Ali Mahfuz, MBA, MD
Resident Physician
Northwell Health - Staten Island University Hospital
New Hyde Parl, New York, United States
Nikita Desai, DO
Resident Physician
SUNY Downstate Health Sciences University
Brooklyn, New York, United States
Verda Mirza, DO
Resident Physician
SUNY Downstate Health Science University
Brooklyn, New York, United States
Jack Mensch, MD
Attending Physician
Northwell Health - Staten Island University Hospital
Staten Island, New York, United States
A 66-year-old female with no significant past medical history presented to the emergency department with acute-onset left-sided upper extremity and facial movement abnormalities. Prior to admission, the patient reported dry mouth, polydipsia, polyuria, recurrent dizziness, and unintentional weight loss of approximately 100 pounds over several months.
Upon admission, laboratory evaluation revealed severe hyperglycemia with a glucose level of 545 mg/dL, anion gap of 27, and A1c of 10.4%, indicative of poorly controlled diabetes. A brain MRI demonstrated an acute infarct in the left genu of the corpus callosum and an intrinsic T1 hyperintense signal in the right lentiform nucleus, suggestive of a metabolic abnormality, likely non-ketotic hyperglycemic hemichorea.
The patient was diagnosed with DKA and an acute ischemic stroke in the ACA. She was admitted to inpatient rehabilitation for right lower extremity monoparesis, gait impairment, encephalopathy, and left upper extremity fine motor impairment secondary to non-ketototic hyperglycemia hemichorea (NKHH).
Discussions:
NKHH is a rare, hyperglycemia-induced movement disorder, often presenting with unilateral involuntary movements such as chorea or hemiballismus typically in patients with poorly controlled diabetes. In this case, the MRI revealed a hyperintense T1 signal in the right lentiform nucleus, a classic imaging finding in NKHH. This abnormality is thought to result from metabolic disturbances affecting the basal ganglia, particularly the putamen, due to hyperglycemia. NKHH often resolves with glucose normalization, although the recovery of motor function can be variable and may require a combination of medical management and rehabilitative therapy. In this patient, management of hyperglycemia led to improvement in hemichorea, although fine motor impairment persisted, necessitating inpatient rehabilitation.
Conclusions: Early recognition and treatment of NKHH are critical to prevent long-term disability and neurological sequelae.
