Non-ketotic hyperglycaemic chorea: a SPECT study.

OBJECTIVE: To study regional blood flow of the striatum in non-ketotic hyperglycaemic choreic patients. METHODS: Brain SPECT was performed with intravenous injection of 20 mCi 99mTc hexamethylpropylene amineoxime in six non-ketotic hyperglycaemic choreic patients and 10 age matched patients with a similar level of hyperglycaemia without chorea as a control. The focal perfusion defects were analysed by visual interpretation and semiquantitative determination with reference to homolateral occipital blood flow. RESULTS: The measured blood flow of striatum on the contralateral side of chorea was significantly decreased. CONCLUSIONS: Hypometabolism of the striatum is seen not only in Huntington's disease, but also in non-ketotic hyperglycaemic chorea. Hypofunction of the striatum is a possible common pathogenesis in the development of contralateral chorea in different diseases. Furthermore, the sensitivity and reliability of SPECT is as good as PET in assessing choreic patients.     

Putaminal petechial haemorrhage as the cause of chorea: a neuroimaging study

OBJECTIVES—A hyperintense putamen on either CT orMRI as a finding associated with chorea has occasionally been describedand is almost always associated with non-ketotic hyperglycaemia. Thecause of the hyperintensity of the striatum in these images is stillcontroversial. Some reports have found that calcification wasresponsible whereas others have advocated petechial haemorrhage as thecause. The purpose of this study was to determine whether hyperintensestriata are caused by petechial haemorrhage or calcification, with the sequential imaging changes.
SUBJECTS AND METHODS—Five patients presentingwith an acute onset of either hemichorea or generalised chorea andshowed either unilateral or bilateral hyperdense striatum on theinitial CT were assessed. Neuroimaging studies including sequential CTand MRI examinations and detailed biochemical tests were performed.
RESULTS—Three patients had pronouncedhyperglycaemia and the other two patients had no biochemicalabnormalities. In all patients, the first CT scans, performed withintwo weeks of the onset of chorea, showed a high density over thestriatum contralateral to the chorea, which diminished or disappearedtwo months later. T1 weighted imaging disclosed hypersignal intensitiesover the striatum contralateral to the chorea on admission whichdiminished two months later. T2 weighted imaging at two months showedhyposignal intensity changes corresponding to the area with hypersignalchanges on T1 weighted images, implying haemosiderin deposition.
CONCLUSION—Based on the evolution of clinicalmanifestations and the findings of neuroimaging, putaminal petechialhaemorrhage might be a new entity causing either hemichorea orgeneralised chorea.

     

Evidence of thalamic disinhibition in patients with hemichorea: semiquantitative analysis using SPECT

OBJECTIVES: Hemichorea sometimes occurs after lesions that selectively involve the caudate nucleus, putamen, and globus pallidus. Some reports have hypothesised that the loss of subthalamic nucleus control on the internal segment of the globus pallidus, followed by the disinhibition of the thalamus may contribute to chorea. However, the pathophysiology is poorly understood. Therefore, clinicoradiological localisation was evaluated and a comparison of the haemodynamic status of the basal ganglia and thalamus was made. METHODS: Six patients presenting with acute onset of hemichorea were assessed. Neuroimaging studies, including MRI and SPECT examinations in addition to detailed biochemical tests, were performed. A semiquantitative analysis was performed by comparing the ratio of blood flow between patients and normal controls. In addition, the ratio of perfusion asymmetry was calculated as the ratio between each area contralateral to the chorea and that homolateral to the chorea. The comparison was made with a two sample t test. RESULTS: The causes of hemichorea found consisted of four cases of acute stroke, one non-ketotic hyperglycaemia, and one systemic lupus erythematosus. Brain MRI indicated lesion sites in the contralateral putamen, globus pallidus, caudate nucleus, and subthalamic nucleus. A significant decrease in the ratio of blood flow in the basal ganglia contralateral to the chorea and a significant increase in the thalamus was found when comparing the perfusion asymmetries, which were calculated as the ratio of cerebral blood flow (CBF) for each region to that in the homolateral occipital area (p<0.05). CONCLUSION: An alteration in CBF in both the contralateral thalamus and basal ganglia reflect the loss of pallidal inhibitory input from the pallidum to the thalamus. This change in CBF may be one of epiphenomena, which implicates an occurrence of hemichorea in humans.     

Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases

Background: Chorea associated with non-ketotic hyperglycemia and high signal intensity lesions on T1-weighted brain magnetic resonance images (C-H-BG) is recognized as a unique syndrome that affects elderly women exclusively. However, its overall clinical features are unclear. Material and Methods: The literature describing patients with C-H-BG from 1985 to 2001 was reviewed using MEDLINE. Their clinical features and those of four patients with C-H-BG at this hospital were analyzed. Results: This study included 49 patients from the literature and four patients at this hospital. Their mean age at the onset was 71.1 years (range=22–92 years). Women were affected more frequently than men (men/women=17:30). The mean serum glucose level measured after the onset of chorea was 481.5 mg/dl (ranging from 169 to 1264), HbA1c level was 14.4% (ranging from 9.9 to 19.2), and the serum osmolarity was 305.9 mmol/kg (ranging from 291 to 335). Forty-seven patients developed hemichorea. Six patients developed bilateral chorea, and magnetic resonance imaging (MRI) showed bilateral basal ganglia lesions. MRI showed that putamen was involved in all cases (isolated PUTAMEN=31 patients, additional basal ganglia LESIONS=22 patients). None had lesions confined to the caudate nucleus or the globus pallidus. In all, except one, the anterior limb of the internal capsule was spared. Follow-up MRI studies were performed in 22 patients. In most, hemichorea improved along with the disappearance of the lesions. In 39 patients, chorea had ameliorated completely. The remaining 14 cases showed some improvement during the follow-up period. The chorea recurred in seven patients. Conclusion: C-H-BG is a benign disorder affecting the elderly. It affects men much more frequently than has been reported. The high signal intensity basal ganglia lesion on the T1-weighted brain MRI study was reversible, and correlated with the clinical improvement in chorea.     

Hemiballism: report of 25 cases.

Twenty three patients with hemiballism and two with biballism were studied. Ischaemic and haemorrhagic strokes were the cause in most patients. Other causes were encephalitis, Sydenham's chorea, systemic lupus erythematosus, basal ganglia calcifications, non-ketotic hyperglycaemia, and tuberous sclerosis. Neuroimaging studies showed a lesion of the subthalamic nucleus in only six patients. In others, different subcortical structures were involved or the results were normal. Only two patients had "pure" hemiballism. The others had other types of dyskinesias, mainly chorea, which was present in 16 patients. The prognosis was usually good.     

非酮症性高血糖舞蹈症七例临床及神经影像学特点

目的 探讨非酮症性高血糖舞蹈症的临床及神经影像学特点.方法 对7例非酮症性高血糖舞蹈症患者进行临床及颅脑CT和MRI检查,分析其临床及影像学特征.结果 7例患者均有糖尿病病史,平素血糖控制不良,发病时血糖较高而酮体正常,表现为单侧肢体、双侧肢体或全身舞蹈样动作.颅脑CT和MRI可见单侧或双侧基底节区异常病灶.单纯药物控制舞蹈症效果不佳,降低血糖后舞蹈症状和神经影像改变可很快恢复,不留后遗症.结论 非酮症性高血糖舞蹈症多见于年龄较大的糖尿病患者,可能与大脑基底核在高血糖状况下脑细胞代谢出现异常有关.颅脑CT或MRI改变具有特征性.本病是可逆性的,对治疗反应较好,一般不留后遗症.     

非酮症高血糖性舞蹈症

目的对非酮症高血糖性舞蹈症患者的临床表现、影像学特征及发病机制进行分析。方法结合相关文献对5例非酮症高血糖舞蹈症患者的临床资料进行分析。结果 5例均急性起病,可表现为单侧或双侧肢体的舞蹈症状;血糖和血渗透压明显增高;影像学表现为特异性斑片状脑出血和纹状体T1高信号;联合应用降糖药物和多巴胺受体阻滞剂可控制舞蹈症状。结论非酮症性高血糖、舞蹈症以及MRI显示为纹状体T1高信号,可能构成舞蹈症临床综合征;影像学改变可能表示由代谢紊乱引起的斑片状脑出血,后者导致基底神经节运动环路受损,从而产生舞蹈样症状。     

Generalized chorea associated with bilateral chronic subdural hematoma

A variety of acquired disorders cause chorea, such as hypoxia-ischemia injury, drugs, toxins, infections, autoimmune disorders, and endocrine and electrolyte abnormalities, but rarely subdural hematoma. We report the case of a 76-year-old woman who presented with sudden onset of generalized choreiform movements 1 day prior to admission. Computed tomography revealed a bilateral lateral convexity subdural effusion, which was later confirmed by magnetic resonance imaging as a chronic subdural hematoma. After surgical evacuation of the subdural hematoma, the choreoathetoid movements ceased completely. We propose that transient ischemia in the bilateral subcortical regions was from compression and distortion of the brain by the bilateral hematomas and resulted in generalized chorea.     

