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Infantile hypotonia and paroxysmal dystonia: a variant of alternating hemiplegia of childhood?

We report 2 children with early onset of hypotonia and frequent episodes of paroxysmal dystonia. The episodes were abolished even by brief naps. One of the children developed alternating hemiplegia in the second decade. These children seem to have a variant of the now well-recognized syndrome of alternating hemiplegia of childhood. In that disorder, episodes of alternating hemiplegia develop before the age of 18 months. This syndrome must be considered in the differential diagnosis of paroxysmal dystonia in childhood.

Adolescent↗

Functional outcome of upper limb tendon transfers performed in children with spastic hemiplegia.

Children with spastic hemiplegia often present with upper limb muscle imbalance. The purpose of this paper was to determine whether reconstructive surgery improved their functional ability. 17 children under the age of 16 years with spastic hemiplegia underwent reconstruction that included tendon transfers, tendon lengthenings and thumb metacarpophalangeal fusion. They were assessed pre-operatively and at an average follow-up period of 2.6 years. Children's abilities were classified according to House's functional rating scale. Tendon transfers improved functional grading by two grades, from good passive assist to fair active assist. Improvement in the arc of wrist motion and forearm rotation was also seen. Parental satisfaction was high. Reconstructive surgery improved the functional abilities in this group of children with spastic hemiplegia.

Adolescent↗

Sensibility deficiencies in the hands of children with spastic hemiplegia.

We evaluated 40 children with spastic hemiplegia due to cerebral palsy for sensory function and relative limb size in the affected and unaffected upper extremities. Sensory function of each limb was evaluated with respect to stereognosis (12 objects), two-point discrimination, and proprioception. Four size measurements of each limb were made: arm and forearm circumference and forearm and forearm-hand length. This study showed that 97% of the spastic limbs had a stereognosis deficit, 90% had a two-point discrimination deficit, and 46% had a proprioception deficit. Thus sensory deficits are the rule rather than the exception in children with spastic hemiplegia. Those children with severe stereognosis deficits had significantly smaller limbs in all four measurement parameters than the children with mild or moderate stereognosis deficits. In the preoperative evaluation of children with spastic hemiplegia, severe size discrepancy is a physical examination tool that can be used as a predictor of severe sensory deficits. This information is helpful for the hand surgeon in establishing realistic surgical goals.

Anthropometry↗

Acute hemiplegia syndrome in childhood.

Three types of clinical features at the onset are well known to be characteristic of acute hemiplegia syndrome (AHS). Type 1 comprises status epilepticus of hemiconvulsions with fever. Representative diseases of this type are the infectious diseases of the central nervous system, acute encephalopathy and cerebral vascular diseases. Type 2 comprises status epilepticus of hemiconvulsions without fever. Cerebral vascular diseases and epilepsy are the major ones of this type. Type 3 comprises hemiplegia or hemiparesis of sudden onset without fever or convulsions. Most patients with this type had cerebral vascular diseases, about half of which were moyamoya disease in Japan. Recent progress in neuroimaging studies has allowed considerable elucidation of the etiology of AHS. Gadolinium-enhanced MRI showed minimal lesions such as capsular infarction more clearly than plain MRI. Acetazolamide test 99mTc-HMPAO SPECT imaging is one of the useful assisted diagnostic techniques for moyamoya disease, because it reveals the reserve capacity of the collaterals. [123I]IMP SPECT is useful for the diagnosis and follow-up of acute disseminated encephalomyelitis (ADEM), as the images of the lesions coincide well with the MRI ones. 99mTc-HMPAO SPECT in a case with alternating hemiplegia revealed normoperfusion in the ictal periods. Four cases of AHS are reported here.

Acute Disease↗

Acute hemiplegia associated with HIV infection.

An acute hemiplegia secondary to a large cerebral infarct is described in a 16-month-old infant with congenitally-acquired human immunodeficiency virus infection. Serial imaging studies during the next year documented improvement in his hemiplegia and a static underlying human immunodeficiency virus encephalopathy. Acquired immunodeficiency syndrome should be included in the differential diagnosis of children with acute hemiplegia.

AIDS Dementia Complex↗

Patient with unusual alternating hemiplegia.

