Rasmussen encephalitis associated with Parry–Romberg syndrome

Abstract

Parry-Romberg syndrome is a rare disorder associated with unilateral facial atrophy involving skin, subcutaneous tissue, skeletal muscle, and bone. Occasionally, there is CNS involvement with epilepsy being the most common CNS manifestation. The authors report a child with Parry-Romberg syndrome with a course strongly suggestive of Rasmussen encephalitis. The boy underwent hemispherectomy, and pathology showed the typical findings of Rasmussen encephalitis, suggesting that these two conditions may share common etiologic factors.

Overlap between linear scleroderma, progressive facial hemiatrophy and immune-inflammatory encephalitis in a paediatric cohort

“…The most common neurological complications are seizures and migraine, which may even be the presenting signs [16]. Most neurological problems are ipsilateral to the cutaneous signs (as in patient 6,7,8,14); contralateral lesions have been described as well as generalized lesions, as in our patient 12 [18].…”

“…Other cases of overlap between PFH and Rasmussen encephalitis have been reported [13,14]. Linear scleroderma en coup de sabre/ progressive facial hemiatrophy Fig.…”

“…Autoimmune encephalitis is characterized by the presence of various autoantibodies both in serum and CSF of the affected individuals [13,14]. In the two patients with the most severe neurological complications, additional autoimmune manifestations in the CSF were found: ANA in the CSF in one and signs of intrathecal IgG production with-several years after the initial presentation-the presence of anti-NMDA antibodies in the other.…”

“…Moreover, overlap between these diseases and encephalitis has been reported [1,13,14]. The pathogenesis of these diseases remains a matter of discussion [15,18].…”

Idiopathic acquired progressive left facial hemiatrophy (Parry-Romberg syndrome) in a 21-year-old man in semi-urban, south-west Nigeria

“…[4][5][6] It also has been associated with status migrainosus, an alien hand syndrome from contralateral thalamic degeneration, as well as an infection with Borriela burgdoferi (the aetiological agent of Lyme disease) -all underscoring the complex nature of this clinical entity.…”