Abnormalities of neurotransmitter enzymes in Huntington's chorea

AbstractStudy ObjectiveWhile monoballismus has been reported to be associated with hemorrhagic lesions in the subthalamic nucleus (Ohnishi, 2009) and multiple sclerosis (MS) (Rosa, 2011), the duration has been reported to be at least six days (Soysal, 2012). A patient with epochs of monoballismus lasting for 45 minutes is presented.MethodsCase Study: A 57 year old right handed female with attention deficit hereditary disorder predominantly inattentive on amphetamine sulphate, presented with two years of memory loss. For instance, after ordering food in restaurants, by the time the food arrives, she could not recall what she ordered. At the onset of this symptom, she noted three epochs of her left arm jerking for 45 minutes. The jerking would begin with low amplitude and low frequency and rapidly progress to the forearm and arm of greater magnitude and low frequency. With her right hand she would try to hold down her left arm without success. There was no associated paresis, sensory phenomena, headaches, dizziness, presyncope, loss of consciousness, or strong emotions. She admitted to frequent jamais vu.ResultsAbnormalities: Neurological Examination: Mental Status Examination: Memory: Immediate Recall: 5 digits forward and 2 digits backwards. Cranial Nerve (CN) Examination: CN I: Alcohol Sniff Test 8 (hyposmia). CN XII: tongue tremor on protrusion. Motor Examination: Drift Test: positive right pronator drift. Gait Examination: Tandem Gait: unstable. Reflexes: 0-1 throughout. Neuropsychiatric Examination: Go-No-Go Test: 6/6 (normal). Animal Fluency Test: 15 (normal). Clock Drawing Test: 3 (abnormal). Center for Neurologic Study Lability Scale: 16 (pseudobulbar affect). Other: MRI with and without infusion: normal.ConclusionTransient tonic-clonic movements of one limb have been described with focal epilepsy associated with diabetic non-ketotic hyperglycemia (Grant, 1985). A metabolic abnormality such as transient hypoglycemia or hyperkalemia can cause a focal dystonia (Soysal, 2012), which theoretically could manifest with monoballismus. This could be a somatic manifestation of underlying conflict, conversion disorder, or as a result of a physical manifestation of panic attack with hyperventilation and tetany (Mihai, 2008). This may be the first manifestation of a generalized cerebral disorder associated with chorea or ballismus such as Wilson’s disease, or Huntington’s Chorea (Mihai, 2008). It is possible that this is a variant of Alien Hand Syndrome with parietal lobe involvement (Shrestha, 2015). But this is unlikely given the absence of hemineglect or hemiagnosia. It is possible that amphetamines may have induced a monochorea. Chronic amphetamine use has been demonstrated to cause chorea (Klawans, 1974) and it theoretically could have caused ballismus movements in this case. In patients who present with short duration monoballismus, evaluation for subthalamic nuclei function, seizure disorders and other origins of ballismus are warranted.Funding AcknowledgementsSmell & Taste Treatment and Research Foundation

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An experimental model for Huntington's chorea?

Behavioural Brain Research ◽

10.1016/j.bbr.2013.12.036 ◽

2014 ◽

Vol 262 ◽

pp. 31-34 ◽

Cited By ~ 5

Author(s):

Dagmar H. Zeef ◽

Ali Jahanshahi ◽

Rinske Vlamings ◽

João Casaca-Carreira ◽

Remco G. Santegoeds ◽

...

Keyword(s):

Experimental Model ◽

Huntington's Chorea

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Verbal and Spatial Memory Span in Patients with Extrapyramidal Diseases

Perceptual and Motor Skills ◽

10.2466/pms.1987.65.2.555 ◽

1987 ◽

Vol 65 (2) ◽

pp. 555-558 ◽

Cited By ~ 8

Author(s):

A. Orsini ◽

N. A. Fragassi ◽

L. Chiacchio ◽

A. M. Falanga ◽

C. Cocchiaro ◽

...

Keyword(s):

Spatial Memory ◽

Progressive Supranuclear Palsy ◽

Memory Span ◽

Normal Subjects ◽

Huntington's Chorea ◽

Spatial Span

Spatial span (Corsi's block-tapping test) and verbal span (Wechsler's Digits Forward test) were measured in 651 normal subjects and in three groups of extrapyramidal patients (Progressive supranuclear palsy, Parkinson, and Huntington's Chorea). Analysis showed Huntington's Chorea patients scored lower on both tests than did controls and other groups.

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