Involuntary Choreiform Movements Research Articles

Neurobehavioral Changes Associated with Rheumatic Fever and Rheumatic Heart Disease: A Narrative Review

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are autoimmune conditions triggered by Group A Streptococcus skin or throat infections. If ARF/RHD is undetected, misdiagnosed or antibiotic treatment is not provided early, patients may develop cardiac failure, leading to premature death. Although it is an easily preventable disease, ARF/RHD remains the most significant cause of heart disease-associated deaths in people under 25 years old, both in low- and middle-income countries and among First Nations in high-income countries. Up to 30% of the patients with ARF/RHD present with a neurobehavioral condition – Sydenham’s chorea (SC). The clinical course of SC is mostly self-limiting and is characterized by the onset of involuntary choreiform movements and neuropsychiatric features such as obsessive-compulsive disorder, tics, depression and anxiety, psychosis, and attention-deficit hyperactivity disorder. While the precise mechanism as to why only a proportion of patients with ARF/RHD develop SC remains unknown, an impaired blood–brain barrier is considered to play a central role in its development. The most well-characterized neurobehavioral outcome is stroke which may occur in isolation or as part of systemic thromboembolism. Both infective endocarditis and mitral valve disease with or without aortic valve disease increase the embolic and ischemic stroke risk. ARF/RHD is known to significantly impact the quality of life with neuropsychiatric consequences. Another neurobehavioral syndrome which occurs in the absence of ARF/RHD is “pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections” (PANDAS). PANDAS has been categorized as a subset of pediatric acute-onset neuropsychiatric syndromes. However, establishing a diagnosis of PANDAS has been challenging. In this review, we discuss the current status of our understanding regarding the different manifestations of poststreptococcal neurobehavioral changes. Particular attention is given to ARF/RHD-associated SC, and we highlight the areas for further research to understand the association between poststreptococcal sequelae and neurobehavioral abnormalities.

Evaluation of Abnormal Movements among Senior Schizophrenics: A Local Study with Reference to Tardive Dyskinesia

Introduction: Tardive dyskinesia (TD) includes involuntary choreiform or athetoid movements of the jaw, lower face, tongue, and extremities, developing in association with the use of an antipsychotic medication, and may develop in about 20 to 40 percent of patients who require long-lasting hospitalization. In the present study, the prevalence of this condition has been measured among an elderly group of schizophrenic patients. Methods: One hundred and one elderly schizophrenic patients, who were hospitalized in the chronic section of a community psychiatric hospital, were selected for the present cross-sectional study. Abnormal Involuntary Movement Scale (AIMS) was employed to screen for patients with schizophrenia who also had TD. Scale for Assessment of Positive Symptoms, Scale for Assessment of Negative Symptoms, Schedule for Assessment of Insight, and Clinical Global Impressions – Severity of illness, as well, had been used as ancillary scales for evaluation of severity of general psychopathology of schizophrenia, and comparing the TD patients with the group of patients without TD, for probing the intervening parameters. Results: While abnormal movements were clear in 38.61% (n=39) of elderly schizophrenic patients, only seven of them (6.93 %) could be diagnosed as TD, based on the above-mentioned criteria. All of them were using conventional antipsychotic medications, accompanied with anticholinergic medications. Among TD patients, three cases had only abnormal facial and oral movements, one patient had atypical facial and oral movements as well as anomalous extremity movements, one patient had irregular facial and oral movements in addition to unusual trunk movements, and lastly, two patients had nonstandard extremity movements. In addition, around 71% of patients with TD were aware of their unusual movements. Between-group analysis did not show any significant difference between patients with TD and patients without TD in age, duration of illness, positive symptoms, negative symptoms, insight, and general psychopathology. Conclusion: According to the findings of the present study, the prevalence of Tardive Dyskinesia among elderly schizophrenic patients, who were using typical antipsychotic medications, is very lower than what has been indicated thus far.

