Clinical Reasoning: A 65-year-old woman with subacute hemiballism

Section 1

A 65-year-old, right-hand-dominant woman with a history of hypertension, hyperlipidemia, poorly controlled diabetes, and remote breast cancer presented with 1 week of progressive, involuntary left hemibody movements. One month prior, she was a restrained driver in a head-on motor vehicle collision with possible brief loss of consciousness. The patient was evaluated at an emergency department and was noted to have musculoskeletal injuries not requiring intervention. No imaging was performed. Since the accident, she described feeling a “mental fog” with slurred speech that has progressively worsened and persistent left shoulder pain.

About 1 week prior to presentation at our facility, she developed nonvoluntary movements starting in her left shoulder, described as “flopping,” with gradual progression to incorporate the whole upper limb that spread to the ipsilateral leg and face. These movements became more frequent and increased in amplitude over a week. There was no change in consciousness, loss of bowel/bladder function, or tongue biting during these movements, and no postevent confusion. Due to interference with daily activities, she presented to the emergency department for further evaluation, where a CT head demonstrated a hyperdense lesion in the right basal ganglia with limited surrounding edema.

The patient’s vital signs were within normal limits and neurologic examination was notable for mild dysarthria with dyskinetic oromandibular facial movements, intact strength throughout (though limited by left shoulder pain), mild left dysmetria, and left dysdiadochokinesia. At rest, she had intermittent ballistic–choreic movements of her left upper and lower extremities with involvement of the face and tongue, which worsened with intentional maneuvers.

Section 2

The initial differential of acquired causes of hemichorea–hemiballism includes vascular, autoimmune/inflammatory, metabolic, infectious, toxin/drug-induced, and neoplastic or other structural etiologies.^1,–,3 In particular, stroke, Wilson disease, thyrotoxicosis, hyperglycemia, and systemic lupus erythematosus have been known to cause this syndrome.¹ Genetic causes were less likely given age and CT abnormalities. Preliminary laboratory studies demonstrated signs of a urinary tract infection (urinalysis with positive leukocyte esterase and 87 white blood cells) without evidence of systemic infection or inflammation (white blood cells 7.1, erythrocyte sedimentation rate 26) and poorly controlled diabetes (serum glucose 425, urine trace ketones, HbA1c 14). Other evidence of infection was not seen in the CSF (negative tuberculosis and bacterial/fungal cultures) or serum (toxoplasma immunoglobulin G and M).

MRI brain with contrast obtained during admission revealed rim-enhancing lesions within the right caudate head and anterior aspect of the right putamen without diffusion restriction but with intralesional hemorrhage (figure). MRI was repeated 2 months later and demonstrated new confluent areas of T1 hyperintensity involving the right lentiform nucleus sparing the right internal capsule with increasing volume loss of the right caudate head and resolution of T2 hyperintensity in this region (figure).

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Figure Initial and follow-up imaging

(A) Initial imaging. (A.a) Initial CT: hypoattenuating and subtle expansile appearance of the right caudate head; right putamen subtly hyperattenuating. (A.b–A.c) Initial diffusion: mild increased signal in area of fluid-attenuated inversion recovery (FLAIR) hyperintensity on trace imaging related to increased diffusivity given high apparent diffusion coefficient values. (A.d) Initial FLAIR: rounded areas of increased signal involving caudate head and putamen. (A.e–A.f) Initial T1 pre and post: hypointense appearance of expansile right caudate head and anterior putamen, which shows peripheral enhancement. Subtle increased T1 signal of remainder of putamen. (B) Follow-up imaging at 2 months. (B.a) FLAIR: resolution of hyperintensity involving right lentiform nucleus. (B.b) T1 precontrast: confluent area of T1 shortening involving the right basal ganglia. (B.c) T1 postcontrast: T1 shortening, without enhancement, of the caudate head, putamen, and globus pallidus. Interval right caudate head volume loss.

Section 3

The differential for rim/ring-enhancing lesions can be remembered with the mnemonic MAGICAL DR: metastasis, abscess, glioblastoma, infarction (subacute), contusion, aneurysm, lymphoma, demyelination, and radiation necrosis or resolving hematoma.⁴ It may also be worthwhile to consider nonabscess infections such as tuberculomas and neurocysticercosis. The common elements in the differential for acquired causes of hemiballism and ring-enhancing lesions narrow the list to favor infectious, neoplastic, and ischemic etiologies. In addition to the enhancing lesion, imaging was also notable for right putaminal T1 hyperintensity, which suggested a possible metabolic process vs substance deposition of lipid, protein, or hemosiderin. This complicates the differential as none of the favored etiologies should present with this hyperintensity, suggesting a possible secondary pathology or an atypical presentation.

Chart review for background was notable for negative lymph nodes at breast cancer lumpectomy and laboratory studies drawn 8 days prior to presentation that demonstrated a blood glucose level of 599 whereas prior glucose levels were 120–200 with a HbA1c 7.0 3 months prior to presentation compared to 14 on presentation.

Section 4

The patient presented with subacute onset of left hemiballism 1 month after head trauma in setting of multiple vascular risk factors, acute-on-chronic hyperglycemia, and history of cancer. Imaging was notable for persistent T1 hyperintensity of the right lentiform nucleus and rim-enhancing lesions of the right caudate head and anterior putamen progressing to caudate volume loss. She was started on quetiapine for the hemiballism and strict diabetes control was recommended. On follow-up 3 months after symptom onset, her HbA1c was 7.3 and her movements had markedly diminished. She was trialed off of quetiapine but had return of less-severe hemiballism for which she restarted quetiapine. The history, laboratory studies, MRI findings with interval change, and clinical progression indicate that hyperglycemic injury with subacute small vessel infarction is the most likely diagnosis.