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Cerebrospinal fluid pressures in subacute sclerosing panencephalitis
Akgün Olmez1, Deniz Yilmaz, Hüseyin Tan
1Hacettepe University, Department of Pediatric Neurology, Ankara, Turkey. dr_akgun@yahoo.com
Increased intracranial pressure is a rare initial symptom of subacute sclerosing panencephalitis (SSPE). High CSF pressure in SSPE patients correlated with frequent myoclonia and a shorter interval between measles and SSPE onset.
Area of Science:
- Neurology
- Pediatric Neurology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disorder.
- Increased intracranial pressure (ICP) is an uncommon initial presentation of SSPE.
Observation:
- Cerebrospinal fluid (CSF) pressures were measured in 58 SSPE patients.
- CSF pressure ranged from 50 to 500 mmH2O, with a mean of 210.9 mmH2O.
- 42% of patients had CSF pressure >200 mmH2O, and 25% had pressure >250 mmH2O.
Findings:
- No correlation was found between CSF pressure and neurological disability, spasticity, or clinical stage.
- Elevated CSF pressure (>200 mmH2O) was associated with frequent myoclonia (p=0.035).
- A shorter interval between measles infection and SSPE onset correlated with higher CSF pressure.
Implications:
- The etiology of elevated ICP in SSPE remains unclear, potentially linked to myoclonic jerks or inflammatory responses.
- Clinicians should consider increased ICP in SSPE patients exhibiting irritability or frequent myoclonia, especially if unable to communicate symptoms.
- This highlights the importance of monitoring ICP in specific SSPE patient subgroups.
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