Hemiballism-hemichorea and non-ketotic hyperglycaemia.

Three patients with hemiballism-hemichorea caused by non-ketotic hyperglycaemia are presented, two of whom had hyperosmolar non-ketotic hyperglycaemic syndrome. In two of the three patients, the hyperkinesia was the initial presenting symptom of their diabetes mellitus. The hypersensitivity of the postmenopausal dopamine receptor, decreased gamma-aminobutyric acid in the brain in non-ketotic hyperglycaemia, coexisting lacunar infarct in the basal ganglion, and pre-existing metabolic dysfunction in the basal ganglion may all have played a part in the pathogenesis of this movement disorder.     

Dystonia-predominant adult-onset Huntington disease: association between motor phenotype and age of onset in adults

BACKGROUND: In juvenile Huntington disease (HD), dystonia as well as parkinsonism and eye movement abnormalities may be the predominant motor signs rather than chorea. Several patients have come to our attention with adult-onset HD in whom there is prominent dystonia and minimal chorea (ie, an adult-onset form of HD that resembles juvenile HD). OBJECTIVES: To estimate the prevalence of these cases of dystonia-predominant HD in a clinic and to study the relationship between the motor phenotype and age of onset in HD. METHODS: The Unified Huntington's Disease Rating Scale (UHDRS) was administered to 127 subjects during their initial visit to the Huntington's Disease Center at the New York State Psychiatric Institute, where dystonia, chorea, bradykinesia, rigidity, and eye movements were rated. The dystonia score was the mean UHDRS rating of dystonia in 5 body regions; the chorea score, the mean rating of chorea in 7 regions; the bradykinesia score, the mean rating of axial and limb bradykinesia; the rigidity score, the mean rating of rigidity in both arms; and the eye movement score, the mean rating of ocular pursuit, saccade initiation, and velocity. Dystonia-predominant HD was defined by the severity of dystonia relative to the severity of chorea. RESULTS: Fifteen (11.8%) of 127 subjects had dystonia-predominant HD. Age of onset correlated negatively (r= -0. 22, P=.02) with the dystonia score divided by the chorea score and negatively (r= -0.28, P=.002) with the severity of dystonia, bradykinesia, and eye movement abnormalities relative to chorea (ie, [(dystonia score + bradykinesia score + eye movement score)/3] - chorea score), suggesting that subjects with younger ages of onset had more severe dystonia, bradykinesia, and eye movement abnormalities relative to chorea. CONCLUSIONS: Cases of adult-onset HD with prominent dystonia and a paucity of chorea may represent 1 in 8 cases in specialty clinics. Age of onset was clearly associated with the motor phenotype. A younger age of onset was associated with more severe dystonia, bradykinesia, and eye movement abnormalities relative to chorea, supporting the notion that in adult-onset HD, the motor phenotype forms a continuum with respect to age of onset.     

Neonatal non-ketotic hyperglycinemia

The typical imaging findings of neonatal non-ketotic hyperglycinemia have rarely been described in the radiologic literature with only few individual cases or small series reported. In this article, we present a case of neonatal onset non-ketotic hyperglycinemia, imaged at 6 days of age, and discuss characteristic MRI and MR spectroscopic findings.     

Paraneoplastic chorea with leukoencephalopathy presenting with obsessive-compulsive and behavioral disorder.

Chorea is a rare manifestation of paraneoplastic disease and is associated with CV2/CRMP-5 antibodies. Obsessive-compulsive disorder and large-scale white matter abnormalities on MRI have not been previously reported in association with these antibodies. We report on a case of CV2 paraneoplastic syndrome with obsessive-compulsive behavior preceding the motor manifestations of chorea with associated leukoencephalopathy on MRI. The literature on paraneoplastic chorea is reviewed.     

Hemichorea associated with polycythaemia vera

Abstract Chorea is a rare complication of polycythaemia vera. Polycythaemic chorea occurs predominantly in females and usually in generalised form. We present a 66-year-old woman with acute onset hemichorea-ballism with no vascular pathology in the basal ganglia region. A clear relationship was observed between the onset of chorea and worsening of haematological parameters in the patient. After repeated phlebotomies the patient's clinical status was improved. Polycythaemic chorea must be considered, especially in the elderly, as early diagnosis leads to effective treatment and prevention of complications.     

