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An Unusual Cause of Involuntary Movements
Varuna Prakash, MD MHSc PGY-2, Internal Medicine Stephen Hwang, MD MPH Staff Physician, Division of General Internal Medicine
Disclosures & Conflicts of Interest
None
An Unusual Cause of Involuntary Movements Varuna Prakash, MD MHSc - - PowerPoint PPT Presentation
An Unusual Cause of Involuntary Movements Varuna Prakash, MD MHSc - - PowerPoint PPT Presentation
An Unusual Cause of Involuntary Movements Varuna Prakash, MD MHSc PGY-2, Internal Medicine Stephen Hwang, MD MPH Staff Physician, Division of General Internal Medicine Disclosures & Conflicts of Interest None The Patient ID: 64 y/o from
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The Patient
ID: 64 y/o from home alone PMHx:
- T2DM (CABG x 4, L corona radiata CVA, L 4th toe amputation, retinopathy,
nephropathy, neuropathy). Last A1C = 10.6 (7 years ago)
- Dyslipidemia
- CHF-HFpEF
- 20 p/y smoking hx
Medications: Stopped all Rx meds including insulin 1 year ago. Now using turmeric, apple cider vinegar, ginger. Functional Status: Previously independent for all ADLs, iADLs
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Presenting Concern
- Fell near Tim Hortons 2 days ago, no LOC, unclear why he fell
- Sudden onset of involuntary flinging movements of L arm and leg x 2 days
- Now also some uncontrolled tongue movements and mild slurred speech
(noticed by sister)
- No mental status changes, headaches, facial droop, dysphagia, limb weakness,
bowel/bladder dysfunction.
- Cardio/Resp/GI/GU/Derm/Constitutional/Exposure RoS negative
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On Examination
- Vitals: BP 177/74, remainder of VS WNL
- Mental Status/Cognition: Normal
- CV/Resp/Abdo/Derm: Unremarkable
- Neuro: Very abnormal…
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On Examination
(with consent)
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DDx – Involuntary Movements
Image adapted from DeLong MR, Wichmann T. Circuits and Circuit Disorders of the Basal Ganglia. Arch Neurol. 2007 Jan 1;64(1):20.
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DDx – Involuntary Movements
- Vascular: Basal ganglia stroke
- Drug or Toxin Induced: Amphetamines,
Stimulants, Antipsychotics (TDs)
- Infectious: Sydenham’s chorea (GAS), cerebral
toxoplasmosis
- Autoimmune: SLE (APLA)
- Endocrine/Metabolic: Hyperglycemia, various
electrolyte dx, chorea gravidarum
- Neoplastic/Paraneoplastic: SCC, anti-Hu/Ri/Yo
- Neurodegenerative, Demyelinating:
Huntington’s Disease
Image adapted from DeLong MR, Wichmann T. Circuits and Circuit Disorders of the Basal Ganglia. Arch Neurol. 2007 Jan 1;64(1):20.
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Initial Investigations
112
MCV 76
6.7 261 133 4.2 91 20 122 17 7.34 / 30 / 17 Lactate 1.7 Ketones +++ Sosm 295 Troponin 0.006 Tox negative
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Initial Investigations
CT Head: “Multiple old lacunar infarcts. Hyperdensity in the R corpus striatum may be due to underlying hemorrhage or calcium deposition. Consideration of hyperglycemic hemichorea. Is the patient diabetic?”
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Non-ketotic Hyperglycemic Hemiballismus-Hemichorea
- Also known as Chorea, Hyperglycemia, Basal Ganglia Syndrome (C-H-BG)
- Epidemiology:
- Rare – largest review is of 53 cases1
- Described most often in older (~71 years), female (W30:M17), Asian patients1
- Mean BG: 26
- Mean HbA1C: 14.4
- Proposed Pathophysiology: Poorly understood. ?Hyperviscosity/BBB disruption
- Diagnosis: MRI shows T1 hyperintensity in striatum, +/- T2 hypointensity
- Treatment: Correction of hyperglycemia
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Workup & Management
- Insulin infusion until Anion Gap closed
- MRI confirmed T1-increased signal within R
lentiform nucleus and caudate, T2-hyposignal in same area, SWI normal, no acute infarct.
- Symptoms resolved!
- Discharged home with Metformin, premixed
Insulin
- Guess the A1C?
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After
(with consent)
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3 Learning Points
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Thank you!
varuna.prakash@utoronto.ca
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References
1. Ondo WG. Hyperglycemic nonketotic states and other metabolic imbalances. In: Handbook of Clinical Neurology [Internet]. Elsevier; 2011 [cited 2018 May 18]. p. 287–91. Available from: http://linkinghub.elsevier.com/retrieve/pii/B9780444520142000215. 2. Narayanan S. Hyperglycemia-Induced Hemiballismus Hemichorea: A Case Report and Brief Review of the Literature. J Emerg Med. 2012 Sep;43(3):442–4. 3. Hansford BG, Albert D, Yang E. Classic neuroimaging findings of nonketotic hyperglycemia on computed tomography and magnetic resonance imaging with absence of typical movement disorder symptoms (hemichorea-hemiballism). J Radiol Case Rep. 2013 Aug;7(8):1–9. 4. Bizet J, Cooper CJ, Quansah R, Rodriguez E, Teleb M, Hernandez GT. Chorea, Hyperglycemia, Basal Ganglia Syndrome (C-H-BG) in an uncontrolled diabetic patient with normal glucose levels on presentation. Am J Case Rep. 2014;15:143– 6. 5. Cosentino C, Torres L, Nuñez Y, Suarez R, Velez M, Flores M. Hemichorea/Hemiballism Associated with Hyperglycemia: Report of 20 Cases. Tremor Hyperkinetic Mov N Y N. 2016;6:402.