Italian standardization of the Apples Cancellation Test.

Hemispatial neglect due to right parieto-temporo-frontal lesions has a negative impact on the success of rehabilitation, resulting in poor functional gain. Recent research has shown that different types of neglect can impact in a different way on rehabilitation outcomes. The availability of a sensitive test, useful for distinguishing egocentric and allocentric forms of neglect, may be clinically important as all current clinical instruments fail to distinguish between these forms of disturbance, yet they differentially predict outcome. The Apples Test is a new instrument useful to evaluate both egocentric and allocentric forms of neglect. In order to establish Italian norms for this diagnostic instrument the test was administered to a sample of 412 healthy people of both genders (201 M and 211 F), aged from 20 to 80 years enrolled from 14 different rehabilitation centers in Italy. Based on the data, we established pathological performance cut-offs for the accuracy score (total omission errors), the asymmetry score for egocentric neglect (omission error difference), the asymmetry score for allocentric neglect (commission error difference) and execution time. The usefulness of the Apples Test for diagnostic purposes is illustrated by presenting three patients with different forms of neglect (egocentric, allocentric and mixed neglect).

Peripheral nerve damage during limb lengthening. Neurophysiology in five cases of bilateral tibial lengthening.

Limb lengthening is used to correct leg length discrepancy and to increase stature. The reported frequency of peripheral nerve complications varies from 5% to 30%, but is probably underestimated. Damage may be direct or be caused by overstretching of the nerves. We have used electrophysiological tests to evaluate five patients during bilateral tibial lengthening by the Ilizarov method. Results after 24 to 107 days of lengthening showed electromyographic evidence of partial muscle denervation in all 10 limbs, with reduced motor conduction velocities in two tibial nerves and three common peroneal nerves. The sensory conduction velocity in the sural nerve was always unchanged. A clear relationship was shown between the amount of tibial lengthening and the degree of electrophysiological abnormality. Our results suggest that subclinical nerve damage is a very frequent complication of tibial lengthening.

Repeatability of surface EMG variables during voluntary isometric contractions of the biceps brachii muscle.

The repeatability of initial value and rate of change of mean spectral frequency (MNF), average rectified values (ARV) and muscle fiber conduction velocity (CV) was investigated in the dominant biceps brachii of ten normal subjects during sustained isometric voluntary contractions. Four levels of contraction were studied: 10%, 30%, 50% and 70% of the maximal voluntary contraction level (MVC). Each contraction was repeated three times in each of three different days for a total of nine contractions/level/subject and 90 contractions per level across the ten subjects. Repeatability was investigated using the Intraclass Correlation Coefficient (ICC) and the standard error of the mean (SEM) of the estimates for each subject. Contrary to observations in other muscles, CV estimates appeared to be very repeatable both within and between subjects. CV showed a small but significant increase when contraction force increased from 10% to 50% MVC but no change for further increase of force. As force increased, MNF showed a slight decrease possibly related to a wider spreading of the CV values. The rate of time decrement of MNF and CV increased with the level of contraction. The normalized decrement (% of initial value per second) was in general higher for MNF than for CV and was more repeatable between subjects at 10% MVC than at 70% MVC. A final observation is that a resting time of 5 minutes may not be sufficient after a contraction at 50% or 70% MVC.

A protocol for the assessment of 3D movements of the head in persons with cervical dystonia.

OBJECTIVE: To design and test a protocol for the assessment of neck movements in patients affected by cervical dystonia by using an electromagnetic system. This approach could overcome the limits of the current assessment scales in this specific field. BACKGROUND: Initial assessment and function recovery during treatments are diagnosed by the clinician using outcome scales which present many drawbacks in terms of easiness of use, sensitivity, and reliability. DESIGN: A three-dimensional motion analysis system was used to record six different head movements. METHODS: Six able-bodied subjects and 10 subjects affected by cervical dystonia participated in this study. For the different head movements three kinematic parameters (a symmetry index and two indexes related to the reduction of the range of motion) have been extracted in order to compare the performance of able-bodied and disabled persons. RESULTS: The features selected allowed highlighting of the differences between able-bodied and disabled subjects for the degrees of freedom of the neck. CONCLUSIONS: Using a motion analysis system, three kinematic features were extracted from head movements. They seem to allow a more objective assessment of the disability and a more appropriated strategy for the management of patients affected by cervical dystonia.

