causes . For example , Huntington ’ s disease ( HD ) is the most common genetic cause of chorea , but generally has a gradual development of symptoms . NHH and other acquired forms of chorea are far more likely to present in a subacute or acute fashion . The most common cause of acute hemichorea is an ischemic or hemorrhagic stroke , which must be considered when patients present with severe hypertension such as this case . However , less than 1 % of strokes result in hemichorea . 11 The key distinguishing feature of NHH is the presence of hyperglycemic crisis , with the absence of ketones . Table 2 outlines the key distinguishing features of NHH , HD , and stroke . As seen in this patient , remission of symptoms occurs after improvement in glycemic control .
CONCLUSION
Diabetes mellitus is an epidemic in WV . Currently , over 15 % of adults in the state are diagnosed with T2DM , with another 11 % having pre-diabetes . 2 Similar presentations of chorea may be more prevalent in WV than other states when considering the higher rates of T2DM but may be underappreciated given the patient demographics . Additionally , patients may present with mild tremors , similar to the onset of symptoms in this case , but be treated for their hyperglycemic hyperosmolar state before the neurological symptoms are noted . All patients with acute onset of hemichorea should be screened for hyperglycemia , even with no history of diabetes . Physicians must assess the barriers to healthcare of their patients with T2DM in an effort to prevent progression to NHH due to lack of access to proper medical treatment .
REFERENCES
1 . National Diabetes Statistics Report 2020 . Estimates of diabetes and its burden in the United States . 2021 .
2 . Shanholtzer B . West Virginia Behavioral Risk Factor Surveillance System Report , 2016 . 2018 .
Autoimmune or Inflammatory Celiac disease Hashimoto encephalopathy Polyarteritis nodosa Sarcoidosis Sjögren syndrome Sydenham chorea Systemic lupus erythematosus Cerebrovascular Arteriovenous malformation Ischemic stroke
TABLE 1 : Causes of Acquired Hemichorea Metabolic or Endocrine Hepatic failure Hyperthyroidism Hypo / hypercalcemia Hypo / hyperglycemia Hypo / hypernatremia Hypomagnesemia Hypoparathyroidism Polycythemia vera Pseudohypoparathyroidism Renal failure Subarachnoid hemorrhage Vitamin deficiency ( B1 , B12 , niacin ) Infection Encephalitis Lyme disease Meningitis Progressive multifocal leukoencephalopathy Toxoplasmosis Tuberculous meningitis
Disease
Genetic Predisposition
CT Imaging
Toxic Alcohol intoxication or withdrawal Carbon monoxide Manganese Mercury Thallium Toluene
TABLE 2 : Comparison of Underlying Causes of Hemichorea
Nonketotic Hyperglycemic Hemichorea
None
Hyper-density in striatum
Huntington ’ s Disease
Autosomal Dominant ; CAG repeat
Caudate nucleus atrophy
None
Stroke
Alterations to lentiform nuclei
Onset Acute , Rapid Insidious Acute , Rapid
Mental Status
Comorbidities
Symptom Development
Unaltered
Progressive psychiatric symptoms
Variable depending on severity
Obesity , T2DM |
Weight loss |
Hypertension , |
|
|
Atherosclerosis , |
|
|
Dyslipidemias |
Remission with glycemic control
Continual worsening of chorea
Variable remission following injury
CT : computed tomography ; T2DM : Type II diabetes mellitus ; CAG : cytosine , adenine , guanine
West Virginia Medical Journal • September 2023 • 19