Impact of social-functioning and sleep on quality of life in chronic inflammatory demyelinating polyneuropathy.

INTRODUCTION/AIMS: The impact of impairment of social functioning and sleep on health-related quality of life (HR-QoL), is unknown in chronic inflammatory demyelinating polyneuropathy (CIDP). The value of the Chronic Acquired Polyneuropathy Patient-Reported Index (CAP-PRI) to identify potential social functioning and sleep issues is equally unknown. METHODS: We performed a cross-sectional evaluation of social functioning and sleep using the "Scales for Outcomes in Parkinson's Disease" (SCOPA) in 40 subjects with clinically-stable CIDP through a structured questionnaire. We assessed HR-QoL through the CAP-PRI. Disability was evaluated through the Overall Neuropathy Limitation Score (ONLS). RESULTS: SCOPA social functioning scores were impaired at least "a little" per averaged item in > 50 % of subjects, and at least "quite a bit" per averaged item in > 20 %. Most affected items were (i) difficulty with work/household/other chores (ii) difficulties with hobbies/sport/leisure activities. SCOPA sleep sub-scores indicated at least "a little concern" for night-time sleep in nearly 50 % of subjects. Abnormal sleep timing was rare. Associations were found between both SCOPA social-functioning and SCOPA sleep scores and the CAP-PRI. Linear regression demonstrated the SCOPA social-functioning score was independently associated with the CAP-PRI. The CAP-PRI showed high association with disability scores, good internal consistency, absence of ceiling effect, absence of significant floor-effect, and good criteria-related as well as construct-related validity. DISCUSSION: Social functioning and night-time sleep are frequently affected in CIDP and impact on HR-QoL. In contrast to traditional disability scales, the CAP-PRI additionally allows adequately capturing these impairments and may represent an adequate holistic outcome measure.

Nitrous oxide-induced myeloneuropathy: a case series.

BACKGROUND: Nitrous oxide (N2O) is the second most common recreational drug used by 16- to 24-year-olds in the UK. Neurological symptoms can occur in some people that use N2O recreationally, but most information comes from small case series. METHODS: We describe 119 patients with N2O-myeloneuropathy seen at NHS teaching hospitals in three of the UK's largest cities: London, Birmingham and Manchester. This work summarises the clinical and investigative findings in the largest case series to date. RESULTS: Paraesthesia was the presenting complaint in 85% of cases, with the lower limbs more commonly affected than the upper limbs. Gait ataxia was common, and bladder and bowel disturbance were frequent additional symptoms. The mid-cervical region of the spinal cord (C3-C5) was most often affected on MRI T2-weighted imaging. The number of N2O canisters consumed per week correlated with methylmalonic acid levels in the blood as a measure of functional B12 deficiency (rho (ρ)=0.44, p=0.04). CONCLUSIONS: Preventable neurological harm from N2O abuse is increasingly seen worldwide. Ease of access to canisters and larger cylinders of N2O has led to an apparent rise in cases of N2O-myeloneuropathy in several areas of the UK. Our results highlight the range of clinical manifestations in a large group of patients to improve awareness of risk, aid early recognition, and promote timely treatment.

Patient perceptions of outcome measures in chronic inflammatory demyelinating polyneuropathy: A study of the Inflammatory Rasch-built Overall Disability Scale.

BACKGROUND: Patients' perceptions of outcome measures used in chronic inflammatory demyelinating polyneuropathy (CIDP) are unknown. METHODS: We performed a cross-sectional evaluation of patient perceptions of the Inflammatory Rasch-built Overall Disability Scale (I-RODS) from 41 subjects with CIDP through a structured questionnaire. We assessed perceived hesitation to provide a response, item importance and relevance, understanding of specific items and factors affecting responses. RESULTS: Hesitation to provide a categorical answer was reported by 20% of subjects or more, for 5/24 (20.8%) items. Uncertainty was most frequent for "travel by public transport" (22.4%) and "catch an object (e.g., ball)" (24%). Six of 24 (25%) items were perceived as unimportant to their disease by at least a third of participants. Items most commonly perceived as unimportant were "travel by public transport" in 53.7%, "catch an object (e.g., ball)" in 61% and "dance" in 65.9%. Several items were frequently perceived as irrelevant. These included "move a chair" (39%), "do the dishes" (46.3%), "catch an object (e.g., ball)" (61%), "travel by public transport" (68.3%) and "stand for hours" (82.9%). The understanding of multiple items such as "read a book", "sit on a toilet" and "take a shower" was found to be highly variable. Fatigue was perceived more commonly than mood (53.7% vs. 17.1%, p = 0.001), and more commonly in younger subjects (p = 0.037), as influencing responses to the I-RODS. CONCLUSIONS: Patient-perceived uncertainty, unimportance, irrelevance and poor understanding of items, as well as fatigue and mood, impact on the value of the I-RODS. Greater emphasis on individualized disability assessments requires consideration in future.

Thromboembolic risk with IVIg: Incidence and risk factors in patients with inflammatory neuropathy.

Our objective was to evaluate whether IV immunoglobulin (IVIg) increases the risk of thromboembolic events in neurology outpatients with inflammatory neuropathies, as there is conflicting evidence supporting this hypothesis, mainly from non-neurologic cohorts. We investigated this question over 30 months in our cohort of patients with inflammatory neuropathies receiving regular IVIg and found a greater incidence of arterial and venous thromboembolic events than population-based rates determined by hospital admissions data. Vascular risk factors were more common in the event group but there were no IVIg administration factors that contributed to the risk. This study suggests that IVIg may have a small but contributory role in determining thromboembolic risk in the inflammatory neuropathy cohort and more evidence is required before it is clear whether the current primary prevention guidelines are appropriate in this group of patients.