A patient with unusual alternating hemiplegia in childhood is reported. The frequency of hemiplegic episodes was lower than that in other reported patients. Interictal positron emission tomography revealed decreased cerebral blood flow in the left hemisphere. After cerebral angiography, postanesthetic irritability occurred which evolved into coma with hemiplegic episodes. We believe that cerebral angiography in a child with alternating hemiplegia may cause hemiplegic episodes and coma. The episodes disappeared almost completely for 6 years after treatment with flunarizine, thus indicating the benefit of the medication for the treatment of alternating hemiplegia.

Basal Ganglia↗

[Factors predicting falls by patients with vascular hemiplegia at a physical medicine center].

OBJECTIVES: To determine factors predicting falls by patients with vascular hemiplegia to establish a program aimed at preventing falls in this population. METHODS: A comparative prospective study performed over 19 months with 44 patients older than 16 years who had had a cerebral vascular accident (CVA, stroke) and were consequently admitted to the Centre Richelie. The exclusion criteria were represented by CVA history, evidence of another form of encephalic lesion, and subsequent admission to hospital after hemiplegia or for follow-up. Assessment consisted of taking note of the mechanism of the fall, possible lesions, and number of falls and analyzing follow-up after the return home. Also included for all patients was information on 20 variables that could be risk factors for fall. RESULTS: The patients' average age was 60.43+/-13.43 years and 20 had had at least one fall (mean 2.2), which allowed us to determine a "falling" group and a control group. Statistical analysis revealed the following factors considered to predict falls: large amount of time prior to hospitalization and lengthy hospitalization, low functional independence measure for entering and leaving, the existence of sensitivity disorders, spatial neglect, failed seated and standing equilibrium, and sedative treatment. In most cases, whatever their nature, falls occurred during transfers (68% of cases). Most often, the post-fall lesions were minor. However, in one case, a femur neck fracture necessitated osteosynthesis. One-third of the patients had a fall at home (as opposed to 5% of the control group). CONCLUSION: Our results confirm those in the literature. The predictive factors for falls in patients with hemiplegia are therefore well established and essentially correlate with the CVA. This consideration must lead to implementation of a prevention program including material-based as well as human measures.

Accidental Falls↗

Hemiconvulsion-hemiplegia syndrome and primary human herpesvirus 7 infection.

We report a case of hemiconvulsion-hemiplegia (HH) syndrome. An 18-month-old female infant had a hemiconvulsion followed by left hemiplegia. Magnetic resonance imaging immediately after the onset of hemiplegia showed high intensity in the right hemisphere in diffusion-weighted images (DWI), while T1- and T2-weighted images were normal. Single photon emission computed tomography showed hypoperfusion of the right hemisphere in the acute phase. Virological analyses proved primary human herpesvirus 7 (HHV-7) infection. DWI are useful for the early evaluation of HH syndrome. Vascular disorders due to HHV-7 infection may have been related to the development of HH syndrome in this patient.

Cerebral Cortex↗

Motor strategies in standing up in children with hemiplegia.

In spastic hemiplegia, the organization of whole body movements is impaired by deficient postural control. We studied segmental motor patterns involved in standing up from supine position in 15 children with spastic hemiplegic cerebral palsy and 14 unimpaired children using a visual analysis scale previously validated for developmental research. This approach examines specific movement patterns in upper limbs, axis, and lower limbs. We found that children with hemiplegia use movement patterns described in normal children but with reduced interindividual variability and a significant preponderance of asymmetric patterns. One previously undescribed stereotyped lower limb pattern was observed in two children with spastic hemiplegia. Emergence of these patterns is consistent with the referent body image theory. This approach can systematically characterize the limited repertoire of movement in patients with disorders of movement and posture and therefore contribute to a better understanding of motor control. The approach may guide management proposals with particular reference to variability and symmetry and might be used as a follow-up tool.

Cerebral Palsy↗

The anatomy of anosognosia for hemiplegia: a meta-analysis.

