Video 1
Video recording of patient’s neurologic examination. This video shows the patient’s choreoathetotic movements at rest while answering questions testing her abstraction and memory.
Table 1
Clinical features of movement disorders secondary to HHV-6 infection or reactivation.
| CASE 1 [1] | CASE 2 [3] | CASE 3 [4] | |
|---|---|---|---|
| Age at onset | 14 months | 1 year | 32 years |
| Sex | Female | Female | Male |
| Presenting symptoms | Fever and generalized clonic status epilepticus, hypotonia and developmental regression | Fever and exanthema subitum | Progressive parkinsonism, symmetric hyperreflexia and cognitive dysfunction 6 weeks after HSCT |
| Description of movement disorder | Progressive truncal ataxia, orofacial dyskinesias, and choreiform movements of the mouth, extremities, and trunk | Decreased voluntary movements and hand ataxia, unable to stand independently or maintain a sitting position | Bilateral bradykinesia, rest tremor, upper limb rigidity |
| CSF studies | 11 WBCs (lymphocytic) Glucose 75 Protein 20 +HHV-6 A and B PCR | Normal -HHV-6 PCR (+HHV-6 IgM in serum) | 45 WBCs (lymphocytic) Glucose 64 Protein 61 +HHV-6 PCR |
| MRI results | Bilateral periventricularand subcortical diffusion abnormalities related to post-ictal edema | Low intensity signals on T1-weighted images and high intensity signals on T2-weighted images in the bilateral putamen consistent with putaminal necrosis | Caudate and putamen hyperintensity in FLAIR and T2-weighted images and non-enhancing in Tl- weighted image |
| Treatment | Levetiracetam IVIg for 5 doses IV foscarnet for 2 weeks | No pharmacologic treatment | IV ganciclovir for 2 weeks Levodopa |
| Recovery | 6 months after discharge patient remained seizure- free with resolution of chorea but persistent hypotonia, poor coordination. complete absence of expression language, other moderate developmental delays | 1 year after discharge she had persistent mild dystonic or athetotic posturing of her extremities but was able to take several paces without support | Disorientation improved with ganciclovir and parkinsonism remained stable with a moderate response to levodopa |
[i] Abbreviations: HSCT, hematopoietic stem cell transplant; WBCs, white blood cells; HHV-6, human herpesvirus-6; PCR, polymerase chain reaction; MRI, magnetic resonance imaging; FLAIR, fluid-attenuated inversion recovery; IVIg, intravenous immunoglobulin; IV, intravenous.