUCTION: Carpal tunnel syndrome (CTS) is a common clinical syndrome seen in the outpatient setting that is easily confirmed by electrodiagnostic testing. METHODS: We describe the case of a patient who presented with the classic symptoms and neurological examination for CTS, but had a normal nerve conduction study and electromyogram. RESULTS: Neuromuscular ultrasound of the median nerve on the symptomatic side revealed penetration of the nerve by a persistent median artery and vein in the mid-forearm, with a positive sonographic Tinel sign over this spot. This finding is an anatomical variation that has been described sparingly in the literature, mostly in cadavers. It has not been reported previously to be a mimic of CTS. CONCLUSIONS: This case demonstrates the diagnostic utility of neuromuscular ultrasound and the importance of considering an anatomical variation involving the median nerve in the differential diagnosis of CTS.

Primary Neurolymphomatosis Presenting With Polyradiculoneuropathy Affecting One Lower Limb.

OBJECTIVES: We report a case of primary neurolymphomatosis (NL) with unusual presentation and excellent treatment response. METHODS: Chart review. RESULTS: A 64-year-old woman presented with 2 months of progressive pain, weakness, and numbness in her right leg. Nerve conduction study/electromyogram suggested a right lumbosacral radiculoplexus neuropathy with associated acute right peroneal neuropathy at the fibular head. L/S spine and right leg magnetic resonance imaging showed thickening and contrast enhancement of the right S1 nerve root and the right distal sciatic, tibial, and common peroneal nerves, as well as a lobular enhancing lesion of the right superficial peroneal nerve. Whole-body fludeoxyglucose-positron emission tomography scan showed no other lesions. A right superficial peroneal nerve lesion biopsy revealed infiltration of the nerve by diffuse large B-cell lymphoma. The lymphoma cells expressed BCL2 but not CD10, suggesting an origin in peripheral blood not lymph nodes. Despite the expression of BCL2, which is considered as a poor prognosis marker, our patient responded very well to the combined radiotherapy and chemotherapy with the R-MPV (rituximab, MTX, procarbazine, and vincristine) regimen. The patient showed marked clinical improvement and complete resolution of lymphoma lesions on the PET scan. CONCLUSIONS: Our case broadens the clinical spectrum and illustrates the importance of early diagnosis and aggressive treatment of primary NL.

Calciphylaxis in a dialysis patient diagnosed by muscle biopsy.

Calciphylaxis is a rare condition seen mostly in patients with chronic renal disease and secondary hyperparathyroidism who develop painful skin lesions and myopathy secondary to extensive small vessel calcification, which leads to tissue ischemia. It is typically diagnosed by a biopsy of prominent skin lesions. Here, we report a 49-year-old man with end-stage renal disease on chronic peritoneal dialysis who presented with weakness, myalgias, and necrotic skin lesions. Multiple skin biopsies were nondiagnostic because of severe extensive necrosis, and the diagnosis of systemic calciphylaxis was eventually made by a muscle biopsy. This case demonstrates the significant muscle involvement in calciphylaxis and highlights the importance of maintaining a high clinical suspicion for patients with risk factors for calciphylaxis, even when skin biopsy does not confirm it.