Mario Armani

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).

Sensory, motor, and autonomic neuropathy in patients with multiple symmetric lipomatosis

Clinical evaluation of 33 male patients affected by multiple symmetric lipomatosis has revealed a previously unreported high prevalence of somatic and autonomic neuropathies. In 84% of the patients, clinical examination revealed signs or symptoms of neural disturbances, ranging from a vibratory sensory loss to severely incapacitating trophic ulcers or Charcots arthropathy. Electrodiagnostic investigations demonstrated a significant reduction of motor and sensory conduction velocity in the peroneal and sural nerves. Morphometric studies of nerve and muscle biopsies from five patients with multiple symmetric lipomatosis revealed a significant reduction in myelinated fiber density (4435 +/- 593 fibers/mm2 in MSL vs 7660 +/- 800 in controls; p less than 0.05), a selective reduction in the large fibers of 7 to 10 micron in diameter, and signs of chronic denervation-reinnervation processes. Bedside tests for autonomic neuropathy were abnormal in 15 of 20 patients studied. Metabolic studies in these patients confirmed a significant increase in plasma high-density lipoprotein fractions consistent with the diagnosis of hyperalphalipoproteinemia, and a significant reduction in plasma low-density lipoprotein fractions (hypobetalipoproteinemia) associated with a marked enhancement of lipoprotein lipase activity in adipose tissue. Thus, a metabolic factor has to be considered in the pathogenesis of MSL neuropathy.

Carnitine palmityl transferase deficiency Clinical variability, carrier detection, and autosomal‐recessive inheritance

A 21-year-old man had recurrent myoglobinuria; his 28-year-old sister had symptoms of fatigability. During prolonged fasting, serum free fatty acid rose in both siblings, but only the sister produced ketone bodies and had elevated creatine phosphokinase activity. Carnitine palmityl transferase (CPT) activity was less than 30% of normal in muscle and platelets. Liver biopsy disclosed a low level of the enzyme in the brother. The parents had intermediate levels of the enzyme in platelets. CPT deficiency seems to have an autosomal-recessive pattern of inheritance and a variable phenotypic expression.

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.

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.

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.

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 post-stroke 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.

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.

Denture cream: An unusual source of excess zinc, leading to hypocupremia and neurologic disease

We read with interest the article by Nations et al.1 describing four additional cases of copper deficiency myeloneuropathy in association with hyperzincemia and chronic use of denture creams containing zinc. We previously reported a patient with copper deficiency myeloneuropathy and zinc excess associated with over 15 years using a similar denture cream.2 The daily amount of zinc oxide applied with the adhesive was about 150 mg. Even though there are no data on zinc bioavailability from this source, we agree with Nations et al. that these findings prompt important safety concerns on the chronic use of denture creams …

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 Wegeners granulomatosis. Immunosuppressive therapy achieved complete clinical resolution and cleared the pulmonary lesions. To the best of our knowledge this is the first report of Wegeners granulomatosis presenting with neurogenic dysphagia due to lower cranial nerve palsy.