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PubMed1989

Motoscopic analysis of gross motor patterns in athetotic cerebral palsied children.

Rolling, sitting, and crawling patterns were motoscopically analyzed in 51 athetotic cerebral palsied children. In rolling, rotation of the trunk and support on the elbows were difficult in some subjects. When sitting, most of the subjects had a "W" sitting pattern in which the hips were adducted and the knees were flexed. As for crawling, reciprocal movements of the four extremities were disturbed. The reciprocal hip movements were insufficient and accompanied the lateral bending of the trunk in many of the children. The hips supported the weight in flexion, and the synchronized extension of the hips, with or without lateral bending of the trunk, propelled the body in some. Creeping on the elbows was shown only in the younger children.

Adolescent↗
PubMed1998

Episodic ataxia and channelopathies.

Clinical details are given of different types of episodic ataxia: type 1, with myokymia, and attacks which usually last a few minutes, and may occur several times a day, and treatment with acetazolamide can reduce the number of attacks; type 2, with interictal nystagmus, and attacks which last for several hours to a day or more, and treatment with acetazolamide is very effective; paroxysmal choreoathetosis with episodic ataxia, with attacks lasting for about 20 min and occurring at varying intervals; and familial hemiplegic migraine, with transient hemiplegia presenting during the aura of a migraine headache, the symptoms improving on treatment with acetazolamide. Their inheritance is of dominant type; and the gene for type 1 is mapped to chromosome 12p near to a cluster of potassium channel genes, and that for type 2 and for familial hemiplegic migraine to chromosome 19p near to calcium channel genes. The differential diagnosis from other conditions with a periodic symptomatology is discussed, especially from a number of metabolic disorders. Treatment is effective for many of these, and the treatment of the episodic ataxias with acetazolamide can sometimes have a dramatic effect. The possible role of the channelopathies in the causation of some periodic neurological disorders is considered; with the expectation that further research will improve the identification of specific diseases, and lead to more effective treatment.

Acetazolamide↗
PubMed1998

Using postural reactions as a screening test to identify high-risk infants for cerebral palsy: a prospective study.

To clarify the predictive value of the seven more commonly used postural reactions (PR) in the 1st year of life regarding the diagnosis of cerebral palsy (CP), we prospectively examined 204 high-risk infants of whom 58 developed CP, 22 had developmental retardation (DR) and 124 were normal at follow-up at 3 years of age. Abnormalities of five or more PR from the 1st month of life were correlated with spastic CP, while five or six abnormal PR were also correlated with athetoid CP. Three or less abnormal PR correlated with a normal outcome. All seven PR tested were significantly abnormal in children with spastic CP from the 1st month compared to normal children. Athetoid children demonstrated abnormalities of the Peiper-Isbert (P-I) reaction and Vojta reaction from the 1st month and of the vertical, horizontal and Collis vertical suspension from the 3rd month. Children with DR had significantly abnormal Collis horizontal and Collis vertical suspension, Vojta reaction and traction response from the 1st month and Peiper-Isbert reaction from the 3rd month. Ataxic children demonstrated significantly abnormal traction response from the 1st month, Collis horizontal reaction from the 7th month and Peiper-Isbert reaction from the 11th month. We conclude that the examination of PR is a useful quantitative and qualitative diagnostic screening tool for high-risk infants from the 1st month of life.

Athetosis↗
PubMed2001

Ictal (99m)Tc ECD SPECT in paroxysmal kinesigenic choreoathetosis.

Paroxysmal kinesigenic choreoathetosis is a rare neurologic disorder characterized by sudden attacks of brief involuntary dyskinetic movement that are precipitated by voluntary movement. A 14-year-old male who presented with frequent brief attacks of hemidystonia triggered by sudden movement is reported. Investigations, including video electroencephalogram and magnetic resonance imaging of brain, were normal. There was excellent and sustained response to carbamazepine. Ictal single-photon emission computed tomography using (99m)Tc ethyl cysteinate dimer revealed increased perfusion of the contralateral basal ganglia, which is associated with onset of choreoathetosis attacks. Our findings provide evidence that hyperactivity of the basal ganglia is associated with the dyskinetic attacks in paroxysmal kinesigenic choreoathetosis.

Adolescent↗
PubMed2002

Neurologic outcome of choreoathetoid encephalopathy after cardiac surgery.

