High-frequency motor rehabilitation in amyotrophic lateral sclerosis: a randomized clinical trial.

OBJECTIVE: Exercise may be physically and psychologically important for people with ALS, especially in the earlier stages of the disease, and, as a consequence, current ALS clinical management includes individualized rehabilitation as part of multidisciplinary care because. However, while recent studies focused on which type of exercise is more indicated to ALS patients, there is no evidence at which frequency training sessions should be performed. METHODS: We performed an assessor blinded randomized clinical trial to investigate the superiority of two different frequencies of exercise on rate of progression in ALS. We enrolled 65 patients in two groups: intensive exercise regimen (IER, five sessions/week) versus usual exercise regimen (UER, two sessions/week). The primary aim was to assess if IER decreased disease progression, measured through Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised, with respect to UER. Secondary aims included assessment of adverse events, tracheostomy-free survival, motor and respiratory functions, fatigue, quality of life and caregiver burden. Treatment regimen consisted for both groups of the same kind of exercise including aerobic training, endurance training, stretching or assisted active mobilization, differing for frequency of intervention. RESULTS: No significant changes in disease progression were found in patients under IER versus UER. At the end of the study, there were no significant differences between the two groups in survival, respiratory function, time to supporting procedures, and quality of life. Adverse events, fatigue, and caregiver burden were not different between the two treatment regimens. CONCLUSIONS: Despite some limitations, our trial demonstrated that high-frequency physical exercise was not superior to UER on ALSFRS-R scores, motor and respiratory functions, survival, fatigue, and quality of life of ALS patients.

Effects of a "test in-train out" walking program versus supervised standard rehabilitation in chronic stroke patients: a feasibility and pilot randomized study.

BACKGROUND: The loss of normal ambulatory function after stroke, besides causing disability, leads to progressive deconditioning and exposes patients to increased risk of cardiovascular diseases and recurrent stroke. Conventional rehabilitation is mainly limited to the subacute period after stroke. Effective, safe and sustainable interventions for patients and healthcare system, including the long-term, should be identified. AIM: To verify the feasibility, safety and preliminary efficacy of an original home-based rehabilitation model compared to a standard supervised program in chronic hemiplegic stroke survivors. DESIGN: Pilot, two-arm, parallel group, randomized, controlled clinical trial. SETTING: Community-dwelling poststroke patient/Hospital. POPULATION: Twelve chronic hemiplegic stroke patients (age=66.5±11.9 years, males, N.=9). METHODS: Participants were randomly assigned for a 10-week period to a structured home-based exercise program (N.=6) and a standard supervised group-setting program (N.=6). The feasibility outcomes included adherence to interventions, retention rate and safety. Satisfaction was also evaluated by the Client Satisfaction Questionnaire. Efficacy was assessed by the 6-minute walk test, Timed Up and Go and Stair Climb tests. The impact on Quality-of-life was estimated using the physical activity domain of the Short Form-36 questionnaire. Operators' time consuming was also calculated. RESULTS: Adherence was 91% in the home-based exercise group and 92% in the standard supervised group. The retention rate was 100%, with no adverse events reported and high satisfaction scores for both interventions. 6-minute walk test and physical activity domain significantly increased in both groups (P=0.03). Timed Up and Go improved in both groups, significantly for the home-based exercise group (P=0.03) while Stair Climb remained stable. Time required to operators to implement the home-based exercise program was 15 hours vs. 30 hours for the standard supervised one. CONCLUSIONS: In a sample of hemiplegic chronic stroke patients, a structured home-based exercise program was feasible, safe and capable of inducing improvements in functional capacity and Quality-of-life comparable to a conventional supervised rehabilitation program A future larger randomized controlled trial will be needed to confirm such results. CLINICAL REHABILITATION IMPACT: With the limitation of a small sample size, the study suggested that a home-based program for chronic stroke might be an effective alternative to traditional supervised programs with the peculiarity of being sustainable for patients and healthcare system.

Relationship between ultrasonographic, electromyographic, and clinical parameters in adult stroke patients with spastic equinus: an observational study.

