Parry Romberg syndrome and linear scleroderma in coup de sabre mimicking Rasmussen encephalitis

Area of Science:

• Neurology
• Dermatology
• Pathology

Background:

• Parry Romberg syndrome and linear scleroderma are rare conditions affecting the skin and underlying tissues.
• Focal neurological deficits and intractable seizures can occur in patients with these conditions.
• The relationship between these rare cutaneous disorders and neurological manifestations requires further investigation.

Observation:

• Two patients presented with Parry Romberg syndrome and linear scleroderma in coup de sabre, respectively.
• Both patients exhibited focal neurologic deficits and intractable seizures.
• The neurological symptoms were ipsilateral to the cutaneous lesions.

Findings:

• Brain magnetic resonance imaging (MRI) revealed progressive hemispheric atrophy in both patients.
• Pathological examination after functional hemispherectomy showed chronic inflammatory features.
• The histopathological findings were suggestive of Rasmussen encephalitis.

Implications:

• These cases highlight a potential link between specific rare connective tissue diseases and severe, progressive brain inflammation.
• Rasmussen encephalitis should be considered in the differential diagnosis of intractable epilepsy with focal neurological deficits and ipsilateral hemispheric atrophy, especially in the context of Parry Romberg syndrome or linear scleroderma.
• Further research is needed to elucidate the underlying mechanisms connecting these distinct conditions.