MyMedR

Idiopathic paroxysmal kinesigenic dyskinesia in Malaysia, a multi-racial Southeast Asian country

Tai ML, Lim SY, Tan CT

J Clin Neurosci, 2010 Aug;17(8):1089-90.

PMID: 20542699 DOI: 10.1016/j.jocn.2009.11.034

Paroxysmal kinesigenic dyskinesia is a rare disorder, and there are few reports of Asian patients with this condition. We reviewed the clinical features of all patients with idiopathic paroxysmal kinesigenic dyskinesia (PKD) seen at a major neurological centre in Malaysia. The charts of 11 patients with idiopathic PKD seen between 1995 and 2008 were reviewed retrospectively. The male:female ratio was 9:2. Ten patients were of Chinese ethnicity, and one was Malay. Three patients (from two families) had a family history of PKD. The involuntary movement was dystonia in 73% of patients. In one patient, attacks were precipitated by vestibular stimulation. One patient had generalized epilepsy. Another patient who did not have epilepsy demonstrated epileptiform discharges. Only slightly over one-quarter of patients had a positive family history. Males, and people of Chinese ancestry, seem to be affected more frequently by PKD in certain Asian populations.

Matched MeSH terms: Chorea/diagnosis*; Chorea/physiopathology*

Idiopathic paroxysmal kinesigenic choreoathetosis: precipitation of attacks by vestibular stimulation

Chew NK, Tan CT, Goh KJ

J Clin Neurosci, 2002 Sep;9(5):604-5.

PMID: 12383430

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.

Matched MeSH terms: Chorea/drug therapy; Chorea/etiology*; Chorea/pathology

Fulltext Hyperglycemia-induced involuntary movements: what you should know

Tan, T.L., Mohd Fazrul, M.

Medicine & Health, 2018;13(1):237-242.

MyJurnal

Hemichorea-hemiballismus (HC-HB) is a movement disorder characterized by involuntary movements involving limbs on one side of the body. Many etiological factors were identified and these included stroke, infection, and neoplasm. However, acute hyperglycemia-induced HC-HB is less well known. We present two cases of non-ketotic hyperglycemia-induced HC-HB. The cases depicted here Illustrate that HC-HB can be the sole presentation from among a variety of neurological manifestations of poorly controlled diabetes which can be easily reversed when hyperglycemia is corrected.

Matched MeSH terms: Chorea

Malaysian siblings with friedreich ataxia and chorea: a novel deletion in the frataxin gene

Spacey SD, Szczygielski BI, Young SP, Hukin J, Selby K, Snutch TP

Can J Neurol Sci, 2004 Aug;31(3):383-6.

PMID: 15376485

BACKGROUND: Friedrich ataxia (FRDA1) is most often the result of a homozygous GAA repeat expansion in the first intron of the frataxin gene (FRDA gene). This condition is seen in individuals of European, North African, Middle Eastern and Indian descent and has not been reported in Southeast Asian populations. Approximately 4% of FRDA1 patients are compound heterozygotes. These patients have a GAA expansion on one allele and a point mutation on the other and have been reported to have an atypical phenotype.

OBJECTIVE: To describe a novel dinucleotide deletion in the FRDA gene in two Malaysian siblings with FRDA1.

SETTING: Tertiary referral university hospital setting.

PATIENTS AND METHODS: A previously healthy 10-year-old Malaysian boy, presented with fever, lethargy, headaches, dysarthria, dysphagia, vertigo and ataxia which developed over a one week period. His neurological exam revealed evidence of dysarthria and ataxia, mild generalized weakness and choreoform movements of the tongue and hands. His reflexes were absent and Babinski sign was present bilaterally. A nine-year-old sister was found to have mild ataxia but was otherwise neurologically intact.

RESULTS: Molecular genetic studies demonstrated that both siblings were compound heterozygotes with a GAA expansion on one allele and a novel dinucleotide deletion on the other allele.

CONCLUSIONS: We describe a novel dinucleotide deletion in the first exon of the FRDA gene in two siblings with FRDA1. Additionally this is the first report of FRDA1 occurring in a family of southeast Asian descent, it demonstrates intrafamilial phenotypic variability, and confirms that atypical phenotypes are associated with compound heterozygosity.