Involuntary choreiform movements in a diabetic patient

Involuntary choreiform movements in a diabetic patient

A case of choreiform movement presentation in a difficult to control epilepsy

Objectives Epilepsy is a prevalent neurological disease; refractory epilepsy is often associated with congenital malformations of the brain. This condition is highly disabling, affecting normal brain development and learning abilities. Our objective is to report a case of a child with right mesial temporal mesial sclerosis, with a rare clinical presentation: choreiform movements. Methods Patient ACSP, 8 years old, female, started epileptic seizure at 4 years of age, characterized by involuntary choreiform movements in the left dimidium, without loss of consciousness, short duration and difficult to control. A year ago he had worsening of the episodes with more prolonged crises, makes a movement of tonic extension of the left arm, to the teeth and leaves the eyes wide open. She is on high doses of anti-epileptic drugs without control. Magnetic resonance imaging shows right mesial temporal mesial sclerosis. Results The patient underwent video-EEG monitoring, presenting choreiform movements in the left side of the body, almost continuous. Ictal EEG: focal slowing characterized by delta theta rhythmic activity in the right temporal region. Inter-ictal EEG: acute near-continuous paroximal waves in the right hemisphere. Conclusions Mesial temporal lobe epilepsy is the most common form of partial epilepsy in adults, the presentation is usually in childhood. It can be sporadic, usually with a positive family history. Anatomic or functional lobectomy is indicated if epilepsy is clinically intractable. The patient is now waiting to undergo neurosurgery.

Muscle atrophy is associated with cervical spinal motoneuron loss in BACHD mouse model for Huntington's disease.

Involuntary choreiform movements are clinical hallmark of Huntington's disease, an autosomal dominant neurodegenerative disorder caused by an increased number of CAG trinucleotide repeats in the huntingtin gene. Involuntary movements start with an impairment of facial muscles and then affect trunk and limbs muscles. Huntington's disease symptoms are caused by changes in cortex and striatum neurons induced by mutated huntingtin protein. However, little is known about the impact of this abnormal protein in spinal cord motoneurons that control movement. Therefore, in this study we evaluated abnormalities in the motor unit (spinal cervical motoneurons, motor axons, neuromuscular junctions and muscle) in a mouse model for Huntington's disease (BACHD). Using light, fluorescence, confocal, and electron microscopy, we showed significant changes such as muscle fibers atrophy, fragmentation of neuromuscular junctions, axonal alterations, and motoneurons death in BACHD mice. Noteworthy, the surviving motoneurons from BACHD spinal cords were smaller than WT. We suggest that this loss of larger putative motoneurons is accompanied by a decrease in the expression of fast glycolytic muscle fibers in this model for Huntington's disease. These observations show spinal cord motoneurons loss in BACHD that might help to understand neuromuscular changes in Huntington's disease.

Uneventful Recovery From a Suicide Attempt with Tetrabenazine: A Case Report

175 Patients with Huntington’s disease (HD) have involuntary choreiform movements, dementia, psychiatric disorders and behavioral abnormalities. Suicide is very frequent with this disease and is the third most common cause of death. Depression is one of the common adverse effects of tetrabenazine, a drug used to treat chorea in HD, and this may increase the suicide rate in this disease. We report a patient who attempted suicide by taking tetrabenazine and recovered after treatment.

Our Experience with Outpatient Anesthesia in a Patient with Huntington Chorea

Huntington chorea is a rare autosome dominant disorder. Men and women are equally affected as it is an autosomally transmitted. Symptoms occur in the third and fourth decade of life and manifest as involuntary choreiform movements, ataxia, and progressive mental deterioration. Patients are at higher risk for some intraoperative complications. Only a few case reports have been published reporting the anesthetic management of patients having Huntington’s chorea and we would like to submit our experiences for this seldom case.

