Case History:
A 14-year-old boy was transferred to a tertiary children’s hospital for
evaluation and treatment of abnormal movements and brain imaging
findings that were initially concerning for a brain tumor. He complained
of progressive involuntary movements of the left upper and lower
extremities for the past week. The movements did not involve the right
side of the body, resolved during sleep, and were noted to be worsening.
He reported that the movements were not painful but were very bothersome
and were not associated with any numbness or tingling sensation. Three
weeks prior to presentation, he presented to the emergency room of an
outside hospital with shortness of breath, weight loss, polyuria,
polydipsia, and polyphagia. Evaluation showed severe DKA where he had a
pH of 6.89, bicarbonate 4 mmol/L, anion gap 22, and glucose 662 mg/dL
(36.8 mmol/L). He was started on an insulin drip and transferred to
another outside hospital for admission and management of new onset T1D,
the official records of which were unavailable. However, he reported
being admitted for 3 days during which he received subcutaneous insulin
injections and he and his mom received diabetes education. After
discharge, he reported having poor glycemic control at home. His past
medical history was otherwise negative, and he had no previous
surgeries. Regarding his birth history, he was born at term via normal
vaginal delivery without any perinatal complications. He lived with his
mom at home, his biological father was not involved, and he had no
siblings. The family received support mainly from maternal relatives. He
was in 9th grade and was active in playing basketball
which he recently stopped because of the movements. He also complained
of some polyuria and polydipsia, otherwise the remainder of his review
of systems was negative. His vital signs were within normal ranges with
a temperature: 37.1°C, blood pressure: 121/71 mmHg, heart rate: 78 bpm,
respiratory rate: 15 br/min, and oxygen saturation of 97%. On physical
exam, his weight was 64.9 Kgs (80.74 %ile), his height was 172 cm
(67.59 %ile) and his BMI was 23.7 kg/m2 (87.12 %ile). He was alert and
oriented and exam was significant for choreiform movements in the left
upper and lower extremities without associated muscle weakness.
Otherwise, the remainder of his neurologic and general exam were normal.
Laboratory studies were notable for hyperglycemia with glucose 321 mg/dL
(17.8 mmol/L), normal electrolytes except for sodium of 132 (135-145
mmole/L) indicating pseudohyponatremia, HbA1c 14.5% (4-5.6%), the
presence of elevated levels of glutamic acid decarboxylase antibodies
10.3 (0-5 IU/mL) further confirming type 1 diabetes, and islet cell
antibodies <5.4 (0-7.4 Units/mL). His urine analysis was
negative for glucose, protein, and ketones for 3 occurrences during his
admission. An interpretation of the brain CT scan performed at the
outside hospital revealed a hyperintense lesion in the right basal
ganglia (Figure 1A and 1B). To further characterize this lesion, a brain
MRI was performed at our institution and was significant for T1
hyperintense/T2 hypointense signal within an atrophic right caudate
nucleus and lentiform nucleus (Figure 2A, 2B and 2C). Given the history
of uncontrolled T1D and the associated imaging findings, he was
diagnosed with nonketotic hyperglycemic hemichorea-hemiballismus which
is a rare complication of uncontrolled diabetes mellitus.
The patient was hospitalized for 3 days during which his insulin doses
were titrated, and he and his parent received 2 days of diabetes
education. He was also prescribed valproic acid 500 mg daily per
neurology recommendations that was gradually increased during outpatient
follow-up to 500 mg in the morning and 750 mg in the evening because his
symptoms persisted after discharge. His movements gradually improved
until they completely disappeared after 6 weeks from initial
presentation. Unfortunately, metabolic and glycemic control of his
diabetes were complicated by social determinants of health. He was
placed in the foster care system due to not having his needs met and his
diabetes remained poorly controlled. Despite his consistently high HbA1c
values with follow-up which ranged between 10.3%-14.4%, he had
significant weight gain from 52.2 Kgs at initial diagnosis of DKA to 77
Kgs at his last follow-up appointment after one year of diagnosis. His
choreiform movements however did not recur despite poor glycemic
control.