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. In light of the abnormal brain MRI performed at the outside hospital, the differential diagnosis was narrowed down to a brain tumor, cerebral infarct, or NHHH. Laboratory studies were notable for hyperglycemia with glucose 321 mg/dl (17.8 mmol/L), normal electrolytes except for sodium of 132 (135-145 mmol/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 T1D, 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,B). 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-C). Given the history of uncontrolled T1D and the associated imaging findings, he was diagnosed with NHHH 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. No follow-up brain imaging was performed. 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% and 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 1 year of diagnosis. His choreiform movements, however, did not recur despite poor glycemic control. A summary of the patient's presentation is presented in Table 2.