Transplantation of GABAergic Cells Derived from Bioreactor-Expanded Human Neural Precursor Cells Restores Motor and Cognitive Behavioral Deficits in a Rodent Model of Huntington's Disease

Huntington's disease (HD) is a neurodegenerative disorder that is characterized by progressive dementia, choreiform involuntary movements, and emotional deterioration. Neuropathological features include the progressive degeneration of striatal γ-aminobutyric acid (GABA) neurons. New therapeutic approaches, such as the transplantation of human neural precursor cells (hNPCs) to replace damaged or degenerated cells, are currently being investigated. The aim of this study was to investigate the potential for utilizing telencephalic hNPCs expanded in suspension bioreactors for cell restorative therapy in a rodent model of HD. hNPCs were expanded in a hydrodynamically controlled and homogeneous environment under serum-free conditions. In vitro analysis revealed that the bioreactor-expanded telencephalic (BET)-hNPCs could be differentiated into a highly enriched population of GABAergic neurons. Behavioral assessments of unilateral striatal quinolinic acid-lesioned rodents revealed a significant improvement in motor and memory deficits following transplantation with GABAergic cells differentiated from BET-hNPCs. Immunohistochemical analysis revealed that transplanted BET-hNPCs retained a GABAergic neuronal phenotype without aberrant transdifferentiation or tumor formation, indicating that BET-hNPCs are a safe source of cells for transplantation. This preclinical study has important implications as the transplantation of GABAergic cells derived from predifferentiated BET-hNPCs may be a safe and feasible cell replacement strategy to promote behavioral recovery in HD.

Impact of dopamine versus serotonin cell transplantation for the development of graft-induced dyskinesia in a rat Parkinson model

Graft-induced dyskinesia (GID), covering a range of dystonic and choreiform involuntary movements, has been observed in some patients with Parkinson's disease (PD) after intracerebral cell transplantation. These dyskinesias have been severe in a number of patients and represent one of the main obstacles for further development of the cell therapy in PD. Serotonin neurons, included into the dopaminergic cell suspension due to the nature of the dissection process, have been suggested as a key factor for the development of GID, since the administration of the serotonin (5-HT)1A-receptor agonist buspirone reduced dyskinesia in transplanted PD patients. In the present study, we characterized GID in the rat PD model after transplantation of dopaminergic grafts containing different amounts of serotonin neurons. The severity of GID was significantly correlated with the amount of grafted dopamine and serotonin neurons, but the r-values were low. However, neither the innervation density of dopamine and serotonin fibers in the grafted striatum nor the dopamine-to-serotonin cell ratio correlated significantly with the severity of GID. The results extend prior knowledge of the role of dopaminergic grafts in the development of GID and show that, in the animal model, serotonin neurons within the graft suspension might be involved, but given sufficient dopamine cells, their impact on GID may be minor.

Epidural anesthesia in a patient with Huntington and #8217;s chorea

Huntington's chorea (HC) is a rare, autosomal, dominant hereditary disorder of the nervous system. Symptoms occur in the third and fourth decade of life and manifest as involuntary choreiform movements, ataxia, and progressive mental deterioration. Only a few case reports have been published describing the anesthetic management of patients with HC. Patients are at greater risk for some intraoperative complications (e.g., regurgitation and pulmonary aspiration), poor respiratory function, prolonged response to succinylcholine and thiopental, increased sensitivity to midazolam, and postoperative shivering leading to rigid spasms. All these factors make an ideal anesthesiological management necessary for this group of patients to guarantee an absolute airway protection during all the anesthesia and a fast and safe recovery. To the best of our knowledge, epidural anesthesia in HC patients has not been reported in the literature. Herein, we present our successful experience using epidural anesthesia in a HC patient.

Cellular therapy and induced neuronal replacement for Huntington's disease.