OBJECTIVE: To find more accurate indices that could affect decisions in spasticity treatment by investigating the relation between ultrasonographic, electromyographic, and clinical parameters of the gastrocnemius muscle in adults with spastic equinus after stroke. DESIGN: Observational study. SETTING: University hospitals. PARTICIPANTS: Chronic patients with stroke with spastic equinus (N=43). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Ultrasonographic features were spastic gastrocnemius muscle echo intensity, muscle thickness, and posterior pennation angle of the gastrocnemius medialis (GM) and gastrocnemius lateralis (GL) in both legs. Electromyographic evaluation included compound muscle action potentials (CMAPs) recorded from the GM and GL of both legs. Clinical assessment of the spastic gastrocnemius muscle was performed with the Modified Ashworth Scale (MAS) and by measuring ankle dorsiflexion passive range of motion (PROM). RESULTS: Spastic muscle echo intensity was inversely associated with proximal (GM and GL: P=.002) and distal (GM and GL: P=.001) muscle thickness, pennation angle (GM: P< .001; GL: P=.01), CMAP (GM: P=.014; GL: P=.026), and ankle PROM (GM: P=.038; GL: P=.024). The pennation angle was directly associated with the proximal (GM and GL: P< .001) and distal (GM: P=.001; GL: P< .001) muscle thickness of the spastic gastrocnemius muscle. The MAS score was directly associated with muscle echo intensity (GM: P=.039; GL: P=.027) and inversely related to the pennation angle (GM and GL: P=.001) and proximal (GM: P=.016; GL: P=.009) and distal (GL: P=.006) muscle thickness of the spastic gastrocnemius. CONCLUSIONS: Increased spastic muscle echo intensity was associated with reduced muscle thickness, posterior pennation angle, and CMAP amplitude in the gastrocnemius muscle. Building on previous evidence that these instrumental features are related to botulinum toxin response, these new findings may usefully inform spasticity treatment decisions.

A heroin addict with focal weakness.

A 24-year-old female with 5 year history of heroin abuse experienced painless stiffness of elbow joints and weakness of shoulder and upper limb muscles. She was injecting herself 4-6 times daily alternatively in the upper extremities, sparing the lower limbs. Electromyography (EMG) showed myopathic changes in clinically affected and unaffected muscles. Magnetic resonance imaging (MRI) revealed muscle fibrosis in directly injected muscles, whereas in subcutaneous fat and within muscles of anterior and posterior compartments of both thighs, not directly injected, there were signal changes supportive of oedema and inflammation. EMG and MRI were congruent in showing abnormalities in muscles not directly injected, suggesting long distant effects of heroin or adulterants with a mechanism either toxic or immunologically mediated.

Primary cutaneous adenoid cystic carcinoma of the upper lid: a case report and literature review.

To report on the diagnostic challenge of an adenoid cystic carcinoma arising from the eyelid. A 77-year-old male was referred to our center with a clinical diagnosis of upper eyelid chalazion for a lesion that had appeared 2 years before. A loss of cilia was observed over the cutaneous area of induration, but there was no reddening or ulceration. Incisional biopsy was performed and the specimen was submitted in formalin for histopathological examination. On light microscopy, the lesion was composed of basaloid epithelial and myoepithelial cells that were arranged in strands or nests and associated with cystic spaces that contained a deeply eosinophilic secretory substance and an Alcian blue-positive material, characteristic of adenoid cystic carcinoma. After histological diagnosis, tumor re-excision was performed to ensure adequacy of resection margins, as well as a sentinel lymph node procedure, resulting in complete excision of the malignant tumor. No recurrence was observed during the first 18 months after surgery. Adenoid cystic carcinoma is a rare and aggressive epithelial malignancy, which tends to grow slowly and should be considered in the differential diagnosis of eyelid tumors simulating chalazion.

Orbital dermoid cyst of childhood: clinical pathologic findings, classification and management.

