Choreoathetotic syndrome following cardiac surgery.

Movement disorders following heart surgery are very unusual. Post-pump chorea is mainly a pediatric complication of heart surgery, typically manifesting after a latent period of normality and is usually related with long extracorporeal circulation time and deep hypothermia. We report a 73-year-old woman, without risk factors predisposing to paroxysmal movement disorders, presenting acute choreoathetoid movements 5 days after aortic valvular replacement with normal extracorporeal circulation time and perioperative normothermia.

Birth-related syndromes of athetosis and kernicterus.

"Athetosis," from the Greek athetos, meaning "without fixed position," is a movement disorder first described by Hammond in 1871. The term described slow, irregular continual movements of the distal extremities. In 1983, Foley defined the athetoid syndrome as "a nonprogressive but evolving disorder due to damage to the basal ganglia of the full-term brain … [with] impairment of postural reflexes, arrhythmical involuntary movements, and dysarthria, [but] sparing … sensation, ocular movements and … intelligence." A decade later, "athetoid syndrome" was replaced by "dyskinetic cerebral palsy." Injury to basal ganglia by various mechanisms, including asphyxia, trauma, perinatal strokes, and kernicterus, is known to cause birth-related athetosis. Kernicterus originally described the neuropathology of bilirubin-induced brain injury, where the deep nuclei of the brain stain yellow. Kernicterus now describes the clinical features of chronic bilirubin encephalopathy, which include an extrapyramidal movement disorder, sensorineural hearing loss, impaired upward gaze, and dental enamel dysplasia. Aggressive treatment of perinatal hyperbilirubinemia has led to a decline in kernicterus so that, today, it is a rare cause of dyskinetic cerebral palsy. In this chapter, we provide a historic overview of athetosis and its formerly common cause, kernicterus. We relate earlier terminology to more recent definitions of impairments in dyskinetic cerebral palsy, including dystonia, chorea, and choreoathetosis.

Limited chronic focal encephalitis: another variant of Rasmussen syndrome?

OBJECTIVE: To describe a more limited and less malignant form of Rasmussen encephalitis (RE). METHODS: Three subjects (all women; 37, 31, and 32 years of age) developed childhood or late onset chronic focal encephalitis, with a relatively nonprogressive form of the disorder. RESULTS: In our patients, clinical features were dominated by partial seizures without marked focal motor deficit and in two with choreo-dystonic movements. The diagnosis of RE was supported by histologic examination and anatomic and functional MRI. CONCLUSIONS: These cases extend the phenotypic presentations of Rasmussen encephalitis and confirm Theodore Rasmussen's suggestion that there may be mild and nonprogressive forms of the disease.

Profound hypothermia with alpha-stat pH management during open-heart surgery is associated with choreoathetosis.

A retrospective chart review was conducted to examine risk factors associated with the occurrence of choreoathetosis, a rare but significant complication of open-heart surgery in children. Ten children were identified as having developed choreoathetosis after cardiac surgery. Their charts were reviewed and compared with 33 age- and diagnosis-matched controls who underwent open-heart surgery during the same time period. Children with choreoathetosis reached lower rectal and esophageal temperatures (p = 0.0018 for both) and spent a greater portion of total bypass time at lower rectal and esophageal temperatures (p < 0.001 for both). Duration of cooling below 20 degrees C esophageal temperature and PaCO2 at the end of the cooling period were significant predictors of choreoathetosis (p = 0.023 and p = 0.0497, respectively) in a logistic regression model, and a greater fraction of choreoathetosis patients had prior developmental delays (p = 0.017). No difference was found in the age at surgery, duration of bypass, aortic cross-clamp time, arterial pH, PaCO2 or mean arterial pressure. The combination of extended exposure to profound hypothermia and alpha-stat pH management strategy and preexisting developmental delay are associated with the development of choreoathetosis following open-heart surgery 61 in children.

[Chorea-athetosis in the anti-Hu syndrome]. / Mouvements choréo-athétosiques et syndrome anti-Hu.

INTRODUCTION: Paraneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndrome with choreo-athetosis. CASE REPORT: A 48-year-old woman developed a small-cell lung carcinoma revealed by an anti-Hu syndrome. The neurological features included choreo-athetosis predominating in the upper limbs, chronic sensorimotor axonal polyneuropathy, and opsoclonus. The cerebrospinal fluid was acellular and contained several oligoclonal IgG bands, not found in the corresponding serum. Magnetic resonance imaging revealed bilateral high-intensity lesions on T2/FLAIR sequence in the corona radiata. Moderate transitory improvement of the paraneoplastic neurological syndrome was observed after several carboplatin-etoposid cycles. CONCLUSION: A paraneoplastic origin must be considered in all cases of unexplained choreo-athetosis. Paraneoplastic choreo-athetosis is most often associated with other neurological symptoms. The most frequent associated tumor is a small-cell lung carcinoma with anti-CRMP5 and/or anti-Hu antibodies. Our patient developed paraneoplastic choreo-athetosis related to an anti-Hu syndrome in the absence of anti-CRMP5/CV2 antibodies. Paraneoplastic choreo-athetosis might result from a central lesion, and/or from proprioceptive deafferentation subsequent to peripheral neuropathy.