Huntington's disease (HD) is an inherited, relentlessly progressive neurodegenerative disease with an invariably fatal outcome. HD is inherited in an autosomal dominant fashion, and is characterized pathologically by the loss of cortical and striatal neurons, and clinically by involuntary choreiform movements accompanied by progressive cognitive impairment and emotional lability. The disorder is caused by an expanded cystosine adenine guanine (CAG) tri-nucleotide repeat encoding polyglutamine (polyQ) in the first exon of the Huntingtin gene. There is a correlation between the number of CAG repeats and disease onset, such that in patients with CAG repeat lengths of 36 to 60, disease symptoms typically manifest after 35 years of age, whereas CAG repeat lengths >60 yield the more severe juvenile form of the disease. Even though mutant huntingtin is expressed throughout the brain, it is characterized by the selective degeneration of medium spiny neurons of the caudate and putamen, which heralds more widespread neuronal degeneration with disease progression. The mechanisms of cell dysfunction and death in HD have been the subjects of a number of studies, which have led to therapeutic strategies largely based on the amelioration of mutant huntingtin-related metabolic impairment and cellular toxicity. Each of these approaches has aimed to delay or stop the preferential degeneration of medium spiny neurons early in the disease course. Yet, in later stages of the disease, after cell death has become prominent, cell replacement therapy (whether by direct cell transplantation or by the mobilization of endogenous progenitors) may comprise a stronger potential avenue for therapy. In this review, we will consider recent progress in the transplantation of fetal striatal cells to the HD brain, as well as emerging alternative sources for human striatal progenitor cells. We will then consider the potential application of gene therapy toward the induction of striatal neurogenesis and neuronal recruitment, with an eye toward its potential therapeutic use in HD.

Demonstration of striatopallidal iron deposition in chorea-acanthocytosis by susceptibility-weighted imaging

Chorea-acanthocytosis (ChAc) is a rare hereditary disorder characterized by involuntary choreiform movements and erythrocytic acanthocytosis [1]. MRI commonly shows a T2-signal increase in the atrophic striatum, which reveals neuronal loss with gliosis on autopsy. Increased brain iron levels are seen in various neurodegenerative diseases [2–5] and can be easily detected by newer MR imaging technologies, such as susceptibility-weighted imaging (SWI) [2, 3]. We attempted to visualize areas of degenerative change according to abnormal iron deposition in ChAc by using SWI. A 35-year-old woman presented with exacerbation of orolingual dyskinesia, dysarthria and cognitive decline during the course of 3 years. There was no family history of similar neurological disorders. Neurological examination revealed orofaciolingual dyskinesia and intermittent choreiform movements of the head and neck. There was involuntary tongue protrusion and self-mutilating behaviour affecting the tongue and lips. Also, her speech was dysarthric. Muscle power and bulk, and sensory function were all intact. She could walk without difficulty. Hyporeflexia was present in the lower limbs. On laboratory examination, the serum creatine kinase (CK) level was elevated at 345 IU/l (normal range 5–217 IU/l). Serum lipids and lipoprotein electrophoresis were normal. There were no abnormal findings in the nerve conduction study, including electromyography, muscle CT and EEG. Cardiomyopathy, cardiac arrhythmia and hepatosplenomegaly were absent. Genetic tests for Huntington’s disease, DRPLA, and spinocerebellar ataxia 1, 2, 3 and 17 were all negative. Peripheral blood smear revealed 20% acanthocytes confirmed by scanning electron microscopy (Fig. 1a). Brain MR imaging (3.0-T MR system, Verio, Siemens, Germany) revealed the bilateral atrophic putamina and head of caudate nuclei with increased signal intensity on T2-weighted (Fig. 1b) and iso-signal intensity on T1-weighted imaging. SWI (TR = 28 ms, TE = 20 ms, flip angle = 20 slice thickness = 2 mm) showed a paramagnetic dark signal in both striata (Fig. 1c), the corresponding area to T2 hyperintensity. Also, dark condensed stripes were observed in the lateral pallidum (arrows, Fig. 1d). Such a paramagnetic signal was not prominent in conventional gradient echo sequences (Fig. 1e). No evidence of calcification or hemorrhage was observed on brain CT (Fig. 1f). Genetic screening for VPS13A gene or Western blot for chorein was not available. The clinical presentation of this patient most likely fits with ChAc. However, correct clinical diagnosis is difficult due to symptom overlap with McLeod syndrome (MLS) [1]. Although neither mutations of the XK gene nor Kell antigen expression was looked for in this patient, the diagnosis of MLS is unlikely based on the lack of J.-H. Lee (&) Department of Neurology, Medical Research Institute, Pusan National University Yangsan Hospital, Beomo-ri, Mulgum-eup, Yangsan-si, Gyeongsangnam-do 626-770, South Korea e-mail: jhlee.neuro@pusan.ac.kr

