Demyelinating hypertrophic inferior alveolar nerve mimicking a nerve tumor.

We herein report a patient with demyelinating inferior alveolar nerve hypertrophy, which was initially suspected to have a nerve tumor. A 39-year-old woman with childhood-onset polyneuropathy presented with tooth pain and visited a dental clinic. An X-ray examination of the mandible revealed enlargement of the mandibular canal, and a nerve tumor was suspected. CT scan and MRI showed hypertrophy of the inferior alveolar nerve along its entire length. We diagnosed the patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which was supported by the spontaneous recovery reported in her childhood, the results from a nerve conduction study and MRI data. CIDP should be considered in the differential diagnosis of mandibular canal enlargement.

Optimization of intravenous immunoglobulin in chronic inflammatory demyelinating polyneuropathy evaluated by grip strength measurement.

BACKGROUND: Optimal dose and timing of repeated intravenous immunoglobulin therapy (IVIg) for intractable chronic inflammatory demyelinating polyneuropathy (CIDP) patients have not been determined. The aim of this study was to optimize dose and timing of IVIg for CIDP patients who need frequent IVIg using daily grip strength measurement. METHODS: Repeated IVIg were administered for two intractable CIDP patients. Grip strength was recorded at home every day to access the clinical change in symptoms, and dose and timing of IVIg were optimized based on the results. RESULTS: The decrement on grip strength was a sensitive indicator of symptom exacerbation. 100 g of IVIg had a limited effect for each patient. In one patient, symptoms maintained after monthly 60 g of IVIg. In another, 100 g of IVIg every 7 weeks resulted in a marked improvement. After receiving 20 g of IVIg weekly, each patient showed further improvement. CONCLUSION: Optimal dose and timing possibly vary in each individual patient. Dose titration of IVIg is necessary to avoid over- and undertreatment. The daily self-monitoring of grip strength is a helpful tool for clinical assessment in CIDP.

Snoring is associated with an impaired motor function, disease severity and the quality of life but not with excessive daytime sleepiness in patients with Parkinson's disease.

OBJECTIVE: There are conflicting results regarding the frequency and clinical significance of sleep related breathing disorders in patients with Parkinson's disease (PD). The aim of this study was to investigate the relationship between snoring and its clinical correlates in patients with PD. METHODS: A total of 93 PD patients and 93 controls were analyzed from a previously conducted cross-sectional study. Snoring was defined as a snoring frequency of ≥ 2 days/week (a score of 2 or higher on the PD Sleep Scale-2 subitem 15). Excessive daytime sleepiness (EDS) was defined as an Epworth Sleepiness Scale score of ≥ 10. RESULTS: Snoring was more prevalent in the patients with PD than in the controls (14.0% vs. 1.1%). The PD patients who snored exhibited greater disease severity, worse scores on the motor section of the Unified Parkinson's Disease Rating Scale (UPDRS) and the Parkinson fatigue scale and more impaired scores in several domains of the Parkinson's Disease Questionnaire, including the domains of mobility, activities of daily living, emotional well-being, communication and bodily discomfort, when compared to those who did not snore. No between-group differences were found in EDS. A higher proportion of the UPDRS motor scores for bradykinesia was seen in the PD patients who snored compared to that observed in the PD patients who did not snore. CONCLUSION: We found that snoring was more frequent in PD patients than in controls. Furthermore, snoring in PD patients was associated with disease severity, an impaired motor function and a decreased quality of life, although it was not associated with EDS.

Probable rapid eye movement sleep behavior disorder, nocturnal disturbances and quality of life in patients with Parkinson's disease: a case-controlled study using the rapid eye movement sleep behavior disorder screening questionnaire.

BACKGROUND: Increasing evidence provides a clear association between rapid eye movement sleep behavior disorders (RBD) and Parkinson's disease (PD), but the clinical features that determine the co-morbidity of RBD and PD are not yet fully understood. METHODS: We evaluated the characteristics of nocturnal disturbances and other motor and non-motor features related to RBD in patients with PD and the impact of RBD on their quality of life. Probable RBD (pRBD) was evaluated using the Japanese version of the RBD screening questionnaire (RBDSQ-J). RESULTS: A significantly higher frequency of pRBD was observed in PD patients than in the controls (RBDSQ-J ≥ 5 or ≥ 6: 29.0% vs. 8.6%; 17.2% vs. 2.2%, respectively). After excluding restless legs syndrome and snorers in the PD patients, the pRBD group (RBDSQ-J≥5) showed higher scores compared with the non-pRBD group on the Parkinson's disease sleep scale-2 (PDSS-2) total and three-domain scores. Early morning dystonia was more frequent in the pRBD group. The Parkinson's Disease Questionnaire (PDQ-39) domain scores for cognition and emotional well-being were higher in the patients with pRBD than in the patients without pRBD. There were no differences between these two groups with respect to the clinical subtype, disease severity or motor function. When using a cut-off of RBDSQ-J = 6, a similar trend was observed for the PDSS-2 and PDQ-39 scores. Patients with PD and pRBD had frequent sleep onset insomnia, distressing dreams and hallucinations. The stepwise linear regression analysis showed that the PDSS-2 domain "motor symptoms at night", particularly the PDSS sub-item 6 "distressing dreams", was the only predictor of RBDSQ-J in PD. CONCLUSION: Our results indicate a significant impact of RBD co-morbidity on night-time disturbances and quality of life in PD, particularly on cognition and emotional well-being. RBDSQ may be a useful tool for not only screening RBD in PD patients but also predicting diffuse and complex clinical PD phenotypes associated with RBD, cognitive impairment and hallucinations.

Nocturnal disturbances and restlessness in Parkinson's disease: using the Japanese version of the Parkinson's disease sleep scale-2.

OBJECTIVE: The aim of this study was to assess the validity and the reliability of the Japanese version of the Parkinson's disease sleep scale (PDSS)-2 and to use this scale to identify nocturnal symptoms and their impact on patient's quality of life. METHODS: A cross-sectional, case-controlled study was conducted consisting of 93 patients with Parkinson's disease (PD) and 93 age- and gender-matched control subjects. The Japanese version of the PDSS-2 was used for the evaluation of nocturnal disturbances. The patient's quality of life was evaluated with the Parkinson's Disease Quality of Life questionnaire (PDQ-39) and their depressive symptoms were assessed with the Beck Depression Inventory-II (BDI-II), respectively. In addition, the Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS) and Parkinson Fatigue Scale (PFS) were administered. RESULTS: As assessed using the PDSS-2, PD patients had significantly impaired scores compared with control subjects (15.0±9.7 vs. 9.1±6.6, p<0.001). The ESS, BDI-II and PFS scores were significantly impaired in PD patients compared with controls. A satisfactory internal consistency and test-retest reliability score were obtained for the PDSS-2 total score (Cronbach's alpha=0.86). The PDSS-2 was correlated with the PSQI, ESS, BDI-II, PFS, PDQ-39 summary index, all of the PDQ-39 domains and Unified Parkinson's Disease Rating Scale part III. The frequency of restless legs syndrome (RLS) was not significantly different between PD patients and controls (5.5% vs. 2.2%), but nocturnal restlessness was significantly more frequent in PD patients than controls. Stepwise linear regression analyses revealed the PDQ-39 summary index and the PSQI global score as significant predictors for the PDSS-2 total score. CONCLUSIONS: Our study confirmed the usefulness of the Japanese version of the PDSS-2 that enables the comprehensive assessment of nocturnal disturbances in PD. The association between RLS and nocturnal restlessness in PD requires further study.