Postoperative encephalopathy with choreoathetosis is a serious neurologic complication of childhood cardiac surgery. An inadequate understanding of the long-term outcome has limited family counseling and the institution of intervention strategies. Our objective was to define the long-term neurologic, cognitive, and behavioral outcome in this group of patients. By retrospective review we identified all cases of choreoathetosis in our cardiac intensive care unit from 1986 through 1995. During this decade we described a sudden transient increase in postoperative choreoathetosis that disappeared as we modified treatment strategies in perioperative care. These children underwent a comprehensive battery of neurologic, cognitive, and behavioral tests to determine in detail their long-term outcome. Of the 36 cases identified of which three were deceased, four were abroad, and eight were lost to follow-up, 21 families were approached, and 15 of the 21 (71%) consented to testing. We found pervasive deficits in memory, attention, and language, with a median full-scale IQ of 67 (range = 40-122), as well as in motor function, including persistent dyskinesia in seven of 15 (47%). The pervasive and enduring cognitive and motor deficits in survivors of postoperative choreoathetosis caution for guarded predictions of outcome and highlight the need for careful neurologic and cognitive evaluation in all children with choreoathetosis after open heart surgery.

Athetosis↗
PubMed1998

Choreoathetosis after cardiac surgery with hypothermia and extracorporeal circulation.

Eleven children, 4-48 months old, with congenital cyanotic heart defects developed choreoathetoid movements 2-12 days after cardiac surgery with hypothermia and extracorporeal circulation (ECC). The abnormal movements mainly involved the limbs, facial musculature, and tongue, leading to a severe dysphagia. The symptoms had an acute onset, after a period of apparent neurologic normality, and had a variable outcome. Of the nine children that survive, three had abnormal movements when last seen (41 days to 12 months of follow-up). The other six children had a complete regression of the choreoathetoid movements 1-4 weeks after onset. No specific finding was observed in the CT scans, cerebrospinal fluid examination, or EEG that could be related to the abnormal movements. Symptomatic therapy with haloperidol with or without benzodiazepines led to symptomatic improvement in six children, although there was no evidence that this treatment modified the evolution of the disease. The authors conclude that the choreoathetoid syndrome after cardiac surgery with deep hypothermia and ECC is an ill-defined entity requiring additional study to better understand its pathogenesis so that preventive measures can be taken to avoid a condition that can lead to permanent and incapacitating neurologic sequelae.

Adolescent↗
PubMed1999

3-Methylglutaconic aciduria type I: clinical heterogeneity as a neurometabolic disease.

3-Methylglutaconic (3-MGC) aciduria with 3-methylglutaconyl-CoA hydratase deficiency (3-MGC aciduria type I) is a rare inherited metabolic disease of L-leucine catabolism. We describe a 9-month-old Japanese boy with this disorder who showed progressive neurological impairments presented as quadriplegia, athetoid movements and severe psychomotor retardation from 4 months of age. This finding indicates the existence of clinical heterogeneity in 3-MGC aciduria type I, suggesting it may present as a neurometabolic disease.

Acidosis, Renal Tubular↗
PubMed2007

[Chorea].

Chorea (from the greek word "dance") is characterized by short, involuntary, irregular, nonrepetitive, adventitious muscular contractions. The resulting movements may occur abruptly at any part of the body, but may also spread from one body part to another. The most important condition showing chorea as its core symptom is Huntington's disease. It is inherited as an autosomal dominant trait. During its course it presents as an admixture of neurological and psychiatric features. Several rare neurodegenerative diseases and conditions of inborn errors of metabolism present with chorea mostly between childhood and adolescence. Among these, the paroxysmal choreoathetoses are important for the differential diagnosis of seizures and transitory ischaemic attacks later in life. In adults, symptomatic chorea may occur in many drug-induced, metabolic, infectious, immunological, toxic, and vascular conditions. In addition to the basic treatments of these illnesses there are selected drugs to provide satisfactory symptomatic relief from the abnormal movement.

Adolescent↗
PubMed2006

Neurologic events in neonates treated surgically for congenital heart disease.

OBJECTIVE: The incidence of acute neurologic events prior to discharge in neonates with congenital heart disease (CHD) was determined and peri-operative characteristics predictive of a neurologic event were identified. STUDY DESIGN: A retrospective chart review over 1 year was conducted of infants <1 month of age with a diagnosis of CHD. Outcomes were measured by the occurrence of an acute neurologic event defined as electroencephalogram (EEG)-proven seizure activity, significant hypertonia or hypotonia, or choreoathetosis prior to hospital discharge. Stepwise logistic regression identified variables most likely to be associated with an acute neurologic event. RESULTS: Surgical intervention occurred in 95 infants who were admitted with a diagnosis of CHD. The survival rate was 92%. Of the survivors, 16 (17%) had an acute neurologic event, with 19% of events occurring preoperatively. Factors associated with neurologic events included an elevated nucleated red blood cell (NRBC) count, an abnormal preoperative brain imaging study, and a 5-min Apgar score <7 (P<0.05). CONCLUSIONS: Neonates with CHD have a significant risk of neurologic events. Preoperative brain imaging, the 5-min Apgar score, and initial serum NRBC counts may identify infants at highest risk for central nervous system injury.