Heterozygosity at Polymorphic Codon 219 in Variant Creutzfeldt-Jakob Disease

Genetic variants of the prion protein gene (PRNP) strongly determine susceptibility to prion diseases. All tested patients with definite variant Creutzfeldt-Jakob disease (vCJD) are homozygous for methionine at a common polymorphism at codon 129. A further genetic polymorphism at codon 219, a common variant in several Asian populations, is considered protective against sporadic CJD. To report a finding of heterozygosity at codon 219 in 2 patients with vCJD. Case reports. MRC (Medical Research Council) Prion Unit and Department of Neurodegenerative Disease, University College London Institute of Neurology, and National Prion Clinic, National Hospital for Neurology and Neurosurgery. Patients Two patients with clinical and investigation findings consistent with the diagnoses of probable vCJD. Clinical and genetic findings. A 34-year-old man had a 15-month history of behavioral change progressing to ataxia, dysarthria, involuntary choreiform movements, and severe cognitive impairment. Cerebrospinal fluid analysis was positive for 14-3-3 protein, electroencephalography showed generalized slowing, and magnetic resonance imaging revealed thalamic high signal bilaterally, typical of vCJD. A 31-year-old woman had a 16-month history of cognitive decline, ataxia, involuntary choreiform movements, and myoclonic jerks. Magnetic resonance imaging showed bilateral pulvinar high signal. The diagnosis was confirmed by a tonsillar biopsy demonstrating abnormal prion protein deposition in a typical pattern for vCJD. PRNP sequencing showed a methionine homozygous codon 129 genotype and an E219K polymorphism in both patients. The E219K polymorphism is neutral or may even confer susceptibility to vCJD. The observations are interpretable in the context of the conformational selection model of prion replication. A barrier to prion disease transmission depends on the degree to which permitted pathologic conformations of the prion protein overlap between the inoculum and the host.

Mitochondrial functional alterations in relation to pathophysiology of Huntington’s disease

Huntington's disease (HD) is an autosomal dominant neurodegenerative disease which is characterized by psychiatric symptoms, involuntary choreiform movements and dementia with maximum degeneration occurring in striatum and cerebral cortex. Several studies implicate mitochondrial dysfunction to the selective neurodegeneration happening in this disorder. Calcium buffering imbalance and oxidative stress in the mitochondria, critically impaired movement across axons and abnormal fission or fusion of this organelle in the cells are some of the salient features that results in the loss of mitochondrial electron transport chain (ETC) complex function in HD. Although several models involving mutant huntingtin, excitotoxins and mitochondrial complex-II inhibitors have been used to explore the disease, it is not clear how disturbances in mitochondrial functioning is associated with such selective neurodegeneration, or in the expression of huntingtonian phenotypes in animals or man. We have carefully assessed various mitochondrial abnormalities observed in human patient samples, postmortem HD brains, cellular, vertebrate and invertebrate models of the disease, to conclude that ETC dysfunction is an integral part of the disease and justify a causal role of mitochondrial ETC dysfunction for the genesis of this disorder.