Evaluation of leg joint trajectories while carrying out passive manipulation by NEUROBike.

During the last decades, many robotic platforms aimed at post-stroke neurorehabilitation of locomotion have been developed. These devices have been designed to enhance the possibilities of conventional rehabilitation providing safe, highly accurate, intensive and prolonged treatments. Nevertheless, up to now, robotic aided therapy has not yet promoted improvements of the motor performance significantly greater than those achieved by the conventional therapy. According to previous studies, we believe that this result may be partially ascribed to two main issues: the rehabilitation mediated by robots is usually provided too late from the trauma and it mainly consists of passive and cyclic manipulation of the legs. Our proposal to overcome some of the supposed limits is NEUROBike, an operative mechatronic platform able to lead leg manipulation as soon as possible after the trauma, that is when patients still lie on their own beds. Moreover, NEUROBike has been designed to provide both passive and cyclic manipulation of leg joints with trajectories similar to those related to natural walking, and motor task involving random efforts. This work presents the comparison between desired and measured leg joint trajectories while NEUROBike provides cyclic and passive leg manipulation. The results show that angular excursions at proximal joints were reasonably comparable with those obtained by the velocity based model even though they were affected by a positive offset involving emphasized flexion of hip and knee during the gait cycle.

Prognostic value of standard EEG in traumatic and non-traumatic disorders of consciousness following coma.

OBJECTIVE: To investigate the prognostic value of standard electroencephalogram (EEG) in predicting the improvement of the level of consciousness in patients suffering from severe disturbances of consciousness following coma caused by acute brain injuries. METHODS: A standard EEG was recorded at admission in our rehabilitation department in a total of 46 patients with impaired consciousness states following coma (22 patients with traumatic brain injuries, 24 patients with non-traumatic brain injuries). We quantified the EEG abnormalities using the scale of Synek (1988) and correlated them with the basal level of cognitive functioning (LCF) scale score and with its variation after three months. RESULTS: EEG scores correlated with LCF scores at admission (p<0.01) and with LCF scores' variation after three months (p<0.01) in patients with traumatic brain injury; EEG scores correlated only with LCF scores variation after three months (p<0.01) in patients with non-traumatic brain injury. CONCLUSIONS: Standard EEG, analysed using the Synek scale, has a good prognostic value in both groups of patients with disorders of consciousness. SIGNIFICANCE: This work may have implications for clinical care, rehabilitative programs and medical-legal decisions in patients with impaired consciousness states following coma due to acute brain injuries.

Transient palsy of hip abductors after a fall on the buttocks.

A fall on the buttocks caused monolateral transient palsy of the hip abductors in two patients. Palsy could be ascribed to acute entrapment of the superior gluteal nerve between the piriformis muscle and the incisura ischiadica major.

Amelioration of nerve conduction velocity following simultaneous kidney/pancreas transplantation is due to the glycaemic control provided by the pancreas.

Diabetic polyneuropathy is a common, disabling chronic complication of diabetes mellitus. Previous studies have suggested that combined pancreas-kidney transplantation can ameliorate nerve conduction. The relative contribution of the correction of hyperglycaemia and uraemia on nerve function is still a matter of debate. Nerve conduction velocity (NCV) was assessed before and after simultaneous pancreas and kidney transplantation, and before and after pancreas graft failure in five insulin-dependent diabetic (IDDM) patients affected by severe diabetic polyneuropathy. Sensory and motor NCV were recorded in five nerves and expressed as a cumulative index for each patient. Metabolic control was evaluated by fasting blood glucose and glycosylated haemoglobin levels. NCV index was below normal values before transplant: -3.8 +/- 0.7 (normal value: 0.89), improved 1 and 2 years after transplant: -3.1 +/- 1.3 and -2.6 +/- 0.9 (p = 0.0019), stabilised until pancreas failure and deteriorated to pre-transplant values 2 years after pancreas graft failure: -3.6 +/- 1.0 (p = 0.034). Fasting blood glucose levels worsened after pancreas graft failure. HbA1c levels, in the normal range during functioning pancreas graft (6.6 +/- 0.6%), deteriorated after its failure (8.0 +/- 0.6%, p = 0.04). Kidney function was preserved. These data support a positive effect of pancreas transplantation per se on NCV in IDDM subjects with diabetic polyneuropathy, thus demonstrating that metabolic control provided by a self-regulated source of insulin not only halts but also ameliorates nerve function, even if polyneuropathy is advanced.

