Myelo-optico-neuropathy in copper deficiency occurring after partial gastrectomy. Do small bowel bacterial overgrowth syndrome and occult zinc ingestion tip the balance?

Acquired copper deficiency has recently been recognized as a cause of myeloneuropathy mimicking subacute combined degeneration due to vitamin B-12 deficiency. A remote history of gastric surgery is frequently associated with this syndrome. However, the very limited prevalence of severe copper deficiency in patients with a history of gastric surgery suggests that additional contributing factors are likely to be involved. We describe a patient with copper deficiency and a previous Billroth II partial gastrectomy for gastric carcinoma, presenting with severe myelo-optico-neuropathy, demyelinating lesions of the brain, and subjective hyposmia. An abnormal glucose breath test also revealed small bowel bacterial overgrowth syndrome. Copper replacement therapy associated with antibiotic therapy was effective in preventing further neurological damage and in obtaining mild improvement. We propose that copper status should be evaluated in all patients presenting with unexplained noninflammatory myeloneuropathy. Small bowel bacterial overgrowth syndrome should be investigated as a cause of generalized malabsorption and a possible contributing factor to copper deficiency after gastric surgery, as should occult zinc ingestion.

Severe dysphagia in lower cranial nerve involvement as the initial symptom of Wegener's granulomatosis.

We observed a 42-year-old woman presenting with severe dysphagia secondary to paralysis of the lower cranial nerves and right phrenic nerve involvement, followed by respiratory failure. An EMG confirmed bilateral denervation of the 9th, 10th, 11th and 12th cranial nerves and right phrenic nerve. Videolaryngoscopy showed bilateral vocal fold immobility. Anemia, elevated ESR, microhematuria and C-ANCA (PR-3) antibodies were detected. Brain MRI and CSF were normal. A chest CT showed bilateral, irregular pulmonary lesions. An 18F-FDG total body scan showed diffuse hypermetabolic regions in both pulmonary bases, in the mediastinic region and in the rhinopharynx, raising the suspicion of a neoplastic process. A transthoracic biopsy disclosed nodular granulomatous aggregates with multinucleated giant cells, supporting the diagnosis of Wegener's granulomatosis. Immunosuppressive therapy achieved complete clinical resolution and cleared the pulmonary lesions. To the best of our knowledge this is the first report of Wegener's granulomatosis presenting with neurogenic dysphagia due to lower cranial nerve palsy.

Predictive factors for ambulation in stroke patients in the rehabilitation setting: a multivariate analysis.

OBJECT: The purpose of this study was to investigate predictive factors for ambulatory recovery in stroke patients undergoing rehabilitation. METHODS: One hundred and eight-five first-stroke hemiplegics, admitted to an inpatient stroke rehabilitation program, were consecutively recruited to the study. Functional status at admission and discharge was evaluated by the Functional Independence Measure (FIM) and its motor component (motFIM), the upper and lower Motricity Index (upMI and lowMI), and the Trunk Control Test (TCT). The outcome variable was the Functional Ambulation Classification (FAC) score, assessed at discharge from rehabilitation. Multivariate analysis was used to assess the relationships between functional outcome (FAC), and the predictive variables. RESULTS: Up- and lowMI, FIM and motFIM, TCT and age at admission were significantly related to ambulatory recovery at discharge. Logistic regression analysis showed that the independent variables related to FAC were age, TCT and FIM: the model correctly allocated 86 out of 100 cases in the construction set and 76% of cases in the validation set. The ROC curve with logistic function output as the risk factor afforded very good accuracy (ROC area=0.94), sensitivity=86.5% and specificity=85.4%. CONCLUSIONS: Our results show that age and level of motor and functional impairment measured at baseline are significant predictors of ambulatory outcome. These findings promise to be of interest in goal optimization in the rehabilitation setting.

Are antiphospholipid antibodies an independent risk factor for atherosclerosis?

The purpose of this study was to check whether antiphospholipid antibodies (aPL) could be an independent risk factor for atherosclerosis. Eighty-five subjects were studied: 45 with primitive antiphospholipid antibody syndrome and 40 controls affected by deep vein thrombosis secondary to known causes. The two groups were homogeneous for age, sex, and risk factors for atherosclerosis. All the subjects submitted to echo-color doppler of the carotid arteries, femoral arteries, and abdominal aorta. The cases were then subdivided into three subgroups on the basis of the positivity to the three subpopulations of aPL. Results demonstrate that there is no correlation between aPL and atherosclerosis. The different positivity to aPL does not modify this conclusion.

Randomized trial of a robotic assistive device for the upper extremity during early inpatient stroke rehabilitation.

