ABSTRACT
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.
ABSTRACT
Attentional disturbance following brain damage is usually evaluated by several neuropsychological tests. In a rehabilitation setting, however, the primary concern is not task performance, but rather functional real-world behavior. To address this requirement, a new assessment system for attentional behavior, BAAD (Behavioral Assessment of Attentional Disturbance), has been developed. This assessment is generally completed by the patient's therapist (occupational therapist, OT) during therapy. The aim of this study was to investigate whether BAAD completed by the family at home (BAAD-FM) yields results that are comparable to BAAD completed by an OT during occupational therapy (BAAD-OT). The subjects were 53 patients with brain damage. BAAD consists of six items thought to be associated with attentional behaviors. Each item is rated (0 to 3) based on the frequency with which the problem behaviors appeared during daily living at home and daily sessions of occupational therapy. The intraclass-correlation coefficient of the total score between BAAD-FM and BAAD-OT was 0.89. The mean (SD) values of the total scores were 3.7 (3.7) and 3.7 (3.6), respectively. Similarly, there were no significant differences in any of the item scores between BAAD-FM and BAAD-OT. The coincidence rate between the two BAAD tests on an item-by-item basis was over 64% for all items but one (43%). In conclusion, the total BAAD-FM score seemed comparable to the total BAAD-OT score and valuable for detecting attentional disturbance.
ABSTRACT
The patient was a 70-year-old man. He was injured in a motorcycle accident and was brought to the hospital suffering from pulmonary contusions, multiple rib fractures, and a dislocation fracture of the left hip joint. Mechanical ventilation and tracheostomy were performed because of decreased oxygenation. As dysphagia and gait disturbance persisted even after his respiratory condition improved, the patient was transferred to our institution for rehabilitation 63 days after the injury. Rehabilitative intervention for the patient's physical impairments progressed smoothly, and the patient regained independence in activities of daily living. However, 1 week before his scheduled date of discharge, the patient suffered from sudden heart failure at 168 days after the initial injury. Traumatic aortic regurgitation was diagnosed based on the following findings : aortic regurgitation rapidly exacerbated after heart failure, no medical history of heart disease, and no other cause for aortic regurgitation. Surgical treatment with aortic valve replacement was performed. Postoperative recovery was favorable, and the patient was discharged to his home after regaining independence in activities of daily living. Traumatic aortic regurgitation is rare, and patients with this disease often suffer heart failure from a few days to several years after injury. This condition needs to be kept in mind during the rehabilitation process following chest trauma.