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PubMed2006

Topiramate: a new agent for patients with alternating hemiplegia of childhood.

Alternating hemiplegia of childhood is a rare syndrome characterized by the onset, before 18 months of age, of frequent attacks of alternating paralysis. Here we report the efficacy of topiramate in four patients with alternating hemiplegia of childhood (AHC) that did not respond to flunarizine, as well as in two newly diagnosed patients. Following treatment with topiramate, the frequency and duration of hemiplegic attacks significantly improved in all patients. Additional symptoms such as seizures, migraine, involuntary movements, autonomic symptoms, and impaired mental development also improved. Topiramate is worth trying when treating patients with AHC as a first trial, or a substitute for flunarizine once the latter agent loses effect.

Anticonvulsants↗
PubMed1999

Congenital hemiplegia in children at school age: assessment of hand function in the non-hemiplegic hand and correlation with MRI.

The aim of this study was to evaluate whether children with congenital hemiplegia show abnormal hand function on the non-hemiplegic side and whether this, if present, can be related to the type and extent of brain lesions on MRI. Twenty-two children with congenital hemiplegia of age ranging between 4.8 and 12.3 years, were assessed with a clinical and MRI assessment. Clinical assessment included a structured neurological examination, assessment of hand grips and the Movement Assessment Battery for Children which also includes one item assessing speed and accuracy in each hand. The results showed that 64% of the children studied showed some degree of functional impairment of the non-hemiplegic hand. Manual dexterity 1 from the Movement ABC was, in our experience, a more sensitive tool to detect minor functional abnormalities than the evaluation of hand grips. The severity of the impairment on the non-hemiplegic side was not significantly related to the severity of impairment in the hemiplegic hand (p > 0.05). In contrast, a significant association was found with the site of lesions as hand function in the non-hemiplegic hand was always normal in children with unilateral lesion and abnormal in the ones with bilateral parenchymal lesions (p < 0.05). Children with predominantly unilateral lesions but with bilateral ventricular dilatation or periventricular changes showed more variable results.

Brain↗
PubMed1996

Regional cerebral blood perfusion in children with hemiplegia: A SPECT study.

Fourteen children with spastic hemiplegia of various etiology: cerebral palsy (seven cases, five with porencephalic cyst); stroke with small deep infarcts (two); hemiconvulsion-hemiplegia-epilepsy syndrome (three); traumatic brain injury (two), were investigated by 99mTc-HMPAO SPECT. Localized and remote perfusion abnormalities were studied. Hypoperfusion corresponding to CT abnormalities was found in each group, but the perfusion deficit extended beyond the boundaries of anatomical defects, most prominently in cases with widespread unilateral epileptic discharges. Ipsilateral cerebellar diaschisis was observed in patients with early cerebral insult (who had porencephalic cyst of pre- or perinatal onset) and crossed cerebellar diaschisis was noted in a patient who sustained traumatic brain injury at a later age. Diaschisis in the overlying cortex, thalamus and basal ganglia was noticed in several cases. Although clinical symptoms or signs could not be unequivocally attributed either to the size of the perfusion defects beyond the boundaries of lesions shown by CT or to the diaschisis, the findings may contribute to reveal age-related abnormal perfusion patterns.

Age of Onset↗
PubMedDate not supplied

[Accompanying symptoms in infantile spastic hemiplegia].

Various concomitant disorders in 535 children with infantile hemiplegia were evaluated; 159 of the cases were particularly well documented. The right/left distribution of the hemiplegia was 56 to 44, the ratio of boys to girls 59 to 41. Severe impairments of hand function (lack of function, first grip) were found more frequently in right hemiplegics than left; the ratio of severe to slight disorders was approx. 3 to 1 in right hemiplegics and 3 to 2 in left hemiplegics. Impairment of hand function was closely related to the quality of sensitivity, the tests of this being based on two-point discrimination and stereognosis. Sixty-two percent of the children were of normal intelligence; of the remainder, approximately equal-sized groups suffered from impairment of the learning faculty or were mentally retarded. There was a positive correlation between reduced intelligence and the severity of impairment of hand function. Impaired speech development was found in 7 percent of the children, right and left hemiplegics being equally affected. Thus, there was no evidence that the brain lesion had any special influence on development of speech. Observations of growth in the legs revealed average differences of length of 2 mm and up to 3.5 cm in isolated cases. After eight year of life there was practically no further increase in the differences in length.

Adolescent↗
PubMed1994

Prediction of outcome in children with congenital hemiplegia: a magnetic resonance imaging study.

