Unravelling cossed wires : dysfunction in obstetric brachial plexus lesions in the light of intertwined effects of the peripheral and central nervous system

Sensory function is impaired in adults with conservatively treated OBPL. There is widespread motor misrouting together with motor functional impairment in conservatively treated OBPL, not explained by muscle weakness. There were no differences in the degree of cocontraction between OBPL patients and healthy subjects for either the triceps or deltoid muscles during supramaximal biceps stimulation. However, elbow stiffness was approximately 1.7 times higher in OBPL patients than in control subjects during voluntary levels of contraction, suggesting a significant effect of misrouting in the patients. In children with OBPL the deficit during automatic arm abduction was not observed during voluntary movements and therefore cannot be explained by a peripheral deficit, suggesting a central component. In adults OBPL affected imagined but not actual elbow flexion suggested an impairment of motor planning. LUMC / Geneeskund

A cross-sectional study of hand sensation in adults with conservatively treated obstetric brachial plexus lesion

Scientific Assessment and Innovation in Neurosurgical Treatment Strategie

Extensive motor axonal misrouting after conservative treatment of obstetric brachial plexus lesions

Paroxysmal Cerebral Disorder

Sleep and circadian rhythm alterations correlate with depression and cognitive impairment in Huntington's disease

Objective: Sleep disturbances are a prominent feature of Huntington's disease (HD) and can substantially impair patiems' quality of life. However, sleep complaints and their association with other symptoms and signs of HD have not yet been assessed in large groups of patients or premanifest mutation carriers. Therefore, we aimed to delineate the nature of subjective sleep disturbances and identify important correlates of sleep impairment in HD patients and premanifest mutation carriers. Subjects & methods: Using standardized questionnaires (including Epworth's Sleepiness Scale, Pittsburgh Sleep Quality Index, SCOPA-SLEEP, and Beck's Depression Inventory), daytime sleepiness, night-time sleep, and depressed mood were assessed in 63 HD patients, 21 premanifest mutation carriers and 84 controls. Results: Night-time sleep impairment was significantly more prevalent in HD patients compared with controls (58.1% vs. 34.9%, p = 0.012), but daytime sleepiness was not (12.7% vs. 7.9%, p = 0.560). Depression was the only independent correlate of night-time sleep impairment in HI) patients, accounting for 10% of the variance. Compared with controls, both sleep onset latency and wake-up time were significantly delayed in HD patients. Moreover, in HD patients, later wake-up time was significantly associated with cognitive score (r = -0.43), total functional capacity (r = -0.54) and depressive symptoms (r = +0.47). In general, the degree of sleep (phase) changes in premanifest mutation carriers lay in between those of HD patients and controls. Conclusions: HD is primarily accompanied by night-time sleep disturbances and a delayed sleep phase, which are associated with depression and lower cognitive as well as functional performance. (C) 2010 Elsevier Ltd. All rights reserved.Pathophysiology of paroxysmal and chronic degenerative progressive disorder of the central and periferal nervous syste

Autonomic symptoms in patients and pre-manifest mutation carriers of Huntington's disease

Background and purpose: Although autonomic function tests have revealed abnormalities of the autonomic nervous system in Huntington's disease (HD), autonomic symptoms and their association with other symptoms and signs of HD have not yet been assessed in large groups of patients or pre-manifest mutation carriers. Therefore, we aimed at delineating the characteristics and correlates of autonomic symptoms in HD. Methods: Using the scales for outcomes in Parkinson's disease-autonomic symptoms (SCOPA-AUT) and Beck Depression Inventory questionnaires, autonomic symptoms and depressed mood were assessed in 63 patients with HD, 21 pre-manifest mutation carriers, and 85 controls. The Unified Huntington's Disease Rating Scale was used to assess other HD symptoms and signs. Results: Relative to controls, patients with HD experienced significantly more gastrointestinal, urinary, cardiovascular and, in men, sexual problems. The most prevalent symptoms were swallowing difficulties, erection and ejaculation problems, dysphagia, sialorrhea, early abdominal fullness, straining for defecation, fecal and urinary incontinence, urgency, incomplete bladder emptying, and light-headedness whilst standing. Pre-manifest mutation carriers experienced significantly more swallowing difficulties and light-headedness on standing up compared with controls. In patients with HD, autonomic symptoms were associated with a greater degree of functional disability, more severe depression, and antidepressant drugs use. However, depression was the only independent predictor of autonomic dysfunction. Conclusions: Autonomic symptoms are highly prevalent in patients with HD and may even precede the onset of motor signs. Moreover, autonomic dysfunction is related to functional disability and depression in HD.Neurological Motor Disorder

Increased brain activation during motor imagery suggests central abnormality in Neonatal Brachial Plexus Palsy

Scientific Assessment and Innovation in Neurosurgical Treatment Strategie

Impaired Automatic Arm Movements in Obstetric Brachial Plexus Palsy Suggest a Central Disorder

Neurological Motor Disorder

Cocontraction measured with short-range stiffness was higher in obstetric brachial plexus lesions patients compared to healthy subjects

We suggest short range stiffness (SRS) at the elbow joint as an alternative diagnostic for EMG to assess cocontraction. Elbow SRS is compared between obstetric brachial plexus lesion (OBPL) patients and healthy subjects (cross-sectional study design). Seven controls (median 28years) and five patients (median 31years) isometrically flexed and extended the elbow at rest and three additional torques [2.1,4.3,6.4Nm] while a fast stretch stimulus was applied. SRS was estimated in silico using a neuromechanical elbow model simulating the torque response from the imposed elbow angle. SRS was higher in patients (250±36Nm/rad) than in controls (150±21Nm/rad, p=0.014), except for the rest condition. Higher elbow SRS suggested greater cocontraction in patients compared to controls. SRS is a promising mechanical alternative to assess cocontraction, which is a frequently encountered clinical problem in OBPL due to axonal misrouting.Pathofysiological analysis of movement disorders in relation to functio