Anosognosia for hemiplegia is the denial of the contralesional motor deficits that may follow brain damage. Although this disturbance has been reported in the neurological literature since the beginning of the last century, only few longitudinal studies have addressed the issue of the anatomical substrate of the disorder. Here we present a comprehensive review of the literature on anosognosia for hemiplegia from 1938 to 2001, taking into account some of its clinical, epidemiological and anatomical aspects. In particular, an attempt has been made to identify the intra-hemispheric lesion locations most frequently associated to the denial behaviour. Our review shows that anosognosia for hemiplegia most frequently occurs in association to unilateral right-sided or bilateral lesions of different brain areas (cortical and/or subcortical). It seems to be equally frequent when the damage is confined to frontal, parietal or temporal cortical structures, and may also emerge as a consequence of subcortical lesions. Interestingly, the probability of occurrence of anosognosia is highest when the lesion involves parietal and frontal structures in combination, if compared to other combinations of lesioned areas. This pattern of lesions suggests the existence of a complex cortico-subcortical circuit underlying awareness of motor acts that, if damaged, can give raise to the anosognosic symptoms.

Adult↗

Alternating hemiplegia of childhood.

We report 22 cases of alternating hemiplegia of childhood. In addition to repeated episodes of hemiplegia lasting from a few minutes to several days, the disease was characterized by an onset before 18 months of age, the occurrence of tonic or dystonic attacks, nystagmus, dyspnea and other autonomic phenomena, and the development of cognitive impairment and of a choreoathetotic movement disorder. All the patients also had episodes of quadriplegia that occurred either when a hemiplegia was shifting from one side to the other or as an isolated manifestation. Such episodes were often severe and followed by developmental deterioration. In all children, sleep consistently relieved both weakness and associated paroxysmal phenomena, but these would reappear 10 to 20 minutes after the children awakened, during long-lasting episodes. Although six patients also had epileptic seizures, the condition seems to be distinct from epilepsy, and the clinical features and poor outcome differentiate it from migraine. Treatment with the calcium-entry blocker flunarizine was partially effective.

Diagnosis, Differential↗

[Anosognosia for hemiplegia with pontine infarction].

INTRODUCTION: Anosognosia for hemiplegia (AHP) is unawareness of unilateral motor deficit. This syndrome usually is reported in association with large lesions of the nondominant frontal and parietal lobes, the perithalamic lesions or their connexions with cortical or subcortical structures. Little is known about AHP in patients with brainstem's infarctions. CASE REPORT: A 79-year-old right handed woman, without history of intellectual deterioration or psychiatric diseases, with hypertension and paroxysmal atrial fibrillation, was admitted with acute left hemiplegia, somatosensory left deficit and slurred speech. Cerebral MRI indicated a recent infarct in the right anteromedial pontine territory. The patient had moderate anosognosia for hemiplegia (AHP) during the first week after the onset of stroke. There was no sign of left spatial hemineglect or left hemiasomatognosia, no persistant mental confusion and no associated significant cortical or subcortical lesions. CONCLUSION: We presumed that AHP is compatible with brainstem's lesions, particularly with pontine infarcts. The pathogenesis of AHP in pontine infarcts may result from the functional deactivation of frontal and parietal areas.

Aged↗

[Hemiplegia and return to domicile].

OBJECTIVE: We have studied the outcome after a stroke on being discharged from a department of physical medicine and rehabilitation in patients with hemiplegia, and the factors influencing the choice of the orientation. MATERIAL AND METHOD: This prospective study was based on 93 patients. The mode of discharged was patient's domicile, institution, the number of death was also noted. The potentially influential factors studied were age, the side with hemiplegia, the aetiology of the hemiplegia, co-morbidity, the delay in starting rehabilitation, the neurological damage evaluated by the Orgogozo score, the initial functional damage evaluated by Functional Independence Measure (FIM) and by the functional score carried out within the framework PMSI(1) (computerised programme of medical care), the existence of aphasia, the existence of a depressive syndrome, presence of hemineglect, presence of superficial or profound sensory disorders, incontinence at the start of rehabilitation and at one month after the stroke, the existence of cognitive or psychiatric disorders. The tests used were the non-parametric test of Mann and Whitney, the chi(2) test and the correlation test. The threshold of significance used was 0.05. RESULTS: Based on 93 patients (47 women and 46 men, average age 64.8) 81 have gone back to their previous domicile, 11 were oriented toward an institution. One patient died. The predictive factors or those linked to an absence of return to the previous domicile were the age, social situation, the delay in starting rehabilitation, presence of aphasia, the initial and final functional damage, the impossibility to walk, the presence of a depressive syndrome, urinary incontinence. CONCLUSION: The authors stress the importance of familial environment and of functional independence in establishing a prognosis for return to the domicile.