The degree of Wallerian degeneration (WD) in the corticospinal tracts seen with magnetic resonance imaging (MRI) was correlated with the distribution and severity of congenital hemiplegia in 20 children aged nine months to nine years. All the children had hemispheric lesions diagnosed with ultrasound in the neonatal period: MRI and clinical assessment were performed from nine months to nine years of age. Hemiplegia was graded as mild, moderate or severe and into predominantly upper or lower limb distribution. WD was assessed by the presence or absence of signal intensity changes in the internal capsule on inversion recovery and spin echo sequences and by the asymmetry of the upper brainstem. The degree of asymmetry was estimated by measuring the cross sectional area (CSA) of the brainstem at three levels and calculating the ratio of the measurements between the side of the lesion and the unaffected side. Infarct size was estimated from the CSA of the infarct at the maximum site of the lesion. Both measurements were correlated with the severity of outcome and the site of involvement. There was a better correlation between severity of outcome and brainstem asymmetry (p = 0.003) than severity of outcome and infarct size (p = 0.02). There was also a significant correlation between upper limb involvement and brainstem asymmetry (p = 0.01). As WD estimated by brainstem asymmetry appears early and is easy to measure, it may be a good marker to estimate later impairment in infants with predominantly unilateral hemispheric haemorrhagic/ischaemic lesions diagnosed in the neonatal period.

Brain Stem↗
PubMed2003

The development of a knee locker with closed-loop functional electrical stimulation (FES) for hemiplegia in gait training.

PURPOSE: A knee locker with closed-loop functional electrical stimulation (FES) system has been developed to prevent the quadriceps weakness and the drop-foot of the hemiplegia during gait training. METHOD: The FES system is triggered by a footswitch on the heel of the disabled foot to stimulate the tibialis anterior muscle for dorsi-flexion and to turn-off the knee locker in the swing phase through the main controller. Besides, the footswitch on the heel of the affected-side can be used to stimulate the quadriceps and to turn-on the knee locker for quadriceps weakness in the stance phase. RESULTS: It is revealed that the mean velocity, cadence, stride length, active ankle motion range, and functional ambulation category (FAC) have improved significantly from 0.15 +/- 0.04 m sec( - 1), 43.3 +/- 15.4 steps min( - 1), 0.36 +/- 0.11 m, 15 degrees, level 2 to 0.43 +/- 0.21 m sec( - 1), 69.4 +/- 19.1 steps min( - 1), 0.73 +/- 0.17 m, 40 degrees, level 4 respectively for the patient. A paired t-test indicated that differences in the electromyography (EMG) of the tibialis anterior and the quadriceps muscles between patient's disabled (affected-side) foot and normal (unaffected-side) foot are not significant (p > 0.05) after 16 weeks of training. CONCLUSIONS: It is concluded that this new knee locker with closed-loop FES system is capable of providing the hemiplegia with restoration to regular walking after appropriate gait training.

Electric Stimulation Therapy↗
PubMed2003

Shoulder pain in hemiplegia revisited: contribution of functional electrical stimulation and other therapies.

OBJECTIVES: Post-stroke shoulder pain is probably the most frequent complication in hemiplegia and has repercussions on motor rehabilitation and the psychological equilibrium of the patient. The strategies for prevention and treatment are presented. AETIOLOGY: Among the various factors contributing to the occurrence of shoulder pain in hemiplegia, some are related to the joint, such as lesion of the rotator cuff tendons, reflex sympathetic dystrophy, inferior-anterior subluxation of the head of the humerus, whereas others are related to the neurologic lesion such as central post-stroke pain, lack of sensibility, unilateral neglect and spasticity. PREVENTION: Efforts should be made from the start to keep the shoulder in an ideal position at all times and movement of the shoulder and upper limb should be carried out with care. TREATMENT: Will be aimed to the cause of pain and passive or active range of motion exercises will be encouraged. Physical, medical and surgical treatments have improved over the last few decades. Functional electrical stimulation in patients with shoulder pain and subluxation, applied early after onset of the stroke, has shown beneficial positive effects on subluxation, pain and mobility. Efforts should therefore be made to better understand the post-stroke shoulder pain in order to provide better outcomes of rehabilitation and thus improve quality of life for patients.

Electric Stimulation Therapy↗
PubMed2006

Relationships between independence level of single motor-FIM items and FIM-motor scores in patients with hemiplegia after stroke: an ordinal logistic modelling study.

OBJECTIVE: To assess the relationships between independence levels of single motor-related Functional Independence Measure (FIM) items and summed FIM-motor scores of patients with hemiplegia after stroke. DESIGN: For each patient FIM scores were assessed 4 times during hospitalization. Ordinal logistic analyses were performed on group data. SUBJECTS: Fifty patients with hemiplegia after stroke staying in a long-term rehabilitation facility. RESULTS: Analyses revealed that FIM-motor scores accounted for much of the variability of independence levels for most of the single FIM items, including dressing upper body, and transfers to bed/chair/wheelchair and to toilet. For these items, the independence levels were proportionally associated with FIM-motor scores. For eating, higher FIM-motor scores (>60) were associated with modified independence and lower FIM-motor scores (<40) correlated with attainment of supervision/set-up levels. For dressing lower body, greater independence was apparent when FIM-motor scores were higher (>60). CONCLUSION: For single FIM items, relative difficulty was comparable with results from previous literatures using Rasch analyses. Moreover, our results revealed that relative difficulty for single items varied greatly between independence levels. With regard to disability task targets, probability of independence evaluated from logistic modelling is an aid to efficient rehabilitation scheduling.

