Clinical and electrophysiological study of peripheral and central neuromuscular changes in connective tissue diseases in children

Abstract

IntroductionChildren with juvenile connective tissue diseases (JCTDs) may have a wide variety of clinical features ranging from fever or a simple arthritis to complex multisystem autoimmune diseases. Aim of the workTo study clinical and electrophysiological peripheral and central neuromuscular changes in children with connective tissue diseases. Patients and methodsThirty children with different JCTDs were enrolled. Clinical and neurological examination and laboratory investigations were done. Electrophysiological evaluation was performed and included: peripheral nerve conduction studies, late responses, somatosensory evoked potential and electromyography. ResultsTwenty patients had juvenile idiopathic arthritis (JIA) (66.7%), 8 patients had juvenile systemic lupus erythematosus (JSLE) (26.7%), one patient had juvenile systemic sclerosis (JSScl), and one patient had juvenile overlap syndrome (JSScl and polymyositis). Clinical neurologic abnormalities were present in 3 patients (ulnar neuropathy, median neuropathy and polymyositis). Electrophysiological abnormalities were detected in 18 patients (clinical in 3 and subclinical in 15 patients) and included ulnar entrapment neuropathy, median axonal neuropathy, demyelinating sensory motor polyneuropathy, deep peroneal nerve entrapment at the ankle (anterior tarsal tunnel syndrome), prolonged posterior tibial somatosensory evoked potential latency and prolonged H reflex latency not explained by peripheral neuropathy, increased H/M ratio and myopathic motor units. The most common electrophysiological abnormalities were present in patients with JSLE. ConclusionClinical neurological abnormalities are not common in JCTDs whereas subclinical neurological abnormalities are common findings. Juvenile systemic lupus erythematosus had the most common abnormalities among JCTDs. Polyneuropathy in JIA is commonly of demyelinating type. Entrapment neuropathy is less frequent than in adults.