非酮症高血糖性舞蹈病

非酮症高血糖性舞蹈病是一特殊的综合征,多见于老年女性糖尿病患者,急性起病,可表现为单侧或双侧肢体的舞蹈症状;血糖和血渗透压增高;联合应用降糖药物和多巴胺受体阻滞剂可控制舞蹈症状;其影像学具有特征性改变,颅脑MRIT1相表现为单侧或双侧基底节区高信号,随着病情的好转,高信号病灶可消失。     

Non-ketotic hyperglycemia in a young woman, presenting as hemiballism-hemichorea

We report a 22-year-old girl presenting with acute onset left sided hemiballism-hemichorea (HH) and non-ketotic hyperglycemia (NKH). Initial brain CT revealed faint hyperdensities, sharply confined to the contralateral nucleus caudatus and putamen. Sequential MRI investigations yielded increasing hypersignal intensities on T1-weighted images and resolving hypodensities on T2-weighted images of the right striatum, leaving small sequelae in the head of the right caudate nucleus. NKH is an unusual cause of HH. The abnormalities seen in neuroimaging are rare, but seem to be quite specific to this syndrome. We give an update on current literature regarding the possible pathophysiological processes underlying this specific clinical entity.     

Progressive MRI abnormalities in late recurrence of Sydenham's chorea

We report four cases of adult recurrence of Sydenham's chorea (SC). The first episodes all followed childhood tonsillopharyngitis. Many years later, subsequent episodes appeared after the triggering circumstances: throat or cutaneous infection, pregnancy, childbirth, contraceptive treatment or stress. Other inflammatory diseases were ruled out and streptococcal serology was weakly positive. Magnetic Resonance Imaging (MRI) revealed focal areas of T2 hypersignal in the caudate nucleus, the pallidum, the putamen and the white matter. One year later, repeat MRI variously gave evidence of the persistence, disappearance or even new appearance of abnormalities. Specific features of SC recurrence in the adult may include: a personal history of chorea after a throat infection during childhood; a second episode of isolated chorea with or without slight neuropsychological disorders; streptococcal serology weakly positive; focal hypersignals involving the basal ganglia; the triggering circumstances. Since SC is considered to be an autoimmune disease, the progressive MRI abnormalities suggest that certain circumstances may trigger the reactivation of persistent immune disorders.     

非酮症高血糖合并舞蹈-投掷运动的临床特点

目的 探讨非酮症性高血糖合并舞蹈-投掷动作的临床特征.方法 回顾性分析8例伴有舞蹈一投掷动作的非酮症性高血糖患者的临床资料.结果 8例患者均为急性发病,舞蹈-投掷动作累及单侧或双侧肢体;发病时血糖水平明显增高(13.2～26 mmol/L),所有患者血浆酮体检测呈阴性;头部CT和MRI显示与临床症状相对应基底节区异常...     

Hyperglycemic hemianopia: a reversible complication of non-ketotic hyperglycemia

Four patients each developed a reversible homonymous hemianopia caused by non-ketotic hyperglycemia. In two patients the homonymous hemianopia was the first manifestation of diabetes mellitus type 2. All four patients had abnormal MRI scans; in the three patients who had late follow-up scans the abnormalities resolved completely. In one patient the progressive visual field defect and unusual MRI findings initially caused concern for a tumor.     

Chorea due to nonketotic hyperglycemia

A 62 year old diabetic and hypertensive male presented with sudden onset generalized chorea. Investigations revealed uncontrolled diabetes with absent ketones and normal serum osmolality. Achievement of euglycemia with insulin therapy abolished the involuntary movements completely within a day. The direct effect of hyperglycemia causing striatal neuronal dysfunction could be the pathogenesis of the chorea in our patient.     

Lower limb monochorea from a globus pallidus infarct

Movement disorders are common following cerebrovascular accidents and they can be hyperkinetic, including hemichorea and hemiballismus, or hypokinetic, as seen in parkinsonian disorders. Monochorea has also been reported due to stroke, albeit rarely. We report a 47-year-old gentleman who presented with a history of sudden onset choreiform movement of his left lower limb. On clinical examination his motor power was normal and there were no abnormal movements in any other limb. MRI of his brain was suggestive of an infarct in the right globus pallidus interna extending up to the posterior limb of the internal capsule. He was treated with clonazepam and trihexyphenidyl. His movements improved significantly within 3 months. Monochorea in a lower limb due to an infarct in the globus pallidus interna is unusual and highlights the complexity of the pathophysiology of chorea.