Activities of Daily Living↗
PubMed1998

Relationships between timing of muscle excitation and impaired motor performance during cyclical lower extremity movement in post-stroke hemiplegia.

We used an ergometer pedalling paradigm to relate abnormalities in the timing of muscle excitation to the impaired ability to perform mechanical work in the plegic lower limbs of persons with hemiplegia. The EMGs of seven leg muscles and pedal forces were measured bilaterally during pedalling for 15 persons with hemiplegia and 12 neurologically intact age-matched control subjects. Subjects were asked to pedal at a moderate workload (135 J) and cadence (40 r.p.m.). While intersubject variability was high, the external mechanical work output of the plegic leg was significantly less (from 79.6% to -28.9% of the work produced by average leg of control subjects) as a result of less positive work and more negative work being done. The timing of EMG in individual plegic limb muscles exhibited two distinct types of abnormalities that were significantly correlated with this lesser work production: prolonged excitation in the vastus medialis and phase-advanced excitation (both early initiation and early termination) in the rectus femoris and semimembranosus. These results suggest that muscles were differently affected depending on their function, external power-producing muscles (e.g. vastus medialis) showing prolonged excitation and muscles that normally maintain crank progression during limb transitions (e.g. semimembranosus) showing phase-advanced excitation.

Aged↗
PubMed1988

Modification of sitting posture of patients with hemiplegia using seat boards and backboards.

The purpose of this study was to evaluate and compare the effects of using seat boards only and a combination of seat boards and backboards on wheelchair posture. Forty-one patients with hemiplegia secondary to cerebrovascular accidents were assigned sequentially to 1) a group that used seat boards only (SB Group), 2) a group that used both seat boards and backboards (SBB Group), or 3) a group that used no boards (Control Group). The SB and SBB Groups used the boards throughout rehabilitation. Wheelchair sitting posture was measured at entry to and discharge from the rehabilitation program. The SB and SBB Groups were measured with and without boards. Anterior-posterior pelvic tilt, lumbar lordosis, thoracic kyphosis, lateral flexion, and lateral pelvic tilt were measured using a gravity goniometer, photographs, and calipers. Use of seat boards was associated with a decreased lateral pelvic tilt of 2.3 degrees at discharge while the seat boards were in place. The use of seat boards and backboards combined was associated with decreased lateral pelvic tilt of 3.1 and 1.6 degrees, increased anterior pelvic tilt of 13.1 and 11.1 degrees, and decreased thoracic kyphosis of 13.0 and 14.2 degrees at entry to and at discharge from the rehabilitation program, respectively, while the boards were in place. At the end of rehabilitation when the boards were removed, no long-term postural changes had occurred in the treatment groups compared with the Control Group. This study demonstrated that seat boards and backboards in wheelchairs improve certain postural deviations of patients with hemiplegia, but that these improvements are not maintained when the boards are removed after 5 to 10 weeks of use.

Analysis of Variance↗
PubMed1990

Relationship of sensory organization to balance function in patients with hemiplegia.

Standing balance and dynamic weight shifting were evaluated in 10 subjects with hemiplegia using a sensory organization balance test (SOT) and the Fugl-Meyer sensorimotor assessment (FMSA). The SOT is a timed balance test that evaluates somatosensory, visual, and vestibular function for maintenance of upright posture. In contrast, the FMSA is a functional status assessment that indicates the amount of assistance needed during various balance tasks and the tolerated duration of the task. The results of both clinical tests were compared to determine whether the SOT correlated with functional ability. The SOT scores were significantly correlated with the FMSA balance subscores and the FMSA total lower extremity scores. Based on the results of this preliminary study, the authors concluded that the SOT may be useful for evaluating a patient's functional status. Further implications for the evaluation and treatment of balance dysfunction in persons with hemiplegia are discussed.

Adult↗
PubMed2002

Ocular motor features of alternating hemiplegia of childhood.

A 14-month-old boy with alternating hemiplegia of childhood, an idiopathic disorder of early childhood causing episodic hemibody tonic spasms and hemiplegia, showed repetitive jerks of abduction of the ipsilateral eye during the spells. The mechanism of this ocular motor abnormality is unknown but may be unique to this disorder.

Anticonvulsants↗
PubMed2004

Shoulder pain in hemiplegia: results from a national rehabilitation hospital in Turkey.

