[A case study of a patient with myotonic dystrophy type 1 whose gait disturbance was improved by gait training with hybrid assistive limbs].

A Japanese woman first noticed dysarthria at the age of 23. She visited a hospital at the age of 32 and was diagnosed as having myotonic dystrophy clinically. She was diagnosed genetically as having myotonic dystrophy type 1 at 47 years old with 160-270 CTG repeats on the DMPK gene. At the age of 48, she needed non-invasive positive pressure ventilation because of hypoxia at night. Her gait function also deteriorated. She could not stand up from the supine position by herself. However, when she stood, she could walk without a cane for a short distance. She was admitted to our hospital to receive rehabilitation against progressive gait disturbance at the age of 53. She received gait training with hybrid assistive limb® (HAL®). We evaluated some parameters such as walking distance of 2-minute walk test (2MWT), gait speed /cadence/stride length of 10-meter walk test (10MWT), before and just after the course. The first course was performed in September 2017 and the second was done in May 2018 so the interval was about six months. After two courses of HAL® gait training, the distance on the 2-minute walk test increased from 111 m to 154 m, the average speed and the cadence of 10MWT improved from 2.01 m/s to 2.78 m/s and from 2.21 steps/s to 3.05 steps/s respectively. The score of the muscular disability quality of life (QOL) rating scale was also improved. The factors including "defecation," "breathing," and "ADL" suggest that the patient's physical abilities improved and she could move easily. Other factors such as "hope", "activity" and "human relationship" suggest that patient's mood improved after the HAL® training.It was suggested that HAL® gait training could improve QOL as well as gait function in patients with progressive neuromuscular disorder.

Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) as a Cognitive Evaluation Tool for Patients with Normal Pressure Hydrocephalus.

External lumbar drainage (ELD) is recognized as a screening method for ventriculo-peritoneal shunting (VPS) candidacy for possible normal pressure hydrocephalus (NPH). This study focused on the ELD predictability of the cognitive outcome after VPS for NPH. In addition, Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) was examined in ELD cognition screening. ELD results were considered positive with any improvement in gait and/or cognition. Among 36 patients examined for possible NPH, 26 underwent VPS because of positive ELD. Cognitive outcome after VPS was assessed at 6-month follow-up. The RBANS scores, examined pre- and post-ELD, were evaluated statistically to identify consistency with the neuropsychologist judgment and the predictability of cognitive outcome after VPS. Among 26 shunted patients, gait was improved in 24. Cognitive improvement was rated in 19, and there were 9 false negative and 5 false positive in ELD cognition screening. The neuropsychologist judgment in ELD cognition screening is most consistent with the RBANS score in delayed memory. The patients rated as improved in cognition after VPS had significantly lower RBANS scores pre-ELD in immediate memory and delayed memory. If both scores at pre-ELD were ≤ 80 (13 patients), all were rated as improved in cognition after VPS. ELD screening was highly predictive of clinical gait improvement but not of cognitive improvement after VPS for possible NPH. Particularly among patients with a positive ELD gait response, pre-ELD low RBANS scores in memory predicted cognitive improvement after VPS. RBANS seems effective in evaluating cognition for NPH.

Histological evidence of intraoperative monitoring efficacy in selective dorsal rhizotomy.

PURPOSE: It has been advocated that intraoperative electrophysiological monitoring is mandatory in selective dorsal rhizotomy (SDR). However, it is still uncertain whether the monitoring procedure effectively differentiates dysfunctional rootlets. We histologically analyzed sectioned rootlets in SDR, in order to confirm the efficacy of the monitoring. METHODS: Seven children with cerebral palsy underwent SDR on the same protocol. The pieces of their sectioned nerve rootlets from L5 were examined histologically using an electron microscope. In each patient, two nerve rootlets, one with the most abnormal response to intraoperative electrical stimulation and the other with the least abnormal response, were examined. The electron microscope findings of the rootlets were compared with the electromyography (EMG) findings in the intraoperative stimulation. RESULTS: Among 14 examined nerve rootlets, definite abnormal EMG findings were seen in 5, which were 4 clonuses and one bilateral spread. All five rootlets with abnormal EMG findings showed axonal degenerations except one case, whose finding was dysmyelination. On the contrary, in the nine rootlets with normal EMG findings, eight rootlets had histologically minimum changes limited to the myelin sheath and one rootlet had dysmyelination without axonal degeneration. CONCLUSIONS: The nerve rootlets with abnormal EMG findings in the intraoperative stimulation have definite histological abnormalities. This indicates that intraoperative monitoring is a meaningful method for identifying the nerve rootlets to be sectioned. This finding should be reminded as a precaution when considering nonselective dorsal rhizotomy, especially, for children who are expected to stand up after the surgery.