Objective: This study aimed to better understand the clinical, electrophysiological, pathological features and prognosis of peripheral nerve involvements in primary immunoglobulin light-chain (AL) amyloidosis. Methods: We retrospectively reviewed the clinical data of eight AL amyloidosis patients with peripheral neuropathy as the initial presentation including clinical features, histopathological findings and treatment. Results: There were seven males and one female aged from 52 to 66 years. Initial symptoms included symmetrical lower extremity numbness, lower extremity pain and carpal tunnel syndrome. Seven patients suffered from severe pain and required pain management. Six patients had predominant autonomic dysfunction. Six patients had cardiac involvement, and one patient had renal involvement. Monoclonal proteins were found in all patients, with IgA lambda in one, IgG lambda in two, lambda alone in three, kappa alone in one and IgM kappa in one. Sural nerve biopsies were performed in 7 cases, all of which showed amyloid deposition in the endoneurium (in the perivascular region in some cases), in addition to moderate to severe myelinated fiber loss with axonal degeneration. Six patients were treated with combined chemotherapy. In three patients who began chemotherapy earlier (6-10 months after onset), two achieved a hematological complete response, and one achieved a partial response. three patients who had delayed chemotherapy (36 months after onset) died between 5 and 12 months after diagnosis. Conclusion: Early recognition of AL amyloidosis with peripheral neuropathy as the initial symptom is very important. Nerve biopsy can help to make the diagnosis. Early diagnosis and chemotherapy are critical to achieve better outcomes.

OBJECTIVE: Discussion of a rare case of dermatomyositis associated with tonsillar neoplasm in an African American woman. BACKGROUND: Dermatomyositis is a syndrome of inflamatory myopathy with multiorgan manifestations which has been linked to immune dysregulation and neoplasia. INTRODUCTION: Many studies have shown five to seven fold increased risk of developing malignancy with dermatomyositis within two years of presentation. Most common cancers reported are adenocarcinomas of lung, breast, ovaries, stomach, pancreas and bladder. Dermatomyositis as a paraneoplastic manifestation of tonsillar squamous cell carcinoma has not previously been described. DESIGN: This is a case report of a 52 year old woman who presented for the evaluation of weakness, facial rash and burning pains. Diagnosis of dermatomyositis was made clinically and corroborated by EMG and muscle biopsy. She was started on prednisone but did not improve. CT chest, abdomen and pelvis along with panendoscopy was done to evaluate for malignancy. She developed swallowing problems, laryngopharyngeal reflux disease and esophageal dysmotility syndrome within 1 year of diagnosis. Approximately 1.5 years after dermatomyositis diagnosis, she developed a right sided neck mass. Biopsy of the mass found metastatic squamous cell carcinoma. Further work up revealed an ulcerating cavity under tonsillolith containing abnormal tissue and this was thought to be the primary malignancy. RESULTS: Patient underwent right radical neck dissection and tonsillectomy followed by chemotherapy and radiation and her muscle strength, facial rash, burning pains and swallowing difficulties improved. CONCLUSION: To our knowledge, this is the first case of dermatomyositis in the setting of tonsillar squamous cell carcinoma. High suspicion of nasopharyngeal carcinoma should be maintained in dermatomyositis patients with otherwise negative routine malignancy screening who exhibit any pharyngeal or esophageal complaints.

OBJECTIVE: Discussion of a case of Inclusion body myositis (IBM) associated with macroglossia. INTRODUCTION: IBM is one of the idiopathic inflammatory myopathies. Exact pathogenesis is unclear but there is an evidence of dysregulation of antigen driven immune response involving T cells. Typical onset is slowly progressive impacting quadriceps often more than hip flexors, ankle dorsiflexors and distal forearm flexor muscles. Swallowing difficulties often are present and mild facial weakness can be seen. Macroglossia has never been reported in association with IBM. In fact inflammatory myopathies of tongue are a rarity. DESIGN: A case report of a 68 year old woman with 12 year progressive difficulty getting out of chair and inability to twist jar lids. Examination showed forearm flexor and quadriceps atrophy and bilateral face, hip flexion and finger flexion weakness. Clinical diagnosis of IBM was further supported by muscle biopsy findings. One year after the diagnosis, she reported painless progressive enlargement of her tongue resulting in difficulty with chewing. On exam the tongue looked enlarged. MRI of the oropharynx was unremarkable. Tongue muscle biopsy showed non-granulomatous inflammatory infiltrates, myophagocytosis and degenerating regenerating fibers without evidence of amyloidosis. Work up for Sarcoidosis with Chest CT and ACE levels was negative. RESULTS: Decision to start patient on steroids for idiopathic myositis of the tongue was deferred as our patient is currently enrolled in an IBM clinical trial. CONCLUSION: Macroglossia in IBM patients has never been reported in literature. Two reported cases of macroglossia were associated with dermatomyositis and granulomatous myositis. It remains unknown whether this is the first reported case of IBM associated with macroglossia or macroglossia due to a second inflammatory myositis.