Objective: To illustrate a novel case of adult-onset choreoathetosis in the setting of HHV-6 infection

Background: Human herpesvirus-6 (HHV-6) has been associated with a spectrum of neurologic disorders, but movement disorders are rare with few published case reports. This case describes a patient who developed a choreoathetotic movement disorder in the setting of HHV-6 infection following allogeneic stem cell transplant for acute myeloid leukemia (AML).

Method: A 72-year-old woman with a history of breast cancer in remission but complicated by secondary AML was admitted for AML consolidation therapy. She was given cyclophosphamide, fludarabine, and total body irradiation followed by allogeneic stem cell transplant. Two and a half weeks post-transplant, she became somnolent and febrile, with rising titers of HHV-6 in the serum and cerebrospinal fluid concerning for HHV-6 encephalitis. Her infectious and metabolic work up was otherwise negative, apart from mild acute renal injury, and MRI brain showed no acute abnormalities. She was treated with ganciclovir followed by acyclovir prophylaxis, with improvement in her mental status and decrease in HHV6 viral load. Over the next month, she developed involuntary movements, and neurologic examination revealed motor impersistence and continuous, irregular, non-stereotyped writhing movements of the head, neck, and upper limbs. She had no history of exposure to neuroleptics, metoclopramide, or prochlorperazine and no personal or family history of movement disorder.

Results: The patient was diagnosed with a choreoathetotic movement disorder secondary to HHV-6 infection. With recovery from her infection, she had gradual symptom improvement. She was treated with valbenazine briefly during this period, without further improvement. Differential diagnosis included a secondary immune response, mild renal failure, or tacrolimus-induced chorea. These were less likely causes, as symptoms began when renal function was improving, with normal tacrolimus levels, and without dosing change.

Conclusion: This is the first literature reported case of adult-onset chorea associated with HHV-6 infection. It is unclear if the movement disorder was secondary to the infection itself or an autoimmune response, however the rarity of this syndrome presents a unique diagnostic and therapeutic challenge. Ultimately infection treatment, with resolution in serum over time led to improvement of symptoms.

References: [1]Cury RG, Contreras Lopex WO. Bilateral striatal lesion due to herpesvirus-6 infection. Journal of the Neurological Sciences. 2015 Nov;358(1-2):538-539. [2]Feinstein E, Walker R. Treatment of Secondary Chorea: A Review of the Current Literature. Tremor and Other Hyperkinetic Movements (N Y). 2020 Jul;10(22). [3]Murakami A, Morimoto M, Adachi S, Ishimaru Y, Sugimoto T. Infantile bilateral striatal necrosis associated with human herpes virus-6 (HHV-6) infection. Brain and Development. 2005 Oct;27(7):527-530). [4]Pulickal AS, Ramachandran S, Rizek P, Narula P, Schubert R. Chorea and developmental regression associated with human herpes virus-6 encephalitis. Pediatric Neurology. 2013 Mar;48(3):249-251.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/choreoathetosis-in-the-setting-of-hhv-6-infection-in-an-allogeneic-transplant-recipient/