Discriminative behavioral assessment unveils remarkable reactive astrocytosis and early molecular correlates in basal ganglia of 3-nitropropionic acid subchronic treated rats

Reactive astrocytosis seems to be strongly implicated in the development and maintenance of inflammatory and neurodegenerative disorders. We design a new toxic model treatment with 3-nitropropionic acid (3-NP), a mitochondrial complex II irreversible inhibitor, to induce in rats Huntington's disease (HD) like syndrome, characterized by hindlimb dystonia, involuntary choreiform movements and reduced global activity. In an attempt to find out whether molecular and morphological changes in the neuro-glial network could be involved in the pathogenesis of this disease, we developed a protocol of subchronic intra-peritoneal 3-NP intoxication. Moreover we set up specific, highly discriminative, behavioral tests to detect very early mild motor disabilities in 3-NP treated rats. This treatment did not cause severe cell death. However, in the Caudate-Putamen (CPu) of all 3-NP treated animals we found a massive astrogliosis, revealed by increased GFAP levels, paralleled by changes of the glial glutamate transporter GLAST distribution. To these glial changes we detected a transcriptional upregulation of c-fos and Sub-P in the striatal medium spiny neurons (MSN). We propose that this model of 3-NP intoxication along with the designed set of behavioral analyses allow to unmask in a very early phase the motor deficits and the underlying morpho-molecular changes associated to the onset of motor disabilities in the HD-like syndrome. Therefore this model unveil the key role played by the different components of the tripartite synapse in the pathogenesis of the HD, a putative non-cell-autonomous disease.

Functional compensation of motor function in pre-symptomatic Huntington's disease

Involuntary choreiform movements are a clinical hallmark of Huntington's disease. Studies in clinically affected patients suggest a shift of motor activations to parietal cortices in response to progressive neurodegeneration. Here, we studied pre-symptomatic gene carriers to examine the compensatory mechanisms that underlie the phenomenon of retained motor function in the presence of degenerative change. Fifteen pre-symptomatic gene carriers and 12 matched controls performed button presses paced by a metronome at either 0.5 or 2 Hz with four fingers of the right hand whilst being scanned with functional magnetic resonance imaging. Subjects pressed buttons either in the order of a previously learnt 10-item finger sequence, from left to right, or kept still. Error rates ranged from 2% to 7% in the pre-symptomatic gene carriers and from 0.5% to 4% in controls, depending on the condition. No significant difference in task performance was found between groups for any of the conditions. Activations in the supplementary motor area (SMA) and superior parietal lobe differed with gene status. Compared with healthy controls, gene carriers showed greater activations of left caudal SMA with all movement conditions. Activations correlated with increasing speed of movement were greater the closer the gene carriers were to estimated clinical diagnosis, defined by the onset of unequivocal motor signs. Activations associated with increased movement complexity (i.e. with the pre-learnt 10-item sequence) decreased in the rostral SMA with nearing diagnostic onset. The left superior parietal lobe showed reduced activation with increased movement complexity in gene carriers compared with controls, and in the right superior parietal lobe showed greater activations with all but the most demanding movements. We identified a complex pattern of motor compensation in pre-symptomatic gene carriers. The results show that preclinical compensation goes beyond a simple shift of activity from premotor to parietal regions involving multiple compensatory mechanisms in executive and cognitive motor areas. Critically, the pattern of motor compensation is flexible depending on the actual task demands on motor control.

Consecutive pregnancy with chorea gravidarum associated with moyamoya disease

Chorea gravidarum is uncommon movement disorder of pregnancy, characterized by involuntary, abrupt, non-rhythmic movements. It can be idiopathic or secondary to the underlying pathology. A 28-year-old, primigravida woman who was 8 weeks and 6 days of gestation presented with a history of involuntary choreiform movements in the left side limbs and facial twitch for 2 weeks. The symptoms started just after onset of severe emesis gravidarum. There was no meaningful medical history or family history, and she was taking no regular medication. Magnetic resonance imaging of the brain revealed moyamoya disease. The symptoms, as well as the hyperemesis gravidarum, improved with gestational age; however, they were sustained up to 30 weeks of gestation. She delivered 2.61 kg, healthy male neonate at 40 weeks' pregnancy by vaginal delivery under epidural analgesia. After 3 years later, she got pregnant again, and delivered at 41 weeks of pregnancy without complication. She experienced similar but milder symptoms to that of the first pregnancy until the late second trimester. After the delivery, full evaluation was lost due to her refusal. In this report, we present the case of a patient with chorea gravidarum in which moyamoya disease acts as an etiologic factor and a review of literature with management proposal.