OBJECTIVE: Shoulder pain is a common complication after stroke that can limit the patients' ability to reach their maximum functional potential and impede rehabilitation. The aim of our study was to examine the occurrence of hemiplegic shoulder pain in a group of Turkish patients and clarify contributing factors such as glenohumeral subluxation, reflex sympathetic dystrophy, tonus changes, motor functional level, limitation in shoulder range of motion, thalamic pain, neglect, and time since onset of hemiplegia. The effect of shoulder pain on the duration of rehabilitation stay was also identified. DESIGN: A total of 85 consecutive patients with hemiplegia admitted to a national rehabilitation center were evaluated for the presence of shoulder pain. A brief history of pain was taken for each patient, and each patient was evaluated by radiographic and ultrasonographic examination. The subjects with shoulder pain were compared with those without pain in regard to certain of the above variables. RESULTS: Of the 85 patients with stroke, 54 patients (54/85, 63.5%) were found to have shoulder pain. Shoulder pain was significantly more frequent in subjects with reflex sympathetic dystrophy, lower motor functional level of shoulder and hand (P < 0001), subluxation, and limitation of external rotation and flexion of shoulder (P < 0,05). Age was also a significant factor in the development of shoulder pain. We were unable to demonstrate a significant relationship between shoulder pain and sex, time since onset of disease, hemiplegic side, pathogenesis, spasticity, neglect, and thalamic pain. There was no prolongation of rehabilitation stay in patients with shoulder pain. CONCLUSION: These results indicate that shoulder pain is a frequent complication after stroke and that it may develop from a variety of factors. To prevent and alleviate shoulder pain, efforts should be directed toward proper positioning of the shoulder, range of motion activities, and the avoidance of immobilization.

Adult↗
PubMed1991

Epilepsy associated with hemiplegia: 1. Seizure manifestations.

1. The seizure manifestations of patients with hemiplegia were reminiscent of partial seizures. The motor manifestations were notably asymmetrical and impairment of consciousness was mild. Nevertheless, tonic seizures, identified by electroclinical correlation were found in 49 patients (88%) although they were predominantly unilateral or asymmetrical. 2. Unclassified seizures characterized by ictal automatisms, which mimic complex partial seizures was observed in 5 patients (9%). 3. Late epileptic seizures of the HHE syndrome were regarded as psychomotor seizures by Gastaut et al. In the present study, however, complex partial seizures were found in only 3 at 28 patients with HHE syndrome (11%) whereas tonic seizures in 23 of these patients (82%). One possible reason for this discrepancy is bias in the selected patient population, because those with transient hemiplegia were not included, and infrequently occurring partial seizures might have been missed. Further study is therefore needed to determine whether the HHE syndrome is associated with complex partial seizures or predominantly unilateral tonic seizures.

Cerebral Cortex↗
PubMed1984

Acute hemiplegia in Kawasaki disease and infantile polyarteritis nodosa.

Although aseptic meningitis, lethargy and irritability occur frequently in Kawasaki disease and infantile polyarteritis nodosa, other neurological manifestations are rare. The authors report one case of Kawasaki disease and one of infantile polyarteritis nodosa, both associated with acute hemiplegia. Both patients had received courses of oral corticosteroids for their underlying disease prior to the onset of the hemiplegia. Pathological studies, as well as the four previously reported cases, are reviewed.

Cerebral Infarction↗
PubMed1993

Single-photon emission computed tomography investigations of alternating hemiplegia of childhood.

Alterations in regional cerebral blood-flow, as determined by single-photon emission computed tomography (SPECT) using technetium [99mTc] hexamethyl propylenamine oxime, were studied in two children presenting with alternating hemiplegia of childhood. Both experienced hemiplegic episodes several times per month, despite marked improvement on flunarizine therapy. SPECT images of both patients revealed focal areas of decreased uptake of the radiotracer, representing impaired regional cerebral blood-flow during, as well as between, seizures. The interictal finding of localized areas of reduced tracer uptake suggest that long-lasting hypoperfusion could be the pathophysiological mechanism by which the slowly resolving hemiplegia, and ultimately the permanent multifocal neurological deficits, are produced.

Brain↗
PubMed1995

Periventricular haemosiderin deposition in patients with congenital hemiplegia.

Two patients with congenital hemiplegia without obvious prenatal, perinatal or neonatal difficulties showed linear low signal intensity lesions along the wall of the dilated lateral ventricles without any parenchymal lesions on T2- and proton density weighted MRI. Haemosiderin deposition secondary to intra-uterine subependymal haemorrhage with intraventricular haemorrhage was considered most likely from the signal intensity, distribution and clinical histories. MRI, which is the only means of detecting haemosiderin deposition, could be beneficial for evaluating the pathogenetic cause of congenital hemiplegia.

Cerebral Ventricles↗