BACKGROUND: A recent Cochrane Review showed that early robotic training of the upper limb in stroke survivors can be more effective than other interventions when improving activities of daily living involving the arm function is the aim of therapy. OBJECTIVE: We tested for efficacy of the study a protocol which involved the use of the NeReBot therapy in partial substitution of standard upper limb rehabilitation in post-acute stroke patients. METHODS: In this dose-matched, randomized controlled clinical trial, 34 hemiparetic participants with movement against gravity in shoulder, elbow, and wrist muscle groups were enrolled within 15 days of the onset of stroke. All participants received a total daily rehabilitation treatment for 120 minutes, 5 days per week for 5 weeks. The control group received standard therapy for the upper limb. The experimental group received standard therapy (65% of exercise time) associated with robotic training (35% of exercise time). Muscle tone (Modified Ashworth Scale), strength (Medical Research Council), and synergism (Fugl-Meyer motor scores) were measured at impairment level, whereas dexterity (Box and Block Test and Frenchay Arm Test) and activities of daily living (Functional Independence Measure) were measured at activity level. All assessments were performed at baseline, at the end of therapy (time T1), at 3 months (time T2), and at 7 months (time T3) after entry. All between-group analyses were tested using nonparametric test with Bonferroni's adjustments for multiple testing. RESULTS: No significant between-group differences were found with respect to demographic characteristics, motor, dexterity, and ADLs at baseline, postintervention (T1) and at follow-up (T2 and T3). CONCLUSIONS: The robot therapy by NeReBot did not lead to better outcomes compared with conventional inpatient rehabilitation.

Upper-limb robot-assisted therapy in rehabilitation of acute stroke patients: focused review and results of new randomized controlled trial.

The successful motor rehabilitation of stroke patients requires early intensive and task-specific therapy. A recent Cochrane Review, although based on a limited number of randomized controlled trials (RCTs), showed that early robotic training of the upper limb (i.e., during acute or subacute phase) can enhance motor learning and improve functional abilities more than chronic-phase training. In this article, a new subacute-phase RCT with the Neuro-Rehabilitation-roBot (NeReBot) is presented. While in our first study we used the NeReBot in addition to conventional therapy, in this new trial we used the same device in substitution of standard proximal upper-limb rehabilitation. With this protocol, robot patients achieved similar reductions in motor impairment and enhancements in paretic upper-limb function to those gained by patients in a control group. By analyzing these results and those of previous studies, we hypothesize a new robotic protocol for acute and subacute stroke patients based on both treatment modalities (in addition and in substitution).

Dysphagia in post-carotid endarterectomy: a prospective study.

Dysphagia can be a debilitating complication in carotid endarterectomy. This study describes our experience in the management of this complication. We prospectively assembled an inception cohort of 19 consecutive, hospital-referred patients with dysphagia post-carotid endarterectomy. We performed swallowing evaluation in all the recruited patients both at the bedside and by fiberoptic endoscopic evaluation of swallowing (FEES) 5 days and 3 months after the operation, using standardized methods and diagnostic criteria. The degree of dysphagia was scored using the Penetration-Aspiration Scale (PAS). All patients were undergoing early rehabilitation treatment and were followed up prospectively for 3 months, during which time recovery of swallowing function and any occurrence of pneumonia were recorded. At baseline, 15 patients presented dysphagia for liquid and solid food and four for solid food only, eight were on parenteral nutrition, and six presented bronchial aspiration (by FEES). The mean PAS score at the first evaluation was 5.2. Ten patients completely recovered swallowing function and returned to their preoperative diet within 1 month, and six did so within 3 months; the other three patients did not complete the follow-up. No patient presented pneumonia. At the 3-month follow-up, one patient presented bronchial aspiration and the mean PAS score was 1.2. This preliminary experience suggests that careful evaluation of swallowing and early rehabilitation may be advisable in these patients.

Robotic-assisted rehabilitation of the upper limb after acute stroke.

