A Very Rare Cause of Shoulder Weakness: Concurrent Traumatic Neuropathies of Accessory, Long Thoracicus, and Suprascapular Nerves

Accessory nerve is one of the most sensitive cranial nerves to injury, and its injuries are usually caused by iatrogenic interventions. As many other causes can mimic its clinical presentation, careful physical examination should be performed while evaluating a patient suspected of accessory neuropathy. In this report, we aimed to illustrate a very rare case with concurrent traumatic cranial neuropathies of accessory nerve, suprascapular nerve, and long thoracicus. A 16-year-old boy was admitted with complaints of shoulder weakness and pain following a trauma of strain injury. He had first taken the misdiagnosis of brachial plexopathy; however, after a detailed physical examination and electrophysiological study, the diagnosis of concurrent traumatic neuropathies of accessory, suprascapular, and long thoracicus nerves was made. In our opinion, during evaluation of these rare traumatic neuropathies, detailed clinical evaluations combined with detailed electromyography investigations may give crucial data for the proper diagnosis

Novel Variant Syndrome Associated With Congenital Hepatic Fibrosis

Congenital hepatic fibrosis is part of many different malformation syndromes, of which oculo-encephalohepato-renal syndrome is the most common. These syndromes largely overlap, and so accurate classification of individual patients may be difficult. We present herein three syndromic siblings who were products of a consanguineous marriage. We investigated in detail at least six organ systems in these patients, namely the liver, brain, eye, kidneys, skeleton, and gonads. The common features observed in these three cases were congenital hepatic fibrosis, retinitis pigmentosa, truncal obesity, rotatory nystagmus, mental retardation, advanced myopia, and high-arched palate. The clinical dysmorphology in these patients was distinct and lacked the major features of the known syndromes associated with congenital hepatic fibrosis. Although some features of these presented cases are similar to those found in Bardet-Biedl syndrome (BBS), the absence of some major criteria of BBS (polydactyly, renal abnormality, and hypogonadism) suggests that this may be a new syndrome. All three patients remain under follow-up in the departments of Gastroenterology, Ophthalmology, and Neurology at Hacettepe University.Wo

Brachial Diparesis Due To Motor Neuronopathy As One of the Predominant Presenting Signs of Occult Small Cell Lung Carcinoma

Sensory neuronopathy is a well-established presentation in paraneoplastic neurological syndromes that is mostly associated with small cell lung cancer and anti-Hu antibodies. Motor neuronopathy, on the other hand, is an extremely rare observation in this syndrome. A 56-year-old man presented with asymmetric brachial diparesis and sensory ataxia. Electrophysiological studies revealed sensory ganglionopathy and progressive anterior horn degeneration in cervical segments. Small cell lung carcinoma with associated anti-Hu antibodies was later diagnosed. The patient did not improve despite the administration of steroids and chemotherapy. Paraneoplastic syndromes may exceptionally present with a bilateral arm weakness. Cases accompanied by sensory ganglionopathy should therefore be promptly investigated for any underlying malignancy.WoSScopu