Full clinical recovery after acute haemolytic uraemic syndrome (HUS) with basal ganglia involvement and severe dystonia. A case report

Objective: Central nervous system (CNS) involvement is the most frequent extra-renal complication of haemolytic uraemic syndrome (HUS) affecting about 20 to 50% of children with HUS. Pathological changes of the basal ganglia have been repeatedly described. However, the clinical course and final outcome is quite variable.

Material and methods: We describe the clinical course, MR imaging results and outcome of a six-year-old Turkish boy who developed an acute E. coli associated HUS with severe neurological symptoms. The case will be demonstrated including serial MRIs and typical video sequences.

Results: The anuric boy required haemodialysis for 4 weeks. He showed opisthotonus and severe dystonia with increased and changing muscle tone mainly in trunk and extremities, did not react to moderate stimulation, and was unable to swallow or perform any directed motility or coordinated ocular movements. Deep tendon reflexes could not be elicited. Standard EEG showed general slowing. A first combined CT/MRI examination revealed swelling and oedema of the putamen, pallidum and caudate nucleus extending to the white matter of the external and internal capsule, but no signs of cerebral bleeding. During several weeks with intensive rehabilitation the boy slowly improved and neurological symptoms decreased. First coordination of ocular movements improved, and he held eye-contact whilst severe dystonia mainly in trunk and extremities was still present. Later, dystonia was constantly improving up to a full recovery of all functions. However, a final MRI showed reduced size of the putamen and persisting gliosis on both sides.

Conclusion: Even in cases with severe neurological symptoms, extended changes of basal ganglia and white matter, and persisting residual basal ganglia changes, full clinical recovery in children with HUS and CNS involvement is possible.