Intravenous immunoglobulin treatment in multifocal motor neuropathy and other chronic immune-mediated neuropathies.

This review deals with the use of intravenous IVIg immunoglobulins in the treatment of chronic immune-mediated neuropathies: multifocal motor neuropathy, chronic inflammatory demyelinating polyneuropathy, neuropathies associated with monoclonal gammopathies. A particular attention is given to case series and trials which compare IVIg to other therapies, such as steroid treatment immunosuppressors and plasma exchange. At present clinical and instrumental data seem to indicate the short term efficacy of IVIg in multifocal motor neuropathies, especially as early treatment; further studies are need in order to prove its long term efficacy in this disease. Concerning chronic inflammatory demyelinating polyneuropathies, short term IVIg efficacy is comparable to that of plasma exchange and in the long term most patients need repeated treatments. Most patients respond to the initial therapy and the initial nonresponders usually improve with a second treatment modality.

Cognitive deficits and their relationship to other neurological complications in chronic alcoholic patients.

In randomly selected chronic alcoholics hospitalised for the first time for detoxification a high prevalence (68%) of cognitive deficits was found. Peripheral neuronal damage was seen in 74%, autonomic neuronal damage in 24%. Cognitive deficits were not correlated with age, daily ethanol intake, duration of alcohol abuse or severity of liver damage. There was no correlation of peripheral, autonomic and central nervous system damage. Alcohol-induced damage of the nervous system is a common complication of chronic alcoholism, whose clinical importance often obscures possible concomitant liver damage.

Clinical and neurophysiological assessment of immunoglobulin therapy in five patients with multifocal motor neuropathy.

High dose intravenous immunoglobulin (IVIg) is an effective treatment for demyelinating neuropathies. IVIg was given to five patients with multifocal motor neuropathy, a motor neuropathy showing a clinical syndrome of asymmetrical weakness and amyotrophy, electrophysiological evidence of motor conduction block and, in many cases, high titres of serum anti-GM1 antibodies. Muscle strength was evaluated by a conventional score before and after each IVIg course. In all patients there was relevant improvement on muscle strength after each immunoglobulin course, but in most cases the clinical benefits partially declined after three to eight weeks. At the eight month follow up, however, the pretreatment examination showed a significant improvement compared with the initial evaluation. The effects of each IVIg course were still present after a number of courses. Electrophysiological study revealed a decrease in conduction block in one or more nerves in all patients. However, conduction block was unchanged or increased in other sites. IVIg treatment did not affect anti-GM1 antibody titres.

Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating neuropathy not responsive to other treatments.

Nine patients with chronic inflammatory demyelinating poliradiculoneuropathy (CIDP) were treated with intravenous immunoglobulin. All patients had been previously treated with prednisone and/or plasma exchange without effect. Objective improvement in clinical condition occurred in six patients. One patient became refractory after two treatment courses, two patients had no response. The results indicate that intravenous immunoglobulin has beneficial effects in a high percentage of patients with CIDP who are unresponsive to other treatments.

Sporadic failure of botulinum toxin treatment in usually responsive patients with adductor spasmodic dysphonia.

Botulinum toxin (BT) injections into vocalis (thyroarytenoid) muscle is currently considered the first-choice treatment for adductor spasmodic dysphonia, producing improvement for an average period of 3 months. In our experience, sporadic failure of BT efficacy can occur even in patients usually responsive to this therapy. The reasons for these episodes have not been clarified. In a retrospective, open trial, we investigated the effect toxin preparation (Botox or Dysport) and injection monitoring (electromyography or laryngoscopy) on the success rate of BT treatment. We studied 15 patients with adductor dysphonia usually responsive to BT therapy. BT was administered into the vocalis muscle in 112 and 36 injections under electromyographic or laryngoscopic guidance, respectively. Botox and Dysport were used in 106 and 42 sessions, respectively. In 29% of all injections, no subjective or objective changes, nor side effects were observed. Failure rate did not differ using electromyographic (28.6%) or laryngoscopic (30.5%) guidance. Failure rates with Botox and Dysport were 30.2% and 26.2%, respectively, but this difference was not statistically significant. These data suggest that treatment failure may occur regardless of the method of injection and of the drug preparation used, possibly due to mislocalisation of vocal folds.