Pseudoathetosis in a patient with leprosy.

A 35-year-old man with borderline tuberculoid leprosy developed Type I lepra reaction 12 days after anti-leprosy treatment. There was acute worsening of neuropathic symptoms and skin lesions. He developed severe sensory ataxia and pseudoathetosis resulting in marked disability. His symptoms significantly improved on corticosteroid therapy.

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.

[Primary Sjögren's syndrome presenting with choreo-athetosis].

A 69-year-old man presenting with choreo-athetosis was proved to have primary Sjögren's syndrome. Choreo-athetosis suddenly appeared in the bilateral legs and the right arm; it was predominant in the right limbs. On the neurological examinations, there was no abnormal finding except for this involuntary movement. Brain MRI showed no abnormal finding. 18F-Fluorodeoxyglucose PET revealed that glucose metabolism was relatively increased in the bilateral striatum and thalamus (left side dominant). Dry eye and thirst were present for 4 years, and a diagnosis of primary Sjögren syndrome was confirmed by ophthalmological examinations, lip biopsy and sialography. Choreo-athetosis disappeared after thioridazine hydrochloride was started, while steroid was not effective.

Emergence of complex, involuntary movements after gamma knife radiosurgery for essential tremor.

Gamma knife radiosurgery is generally considered a safer alternative to traditional pallidotomy or thalamotomy. We report the case of a 59-year-old patient with essential tremor who developed a complex, disabling movement disorder following gamma knife thalamotomy. This case illustrates the need for long-term follow-up to fully evaluate the potential for complications following radiosurgery.

[Pseudochoreoathetosis as manifestation of cervical cord lesion. Case report]. / Pseudochoreoatetoza jako objaw uszkodzenia rdzenia kregowego w odcinku szyjnym. Opis przypadków.

The involuntary movements of choreoathetotic type are commonly regarded as a sign of basal ganglia lesion. However, such movements can also occur in pathological processes involving the cervical spinal cord. This condition is referred to as pseudochoreoathetosis. Involuntary movements can be related to lack of proper coordination between agonist and antagonist muscles, their simultaneous activation due to impairment of reciprocal inhibition. The characteristic feature of pseudochoreoathetosis is proprioceptive sensory loss. In this paper we present 4 patients who developed various involuntary limb movements in early stage of the disease. Lesions in the cervical spinal cord were confirmed by MRI. In case 1 the cause was astrocytoma, in cases 2 and 3--demyelination, in case 4 the precise character of the lesion could not be established. Pseudochoreoathetosis is a rare condition which often remains unrecognized. The presented cases emphasise the importance of early and correct diagnosis leading to proper therapeutical procedure.

Meningoencefalitis por cryptococcus: reporte de un caso clínico y revisión de la literatura / Cryptococcal meningoencefalitis: a clinical case report and literature review

La infección por cryptococcus en el niño es muy infrecuente especialmente si es inmunocompetente. Se presenta un preescolar inmunocompetente con meningoencefalitis grave con líquido cefalorraquídeo claro y signos neurológicos de focalización, tratado como meningoencefalitis herpética. Evolucionó inicialmente con leve mejoría, reingresando por aumento del compromiso neurológico. Se amplió el estudio etiológico y se detectó cryptococcus en el LCR. Se comenta el cuadro clínico de la meningoencefalitis cryptococócica su diagnóstico y revisión de la literatura. Nos parece aconsejable incluir el test de tinta china en toda meningoencefalitis a líquido claro de etiología no precisada

Paroxysmal kinesigenic choreoathetosis associated with frontotemporal arachnoid cyst--case report.

A 17-year-old male presented with paroxysmal kinesigenic choreoathetosis (PKC) associated with frontotemporal arachnoid cyst. Xenon-133 single photon emission computed tomography detected a slight but equivocal decrease in regional cerebral blood flow in the vicinity of basal ganglia associated with the PKC episodes. PKC continued after surgical removal of the cyst but was well controlled by oral administration of carbamazepine. Whether the pathogenesis of symptomatic PKC was associated with the cortical lesion could not be determined in the present case.

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.