Pearls & Oy-sters: Pisa syndrome: an unusual feature of adult-onset fulminant SSPE.

CASE REPORT A 25-year-old man was referred to our department, a tertiary care neurology facility, with complaints of slowness of movements and a body tilt to the right for 3 months. These complaints were accompanied by multiple falls and behavioral changes, including apathy, lack of self-care, and decreased verbal output. He had a history of high-grade fever lasting for 5 days prior to onset of these symptoms. The patient had a history of measles at age 3 years; he had not been vaccinated owing to sociocultural beliefs. The family history was not suggestive of juvenile parkinsonism, Huntington disease, genetic dystonias, or spinocerebellar ataxias. On examination, the patient was conscious but apathetic, with masked facies and a blink rate of 9–10 per minute. The vital parameters were normal and there was no postural drop in blood pressure. There was decreased verbal output with intact comprehension for simple commands. Mini Mental State Examination score was 20/30. The range of ocular movements was full with slowing of saccades. Rigidity without cogwheeling was present in all 4 limbs while the power was normal. Involuntary shock-like movements involving the trunk and limbs (left. right) were noted. The deep tendon jerks and superficial reflexes were normal. There were no frontal release signs. Bradykinesia was present while the pull test was positive. Gait analysis revealed a relatively wide-spaced stance with small, hesitant steps. The gait was interrupted by myoclonic jerks (video on the Neurology® Web site at Neurology.org). Lateral bending of the trunk (truncal dystonia, pleurothotonus) (figure 1) and striatal toe were noted in addition; there was no suggestion of dystonia elsewhere in the body. Hemogram, blood sugar, liver function tests, renal function tests, and thyroid function tests were normal. Enzyme-linked immunosorbent assay for HIV and tests for hepatitis B and hepatitis C viruses were also negative. EEG revealed a background of an average 20 mV amplitude a activity, interrupted by generalized periodic bursts of stereotypic high-amplitude slow wave R complexes occurring every 2–3 seconds (figure e-1). CSF analysis revealed 10 cells, all lymphocytes, protein 28 mg/dL, and sugar 85 mg/dL, with corresponding blood sugar of 103 mg/dL. CSF immunoglobulin G antimeasles antibody titers were 23.05 Novatech units (NTU) (normal ,9 NTU, interdeterminate 9–11 NTU, increased.11 NTU; Novatech Immune Diagnostica GmbH, Germany). CSF was negative for other viral markers. MRI of the brain showed subcortical white matter hyperintensities involving the frontal, parietal, and occipital regions on T2-weighted and fluid-attenuated inversion recovery sequences; no contrast enhancement was observed. The patient was initiated on clonazepam and trihexyphenidyl, and the dosage was titrated to 3 mg of clonazepam in 3 divided doses and 12 mg of trihexyphenidyl in 3 divided doses. He was also administered interferon-1a 6 million units intrathecally, once a week. The patient had a rapidly progressive downhill course. He became bedbound in 10 days and died on the 26th day of admission. Genetic test reports, available posthumously, revealed heterozygous TLR3 (Toll-like receptor 3) gene polymorphisms involving rs3775290, rs3775291, and rs3775296.