Matched MeSH terms: Chorea/genetics*

Fulltext Paroxysmal dyskinesia as an unusual and only presentation of subcortical white matter ischaemia: a report of two cases

Norlinah MI, Shahizon AM

Med J Malaysia, 2008 Dec;63(5):410-2.

PMID: 19803303 MyJurnal

Secondary paroxysmal dyskinesias (PxD) have been previously reported in patients with multiple sclerosis, lacunar infarcts, head trauma, metabolic disorders such as hyperglycaemia, hypocalcaemia, migraine and central nervous system (CNS) infections. The causative lesions typically involve the basal ganglia structures, medulla and rarely the spinal cord. We report two patients who presented with paroxysmal dyskinesias as the only manifestation of subcortical white-matter ischaemia. Patient 1 presented with 3-year history of paroxysmal kinesigenic dyskinesia (PKD) and patient 2 with 6-month history of paroxysmal nonkinesigenic dyskinesia (PNKD). All investigations, including CSF oligoclonal bands were negative, except for a brain MRI which showed multiple, non-enhancing subcortical white matter lacunar infarcts. Therefore, subcortical white matter ischaemia should also be included in the differential diagnosis of PxD.

Matched MeSH terms: Chorea/diagnosis*; Chorea/drug therapy; Chorea/etiology*

Fulltext A case of hyperosmolar hyperglycaemic state with involuntary movements - diagnostic dilemma and clinical considerations

Abd Hamid H, Umar NA, Othman H, Das S

Arch Med Sci, 2010 Dec;6(6):987-90.

PMID: 22427779 DOI: 10.5114/aoms.2010.19315

Hyperosmolar hyperglycaemic state (HHS) is a medical emergency which needs immediate medical intervention. A 37-year-old Chinese woman with a history of hypertension attended the Emergency Department. She had a two-day history of involuntary movement, i.e. chorea of the upper limbs, preceded by a one-week history of upper respiratory tract infection. She also had polyuria and polydipsia, although she was never diagnosed as diabetic. The main aim of reporting the present case was to highlight the importance of biochemical investigations involved in the diagnosis of involuntary movements.
Study site: emergency department, Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM)

Matched MeSH terms: Chorea

Fulltext Hemiballismus in Uncontrolled Diabetes Mellitus

Jaafar J, Rahman RA, Draman N, Yunus NA

Korean J Fam Med, 2018 May;39(3):200-203.

PMID: 29788710 DOI: 10.4082/kjfm.2018.39.3.200

Hemiballismus, a subtype of chorea, is a rare movement disorder, and is most commonly found secondary to stroke. Movements are involuntary, violent, coarse, and have a wide amplitude. There is increasing report of hemiballismus occurring in non-ketotic hyperglycemia. Spontaneous improvements or remissions were observed in many patients, and treatment should be directed towards the cause of hemiballismus. There is no randomized control trial to guide clinicians in deciding the best treatment option when managing hemiballismus. Symptomatic treatment includes the use of drugs such as dopamine receptor blocker and tetrabenazine. Surgical treatment is reserved for severe, persistent, and disabling hemiballismus. This case is of an elderly woman with long standing uncontrolled diabetes who presented with abnormal movement in her left upper limb for 2 months, which resolved slowly with good control of her glucose levels. Treating physicians need to have a high index of suspicion to prevent mismanagement of the condition.

Matched MeSH terms: Chorea

Fulltext Diabetic non-ketotic hyperglycaemia presenting as chorea--a case report

Lim TO, Ngah BC

Med J Malaysia, 1990 Sep;45(3):260-2.

PMID: 2152091

We report a patient with hyperosmolar non-ketotic hyperglycaemia who presented with chorea and septic arthritis on his knee. The chorea resolved completely and quickly with correction of the metabolic disturbance, only to return just as quickly when his metabolic disturbance subsequently deteriorated as a result of overwhelming septicaemia, suggesting coexisting cerebral ischaemia, although the basis of focal neurological sign in non-ketotic hyperglycaemia remains controversial.

Matched MeSH terms: Chorea/etiology*

Osmotic Demyelination Syndrome With Evolving Movement Disorders

Tan AH, Lim SY, Ng RX

JAMA Neurol, 2018 07 01;75(7):888-889.

PMID: 29799978 DOI: 10.1001/jamaneurol.2018.0983

Matched MeSH terms: Chorea/etiology*

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