ABSTRACT
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired, immune-mediated neuropathy affecting peripheral nerves and nerve roots. It is characterized by symmetric weakness involving both proximal and distal muscles; it can be relapsing-remitting or progressive in course. The clinical manifestations of CIDP are various and may present with atypical features, like myokymia, tremor, or tremor-like phenomena, which may mislead the clinician in diagnosis.
Subject(s)
Myokymia , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating , Humans , Myokymia/diagnosis , Myokymia/etiology , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnosis , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/complicationsABSTRACT
PURPOSE: A wave is a late response recognized during recording of F waves. Though they might be seen in healthy subjects, their presence assumes significance in a patient presenting with polyradiculoneuropathy. METHODS: In this prospective study, 75 patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) were enrolled. They were divided into two groups based on the presence or absence of A waves. Clinical features, electrophysiological parameters and extent of clinical recovery in short-term follow-up were analyzed. RESULTS: A waves were present in 49 out of 75 patients (65%). Most common pattern observed was multiple A waves. Prevalence of A waves was more in lower limb nerves than upper limb nerves. Occurrence of A waves correlated with the presence of conduction block. Patients with A waves had higher Hughes grade (P = 0.003) and lower Medical Research Council sum score at 6 weeks of follow-up (P = 0.04) as compared to patients without A waves. CONCLUSIONS: A waves are common in acute inflammatory demyelinating polyradiculoneuropathy form of Guillain Barre syndrome and are considered as a marker of demyelination. Long-term follow-up studies are required to ascertain their significance in prognostication and assessing recovery.
Subject(s)
Guillain-Barre Syndrome/diagnosis , Guillain-Barre Syndrome/physiopathology , Neural Conduction , Peripheral Nerves/physiopathology , Adult , Aged , Follow-Up Studies , Humans , Middle Aged , Prospective Studies , Young AdultABSTRACT
We report an unusual presentation in a 9-year-old girl with unilateral circumferential thinning of the entire right upper limb without any other neurological deficit, with normal nerve conduction and electromyography initially thought of as a neurodegenerative disorder based on clinical presentation. Magnetic resonance imaging of the upper limb showed partial lipoatrophy with normal glucose metabolism and lipid profile and negativity for HIV and autoimmune disease (panniculitis) with no family history of similar disorder. Remember to think out of box before labeling neurodegenerative disease.