Activities of Daily Living↗

Epileptic seizures difficult to differentiate from alternating hemiplegia in infants: a case report.

A child with epileptic seizures had a clinical course similar to that of alternating hemiplegia of infancy (AHI). Tonic hemiplegia began at 2 months of age, and atonic alternating hemiplegia and tetraplegic attacks began at 2 years of age. Clinical findings were paroxysmal ocular movement abnormalities, choreoathetotic involuntary movements, and severe developmental retardation. An interictal EEG at 6 years of age showed multiple independent spike discharges. An ictal EEG showed diffuse irregular spike-wave and slow wave bursts following focal spikes. The present case suggests that a long-term extensive follow-up is necessary to differentiate epileptic seizures from AHI.

Child↗

Acute hemiplegia with lacunar infarct after varicella infection in childhood.

We report 4 cases of acute hemiplegia and a small low-density lesion on computerized tomography (CT) after varicella infection. In 3 of them, CT in the acute hemiplegic stage, and later, reveals the development of lacunar infarct around the internal capsule. Focal low density may be caused by occlusive vascular lesions of the penetrating arteries. Varicella infection may play an important role as one of the causes of acute hemiplegia in childhood producing lacunar infarct, as well as delayed hemiplegia, reported previously in herpes zoster ophthalmicus.

Acute Disease↗

Alternating hemiplegia of childhood: clinical manifestations and long-term outcome.

We present our analysis of 44 patients with alternating hemiplegia of childhood. The clinical course usually consisted of three phases. The first was dominated by abnormal eye movements and dystonic episodes, the second by hemiplegic spells and psychomotor regression, and the third by persistent developmental delay and fixed neurologic deficits. The age of onset was 0-54 months (mean = 7.9 +/- 13 months). The presenting signs included abnormal ocular movements in 65%, dystonia in 60%, and hemiplegia in 32%. Patients with an early onset of the disorder and an early appearance of hemiplegic spells faired the poorest developmentally. Developmental delay was present in 91%, ataxia in 68%, choreoathetosis in 50%, and seizures in 18%. Laboratory investigations suggested mitochondrial abnormalities and cerebrovascular dysfunction in several patients. Numerous therapies were largely ineffective. Flunarizine reduced the duration, severity, and frequency of the hemiplegic attacks in 78%. Patients who received flunarizine did not differ developmentally from those who did not. Our data suggest that flunarizine does not adversely affect and may favorably influence the outcome in patients with alternating hemiplegia of childhood. Additionally, the occurrence of autosomal-dominant cases of the syndrome, although rare, suggests that, in addition to mitochondrial dysfunction, genetic factors may be important.

Adolescent↗

Acute childhood hemiplegia associated with chickenpox.

Although primary varicella-associated central nervous system complications and herpes zoster ophthalmicus with delayed hemiplegia are well known, and chickenpox is a common infection, cerebral vasculopathy associated with chickenpox has only been described recently. We report the case of an 18-month-old girl who developed a right hemiplegia 10 days after the onset of a primary varicella infection. She has the shortest latent interval in the literature. Cranial computed tomography and magnetic resonance imaging suggest an infarction involving the left putamen and internal capsule. Magnetic resonance angiography is normal. Possible causes of acute infantile hemiplegia are excluded. Neurologic signs gradually improve without any specific treatment. A review of the literature is also presented.

Acute Disease↗

Shoulder pain in hemiplegia.

Shoulder pain is a common complication in poststroke hemiplegia that reduces functional recovery. Many types of shoulder pathology have been suggested as causes of shoulder pain in hemiplegia,including shoulder subluxation, capsulitis, tendinitis, rotator cuff injury, bursitis, impingement syndrome, spasticity, complex regional pain syndrome, brachial plexus injury, and proximal mononeuropathies. More than one type of pathology may exist in a given patient. Shoulder pain improves in many cases with prompt diagnosis and appropriate management. Although the relationship between subluxation and pain is controversial, upper limb support to reduce subluxation is the standard of care and may prevent the development of pain and secondary complications. Further work is needed to elucidate the natural history of shoulder pain in hemiplegia, including the identification of physiologic common denominators that can lead to improved strategies to treat and prevent shoulder pain.

Brachial Plexus Neuropathies↗