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Posterior leukoencephalopathy syndrome may not be reversible
N L Antunes1, T N Small, D George
1Department of Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
Pediatric Neurology
|April 20, 1999
Summary
Reversible posterior leukoencephalopathy syndrome can manifest as oculogyric crisis, a rare presentation. Prompt diagnosis and cyclosporine withdrawal are crucial for preventing irreversible neurologic deficits in patients with this condition.
Area of Science:
- Neurology
- Radiology
- Immunology
Background:
- Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized by acute reversible encephalopathy and transient occipital lobe abnormalities.
- Typical symptoms include seizures, headache, altered mental status, and blindness, often linked to hypertension and immunosuppressive therapy.
Observation:
- A 2-year-old male with Down syndrome, post-allogeneic bone marrow transplantation, presented with severe oculogyric crisis.
- The patient was on cyclosporine for graft-vs-host disease prophylaxis and treatment for hypertension.
- Head CT revealed bilateral occipital white matter lucencies with punctate hemorrhage.
Findings:
- Discontinuation of cyclosporine led to clinical improvement.
- Follow-up MRI showed an area of leukomalacia, indicating a permanent neurologic change.
- Oculogyric crisis as a presenting symptom of RPLS is not previously described in the literature.
Implications:
- Recognizing oculogyric crisis as a potential symptom of RPLS is critical for timely diagnosis and intervention.
- Concurrent medications in transplant patients can complicate diagnosis, potentially delaying treatment and leading to irreversible neurologic deficits.
- Early identification and management of RPLS can prevent severe neurological sequelae.