Presence of carpal tunnel syndrome in diabetics: effect of age, sex, diabetes duration and polyneuropathy.

Common thought is that diabetic neuropathy is a predisposing factor to entrapment syndromes. Carpal tunnel syndrome (CTS) is the most frequent entrapment neuropathy; females and old people are most frequently affected (Comi et al., 1978). Prevalence of CTS in diabetics and associated risk factors were studied in 401 patients (208 males and 193 females) with insulin-dependent and non-insulin-dependent diabetes using electrophysiological techniques. Median nerve sensory and motor conduction velocity, ulnar and peroneal nerve motor conduction velocity and sural nerve sensory conduction velocity were investigated in all patients. Diagnostic criteria for CTS were the presence of delayed median nerve sensory conduction velocity in the palm-wrist tract and of increased distal motor latency. Polyneuropathy was defined by slowing-down of conduction velocity in two or more nerves. Forty-five patients (11.2%), 36 females and 9 males, showed CTS. One-hundred-sixty-eight patients (41.8%), 74 females and 94 males, were suffering from peripheral neuropathy. The strongest risk factors for CTS, in order of importance, were: female sex, older age and presence of neuropathy. Polyneuropathy but not CTS was related to duration of diabetes.

Neurophysiological study of the effect of combined kidney and pancreas transplantation on diabetic neuropathy: a 2-year follow-up evaluation.

Previous study have reported a significant improvement of peripheral neuropathy following combined pancreas and kidney transplantation attributed to improvement of blood glucose control by some authors and to elimination of uraemia by others. To asses the specific role of uraemia and hyperglycaemia in neuropathy, 16 diabetic uraemic patients with combined pancreas and kidney transplantation were compared to 9 diabetic patients with a renal graft only. Neurophysiological studies of peripheral neuropathy included ulnar and deep peroneal nerve motor conduction velocity, median and sural nerve sensory conduction velocity were performed at baseline and 1 and 2 years after transplantation. One year after transplantation mean nerve conduction velocity significantly improved in both groups. However, changes were statistically significant in the kidney-pancreas group only. At the 2 year follow-up nerve conduction velocity had increased further in the pancreas-kidney group only. These data suggest that improvement of nerve conduction velocity following pancreas and kidney transplantation is predominantly due to the long-term euglycaemic state.

Polyneuropathy associated with IgA monoclonal gammopathy: a hypothesis of its pathogenesis.

We describe three patients with chronic progressive polyneuropathy associated with IgA monoclonal gammopathy. Two patients had a prominent sensory neuropathy and one had a prominent motor neuropathy. Sural nerve biopsies showed axonal degeneration in all cases. In immunocytochemical studies patients' IgG immunostained axons. By Western immunoblot a band of IgG reactivity with an axonal protein of 66 kDa was found. No band of IgA and IgM were found. We suggest the possibility that the IgA monoclonal protein may act as a stimulating factor of preexisting B cell clones eliciting an immune reaction against nerve antigens.

Evaluation of central nervous conduction by visual evoked potentials in insulin-dependent diabetic children. Metabolic and clinical correlations.

Peripheral neuropathy is a well-known complication of diabetes, but few data are available on central lesions. Visual evoked potentials (VEPs) seem a reliable and feasible technique for detecting a conduction delay in the central nervous system. Seventy-one insulin-dependent type 1 diabetic children (mean age 15 +/- 3 years) and 33 controls were investigated for central neuropathy. We used a pattern of reversal stimulation with television display of a checker board pattern (15 min and 30 min check size). The latencies of the positive peak (P100 wave) were significantly lengthened in 17 patients (27%) but no correlation was found between VEPs and age, duration of diabetes, insulin requirement and HbA1 level. A negative correlation was found between VEPs and peripheral nervous conduction velocity. VEPs measurement seems a simple and reliable technique for detecting early alterations in CNS function in diabetics. Our data suggest that central and peripheral nervous alterations progress simultaneously.

Polyneuropathy in hypothyroidism: clinical, electrophysiological and morphological findings in four cases.

Clinical, neurophysiological and morphological studies of four patients with polyneuropathy and secondary hypothyroidism are reported. Neurophysiological studies revealed signs of muscle denervation and reduction of conduction velocity in all the patients. Sural nerve biopsies showed axonal degeneration in all cases but one. All the patients were treated with replacement therapy and clinical symptomatology and neurophysiological parameters improved in all patients.