Severe Pure Acute Motor Axonal Neuropathy

2015 
Acute motor axonal neuropathy (AMAN) is a subtype of Guillain-Barre syndrome. Characteristic electrophysiological features of AMAN are reduced amplitude or absence of muscle action potentials. Our patient described progressive weakness in his leg and was not able to independently walk; he had a feeling weakness in his arms within 24 h after the onset of symptoms. He was diagnosed with AMAN according to the clinical and electrophysiological features. He could independently walk after two years following intensive physical therapy and monitoring. Neurologists and physiatrist should conduct long term monitoring and rehabilitation for patients with AMAN because neurological deficits may persist for a long time. AMAN was first reported to occur in patients having peripheral axonal dysfunction without demyelinating findings in the electroneuromyograph (ENMG) and is seen mostly during summers in the rural areas of China. Some cases were reported from some countries out of Asia, such as Mexico, in spite of the absence of seasonal predisposition. AMAN is characterized by acute motor paralysis, loss of reflexes or hyporeflexia, and peripheral axonal involve- ment without demyelinating findings in ENMG. In a study the median age of patients with AMAN in China was found to be 10 years (3). In this study, it was aimed to present an 8-year-old patient with AMAN and who had muscle weakness in the lower extremity at the beginning and then developed tetraplegia as a result of pure motor involvement without sensory involvement in a short time. The patient was treated with intravenous immunoglobulin (IVIG) therapy. CASE REPORT An 8-year-old male patient presented to our outpatient clinic with complaints of incapability of walking and moving his hands and arms. The patient who had not experienced such a complaint before complained about loss of strength that had suddenly occurred in his right leg a week ago. He stated that the weakness spread to the other leg and arms within the first 24 h after the onset of the symptoms. He told that he did not have any pain at the onset of symptoms but that he began to feel pain as the weakness progressed. The investigation of his medical history revealed no previous infection, surgical intervention, or vaccination. Moreover, he did not have a history of fever, vomiting, and diarrhea before the illness; no serious pathological history was found in his family and relatives. The patient was conscious, and he was completely oriented and cooperative. His systemic examination results were normal. The eyes were at the midline and moving to every direction. The patient could count fingers at 6 m. His pupils were isocoric; direct and indirect light reflexes were positive, and the examination of the fundus was normal. No pathological changes were detected on cranial nerve examination.
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