Acute Disease↗
PubMed1984

Reproducibility and variability of speech muscle activity in athetoid dysarthria of cerebral palsy.

Athetoid dysarthria is thought to result from involuntary movements which are variable and irregular in nature. In this study, electromyographic (EMG) activity recorded from six speech muscles was quantified during repetitions of a test sentence by normal and athetoid adult subjects. In the athetoid subjects the articulation of the test sentence was disrupted intermittently by involuntary activity which usually occurred in the time intervals between the syllables in the test sentence, rather than during articulation of the syllables themselves. The EMG activity associated with each syllable in the test sentence was partitioned into reproducible and variable components. The ratio of the reproducible component to the variable component--the signal-to-noise ratio--did not differ significantly between the two subject groups. In the athetoid subjects, however, the reproducible component of the EMG activity was grossly abnormal. We concluded that this abnormal voluntary activity, rather than variable involuntary activity, was the primary cause of athetoid dysarthria.

Adult↗
PubMed2002

The neurobehavioural effects of kava.

OBJECTIVE: This review considers the context in which kava is used, together with its underlying psychopharmacological mechanisms, to investigate the neurobehavioural effects associated with kava use. METHOD: We conducted a systematic search using the computerized databases MEDLINE, OVID and PsychLIT for all articles containing any of the following words: kava, kavain, kawa and Piper methysticum. In the opinion of the authors, all articles from this collection containing data that could inform the neurological and cognitive sequelae of kava use were included for the purpose of this review. RESULTS: The use of kava occurs among indigenous populations in the South Pacific and in northern Australia, while also being used throughout the western world as a herbal medicine. Animal studies show that kava lactones alter neuronal excitation through direct interactions with voltage-dependent ion channels, giving rise to kava's muscle relaxant, anaesthetic, anxiolytic and anticonvulsive properties. Several isolated cases of psychotic and severe dystonic reactions following kava use suggest that kava also has psychoactive properties, yet there is no conclusive evidence that kava interferes with normal cognitive processes. CONCLUSIONS: Kava is effective in the treatment of tension and anxiety. There may be risk-factors for severe motor and psychiatric responses to kava use, although these are not well-understood. Given the increasingly widespread use of kava, further investigation is necessary to gain an understanding of its immediate neuropsychiatric effects and long-term cognitive effects.

Adult↗
PubMed2000

Athetoid quadriplegia and literacy.

OBJECTIVE: To compare literacy levels in athetoid quadriplegic (AQ) patients born in the 1960s and 1970s with those born in the 1980s and 1990s. METHODOLOGY: Nine patients with AQ born between 1966 and 1972 were compared with eight patients born between 1983 and 1991. RESULTS: Reading difficulties were found in almost all patients with AQ, despite normal intellect. Those in the earlier period were for the most part functionally literate, while those in the later period were functionally illiterate. CONCLUSIONS: Specific and intensive reading education may be required in patients with AQ to obtain functional literacy.

Athetosis↗
PubMed2003

Choreoathetosis related to lithium intoxication.

A 76-year old woman was admitted because of severe gait ataxia and dysarthric speech that had worsened over the last 24 h. The patient was initially suspected of having repeated transitory ischemic attacks (TIAs) as her daughter reported a similar episode that had happened 3 weeks prior to admission. The onset of spontaneous twisting, choreoathetotic movements of both hands and arms and worsening of symptoms one hour after admission together with a history of lithium therapy lead to the correct diagnosis and appropriate treatment.

Aged↗
PubMed2002

Idiopathic paroxysmal kinesigenic choreoathetosis: precipitation of attacks by vestibular stimulation.

A 24-year-old woman presented with a 3.5-year history of paroxysmal dystonia that was precipitated by sudden movement, especially when she started to walk. It was characterised by shrugging of shoulders, flexion of the neck and thoracic spine, and stiffness of the right leg followed by falls. Each attack lasted for less than 5min. Inadequate sleep and stress were exacerbating factors. There was no similar family history. Physical examination and investigations were normal. The following manoeuvres that caused vestibular stimulation precipitated attacks: turning her head from side to side while standing still, sitting still on a rotating chair and an ice-water caloric test. She had partial responses to phenytoin and levodopa, and a good response to haloperidol. Vestibular stimulation as a precipitating factor in paroxysmal kinesigenic choreoathetosis has not been reported previously.

Adult↗