To analyze the characteristics and treatment outcomes of pediatric orbital dermoid cysts. Chart review of consecutive pediatric biopsy-proven dermoid cysts surgically removed at the Department of Ophthalmology, Maggiore Hospital, between 2000 and 2007. We excised dermoid cysts from 30 children (30 eyes) whose mean age at the time of surgery was 24 months (range 6-84). The most common presentation of the cyst was a palpable or partially palpable mass (100%), followed by a superior lid ptosis (10%). Twenty patients (67%) had superficial cysts with margins well-definable by palpation, and 10 patients (33%) had deep cysts that extended beyond the orbital rim with an incomplete palpation of margins requiring imaging studies. The most frequent localization of the cysts was the superior temporal zygomatico-frontal suture (86.6%), followed by the superior nasal frontal suture (10%). Complete removal of the cysts was achieved and confirmed histopathologically, and there were no recurrences among the patients at a mean follow-up of 28 months (range 6-73). One child, however, developed a temporary orbital hematoma. The superficial cysts had an anatomic mean diameter of 10 mm (range 2.5-15), and the mean age of the patients at surgery was 19 months (range 6-31). The deep cysts had a larger diameter with a mean of 14 mm (range 10-30) (P = 0.008), and the children were older at presentation with a mean age of 34 months (range 15-84) (P = 0.03). There was a statistically significant difference (P < 0.05) between the two groups (superficial and deep) when comparing age and diameter. Cysts with palpably distinct margins (superficial) can be easily and completely excised with no recurrence. Cysts with indistinct margins need detailed computed tomography or magnetic resonance imaging investigation because they may require deep orbital dissection. Complete excision of the cyst's capsule may be difficult as a result. In our review we have not found cysts with intracranial extension.

Repeatability of a new protocol for gait analysis in adult subjects.

The reliability of kinematic measurements has a top priority in gait analysis. The aim of the present work was to assess the inter-trial, inter-session and inter-examiner variability of an anatomical-based protocol by an established method. The gait of two young adult volunteers was analyzed by four examiners with different degrees of experience in three sessions 1 week apart. The data of five trials of level walking were collected and the rotations in the three anatomical planes of the pelvis, hip, knee and ankle were calculated. The mean value over the gait cycle of the standard deviation of these rotations was calculated for each of the 24 groups of five trials (inter-trial), the eight groups of five trials multiplied by three sessions (inter-session), and the two groups of five trials multiplied by three sessions multiplied by four examiners (inter-examiner). For each rotation, the inter-examiner variability was larger than the inter-session, and the latter larger than the inter-trial. The present ratio between inter-examiner and inter-trial variability was found to be smaller than that of the conventional protocol for each of the gait variables.

Inter-operator reliability and prediction bands of a novel protocol to measure the coordinated movements of shoulder-girdle and humerus in clinical settings.

A clinical motion analysis protocol was developed to measure the coordinated movements of shoulder-girdle and humerus (girdle-humeral rhythm--GD-H-R) during humerus flexion-extension (HFE) and ab-adduction (HAA), through an optoelectronic system. In particular, the protocol describes the GD-H-R with 2 angle-angle plots for each movement: girdle elevation-depression and protraction-retraction vs HFE, and vs HAA. Each of these plots is further divided in two subplots, one for the upward and one for the downward phases of the movement. By involving 11 participants and 2 operators, we measured the protocol's inter-operator reliability which ranged from very-good to excellent depending on the angle-angle plot (median values of the inter-operator coefficient of multiple correlation for the angle-angle plots higher than 0.94). We then computed the subjects' average control patterns, together with statistically meaningful prediction bands. +/-1SD confidence bands were also computed and their width ranged from +/-0.5 degrees to +/-4.6 degrees. Based on these results we could conclude that the method is robust and able to identify even limited differences in the GD-H-R.

European consensus table on the use of botulinum toxin type A in adult spasticity.

A group of clinicians from across Europe experienced in the use of botulinum toxin type A for the treatment of spasticity following acquired brain injury gathered to develop a consensus statement on best practice in managing adults with spasticity. This consensus table summarizes the current published data, which was collated following extensive literature searches, their assessment for level of evidence and discussion among the whole group. Published information is supplemented by expert opinion based on clinical experience from 16 European countries, involving 28 clinicians, who treat an average of approximately 200 patients annually, representing many thousand spasticity treatments with botulinum toxin per year.