RESUMEN
Typical cases of neuralgic amyotrophy present with sudden onset of excruciating pain in the shoulders and upper extremities, followed by marked muscle weakness and atrophy over a period of hours to days. Neuralgic amyotrophy is not confined to the brachial plexus, and difficulties in its diagnosis may delay the start of rehabilitation therapy. Here, we report a case of rehabilitation of a patient with neuralgic amyotrophy presenting with Collet-Sicard syndrome (9th, 10th, 11th, and 12th cranial nerve disorder)．A 44-year-old man developed severe pain from the left posterior neck to the occipital region, followed by sporadic onset of dysarthria, dysphagia, and difficulty in raising the left upper limb over several weeks. Nerve conduction studies showed marked bilateral differences in the amplitude of the compound muscle action potential recorded from the trapezius during accessory nerve stimulation. Needle electromyography showed abnormal resting potentials in the left trapezius and left side of the tongue and a decrease in the interference pattern during voluntary contraction. Based on the clinical course, neurological and laboratory findings, a diagnosis of neuralgic amyotrophy was made. Speech language hearing therapy was performed for dysarthria and dysphagia, and physical therapy was performed for difficulty in raising the left upper limb due to accessory nerve palsy. Rehabilitation along with recovery from inflammation-induced neuropathy allowed the patient to become independent in activities of daily living.
RESUMEN
Typical cases of neuralgic amyotrophy present with sudden onset of excruciating pain in the shoulders and upper extremities, followed by marked muscle weakness and atrophy over a period of hours to days. Neuralgic amyotrophy is not confined to the brachial plexus, and difficulties in its diagnosis may delay the start of rehabilitation therapy. Here, we report a case of rehabilitation of a patient with neuralgic amyotrophy presenting with Collet-Sicard syndrome (9th, 10th, 11th, and 12th cranial nerve disorder)．A 44-year-old man developed severe pain from the left posterior neck to the occipital region, followed by sporadic onset of dysarthria, dysphagia, and difficulty in raising the left upper limb over several weeks. Nerve conduction studies showed marked bilateral differences in the amplitude of the compound muscle action potential recorded from the trapezius during accessory nerve stimulation. Needle electromyography showed abnormal resting potentials in the left trapezius and left side of the tongue and a decrease in the interference pattern during voluntary contraction. Based on the clinical course, neurological and laboratory findings, a diagnosis of neuralgic amyotrophy was made. Speech language hearing therapy was performed for dysarthria and dysphagia, and physical therapy was performed for difficulty in raising the left upper limb due to accessory nerve palsy. Rehabilitation along with recovery from inflammation-induced neuropathy allowed the patient to become independent in activities of daily living.
RESUMEN
This report illustrates a case of chronic inflammatory demyelinating polyneuropathy (CIDP) masquerading as neurofibromatosis caused by multifocal enlargements of spinal nerve roots. At age 73, the patient reported a 6-year history of numbness, weakness and pain in the hands and legs, but he could but he could walk independently with a cane. And although tremor was present, he could still draw. T2-weighted magnetic resonance imaging (MRI) through the cervical spine demonstrated spinal cord compression bilaterally at C 6-7, caused by neurofibroma-like cervical root tumors and enlargement of the spinal nerve roots and the brachial and lumbosacral nerve plexuses. Nerve conduction studies showed very little evoked response, with the exception of the median nerve which demonstrated prolonged distal latency and reduced compound muscle action potential with temporal dispersion, suggesting a diagnosis of demyelinating neuropathy. Somatosensory evoked potentials of the median nerve revealed prolonged latency, and motor evoked potentials obtained from the abductor pollicis brevis and abductor digiti minimi by transcranial magnetic stimulation demonstrated prolonged latency and temporal dispersion. Sural nerve biopsies showed segmental demyelination, remyelination (onion-bulb formation), axonal loss, and lymphocyte infiltration suggesting CIDP. The patient did not have a positive family history and declined further genetic studies. We could therefore not rule out the possibility of a hereditary hypertrophic neuropathy such as Charcot-Marie-Tooth disease.