Chorea–acanthocytosis presenting with schizophrenia symptoms as first symptoms

CHOREA–ACANTHOCYTOSIS (ChAc) is a progressive neurological disorder that is characterized by choreiform movements and acanthocytes in the peripheral blood.1 It also involves various behavioral and cognitive impairments such as the subcortical type of dementia; impulsiveness, irritability, concentration deficit, or apathy.2 We describe herein a case of ChAc associated with schizophrenia. A 24-year-old man was admitted for delusions of persecution and social withdrawal. Six months before the hospital visit, he sometimes did not go to work and was isolated from his work colleagues. In addition to subsequent delusion of persecution, reference and observation, he gradually showed a loss of concentration, along with irritability, loosening of association, auditory hallucination, and bizarre behaviors. At the beginning of treatment there were no laboratory abnormalities, and his serum beta lipoprotein and cholesterol levels were normal. The high plasma level of homovanillic acid (17.8 ng/mL) on admission decreased to 12.4 ng/mL after recovery. Plasma levels of 5-hydroxyindole acetic acid and 3-methoxy-4-hydroxyphenylglycole were in the normal range. He had no neurological abnormalities. He was diagnosed as having schizophrenia and was treated with various antipsychotic drugs, such as haloperidol (9–18 mg), chlorpromazine (150 mg), and risperidone (2–6 mg). One week after initiating treatment, malignant syndrome was suspected because of high-grade fever and elevated serum creatine kinase (>1000 mU/mL). After recovery he was given anti-psychotics with low potency, such as sulpiride and oxpertine. Subsequently he developed schizophreniform negative symptoms including social withdrawal, poverty of speech, and hypobulia. In addition, he showed personality change characterized by childish behaviors. Two months after discharge, choreiform involuntary movements in the upper and lower extremities gradually increased, which caused stumbling during walking. He also showed orofacial dyskinesia and decreased muscle stretch reflex. Laboratory investigation 2 years later showed that acanthocytes were present in 12–21% of red blood cells. Cerebral computed tomography showed slight bilateral atrophy of the caudate nucleus. He had epileptic seizures and subsequently has been given carbamazepine. Due to all these findings and negative family history, he was diagnosed as having sporadic ChAc. The most striking finding in this report is that the initial symptoms of this case were indistinguishable from those of schizophrenia. Since first described by Levine et al.,3 various psychiatric symptoms have been documented in ChAc.2,4 Although psychosis is generally uncommon, there have been a few articles that described the relationship between ChAc and schizophrenia.4 Accordingly, we considered that the schizophreniform symptoms in the present case were not complications of schizophrenia but features of ChAc. Received 20 August 2008; revised 18 October 2008; accepted 30 October 2008.

Demonstration of Cerebellar Atrophy in Neuroacanthocytosis of 2 Siblings

Neuroacanthocytosis is a rare hereditary disorder characterized by involuntary choreiform movements and erythrocytic acanthocytosis in the peripheral blood. Clinical manifestations of this disorder resemble those of Huntington disease (HD). Neuroimaging features of neuroacanthocytosis are atrophy and signal intensity change of the striata on MR imaging, as in HD. We report herein the cases of 2 siblings with neuroacanthocytosis showing cerebellar atrophy as well as atrophy and signal intensity changes of striata.

Movement and extrapyramidal disorders associated with interferon use in HIV/hepatitis C coinfection

Movement and extrapyramidal disorders associated with interferon use in HIV/hepatitis C coinfection