OBJECTIVE: To investigate whether early therapy with a novel robotic device can reduce motor impairment and enhance functional recovery of poststroke patients with hemiparetic and hemiplegic upper limb. DESIGN: A single-blind randomized controlled trial, with an 8-month follow-up. SETTING: Neurologic department and rehabilitation hospital. PARTICIPANTS: Thirty-five patients with acute (< or =1 wk of onset), unilateral, ischemic embolic, or thrombotic stroke. INTERVENTIONS: Patients of both groups received the same dose and length per day of standard poststroke multidisciplinary rehabilitation. Patients were randomly assigned to 2 groups. The experimental group (n=17) received additional early sensorimotor robotic training, 4 hours a week for 5 weeks; the control group (n=18) was exposed to the robotic device, 30 minutes a week, twice a week, but the exercises were performed with the unimpaired upper limb. Training by robot consisted of peripheral manipulation of the shoulder and elbow of the impaired limb, correlated with visual stimuli. MAIN OUTCOME MEASURES: The Fugl-Meyer Assessment (FMA) of upper-extremity function (shoulder/elbow and coordination and wrist/hand subsections) to measure each trained limb segment; the Medical Research Council (MRC) score to measure the strength of muscle force during 3 actions: shoulder abduction (MRC deltoid), elbow flexion (MRC biceps), and wrist flexion (MRC wrist flexors); the FIM instrument and its motor component; and the Trunk Control Test (TCT) and Modified Ashworth Scale (MAS). RESULTS: Compared with the patients in the control group, the experimental group showed significant gains in motor impairment and functional recovery of the upper limb after robot therapy, as measured by the MRC deltoid (P< or =.05) and biceps (P<.05) scores, the FMA for the proximal upper arm (P<.05), the FIM instrument (P<.05), and the FIM motor score (P<.01); these gains were also sustained at the 3- and 8-month follow-up. The FMA and MRC wrist flexor test findings did not differ statistically either at the end of training or at the follow-up sessions. We found no significant differences in MAS and TCT in either group in any of the evaluations. No adverse effects occurred and the robotic approach was very well accepted. CONCLUSIONS: Patients who received robotic therapy in addition to conventional therapy showed greater reductions in motor impairment and improvements in functional abilities. Robotic therapy may therefore effectively complement standard rehabilitation from the start, by providing therapeutic support for patients with poststroke plegic and paretic upper limb.

A novel robot device in rehabilitation of post-stroke hemiplegic upper limbs.

BACKGROUND AND AIMS: In this pilot study, we introduce the "NeReBot", a novel robotic device designed and programmed for clinical neurological applications. The aim of the study was to test whether additional sensorimotor training of paralyzed or paretic upper limbs, delivered by NeReBot, enhanced motor and functional outcome in stroke patients. METHODS: Twenty patients with post-stroke hemiparesis or hemiplegia received standard poststroke multidisciplinary rehabilitation, and were randomly assigned either to exposure to the robotic device without training or to additional sensorimotor robotic training (about 4 h/week) for 4 weeks. Robot training consisted of peripheral manipulation of the shoulder and elbow of the impaired limb, correlated with visual stimuli. RESULTS: At hospital discharge, impairment and disability had declined in all patients, but the group with robot training showed higher gains on motor impairment and functional recovery, which were maintained at the 3-month follow-up. No adverse events resulted from robot-assisted therapy. CONCLUSIONS: According to our results, NeReBot therapy may efficaciously complement standard post-stroke multidisciplinary rehabilitation and offer novel therapeutic strategies for neurological rehabilitation.

Robot-aided intensive training in post-stroke recovery.

The successful motor rehabilitation of stroke patients requires an intensive and task-specific therapy approach. The plasticity of the adult human brain provides opportunities to enhance traditional rehabilitation programs for these individuals. Intensive robot-aided sensorimotor training may have a positive effect on reducing impairment and disability and increasing reorganization of the adult brain. This approach may therefore efficaciously complement standard post-stroke multidisciplinary programs as shown by recent experimental trials.

Facioscapulohumeral muscular dystrophy and occurrence of heart arrhythmia.

BACKGROUND: Subjects with facioscapulohumeral muscular dystrophy (FSHD) do not generally suffer from significant cardiac symptoms. Although with heterogeneous results, studies reported to date indicate that heart alterations unrelated to cardiomyopathy are possible in FSHD. PATIENTS AND METHODS: We describe the findings of a multicenter investigation aimed at detecting cardiac abnormalities in 83 FSHD patients, 44 males and 39 females with a mean age of 47 years. All patients underwent clinical heart examination, 12-lead electrocardiography and 24-hour Holter monitoring; echocardiography was also performed on most patients. RESULTS: Among the 83 patients, 62 with no cardiovascular risk factors were identified. Ten of them manifested clinical or subclinical cardiac involvement: 5 reported symptoms represented mostly by frequent palpitations secondary to supraventricular arrhythmia and another 5 exhibited electrocardiographic signs of short runs of supraventricular paroxysmal tachycardia. In the absence of cardiovascular risk factors, we found symptoms or signs of heart involvement of mainly arrhythmic origin in 10 of our 83 FSHD patients (12%). CONCLUSIONS: Considering our data and those available in the literature as a whole, arrhythmic alterations seem to be detected more frequently than expected in FSHD patients.