Neurological Manifestations in Parry–Romberg Syndrome

Abstract

Parry–Romberg syndrome (PRS) is a variant of morphea usually characterized by a slowly progressive course. Clinical and radiological involvement of the central nervous system may be observed in PRS.We describe 2 patients with PRS and neurological symptoms (one with trigeminal neuralgia associated with deafness, and the second with hemifacial pain associated with migraine without aura) in conjunction with abnormal cerebral MRI including white matter T2 hyperintensities and enhancement with gadolinium. Despite the absence of specific immunosuppressive treatments, both patients have presented stable imaging during follow-up without any clinical neurologic progression. We have performed a large review of the medical literature on patients with PRS and neurological involvement (total of 129 patients)Central nervous system involvement is frequent among PRS patients and is inconsistently associated with clinical abnormalities. These various neurological manifestations include seizures, headaches, movement disorders, neuropsychological symptoms, and focal symptoms. Cerebral MRI may reveal frequent abnormalities, which can be bilateral or more often homolateral to the skin lesions, localized or so widespread so as to involve the whole hemisphere: T2 hyperintensities, mostly in the subcortical white matter, gadolinium enhancement, brain atrophy, and calcifications. These radiological lesions do not usually progress over time. Steroids or immunosuppressive treatments are controversial since it remains unclear to what extent they are beneficial and there is often no neurological progression.

Late progression of neurological symptoms and MRI T2 hyperintensities in Parry-Romberg syndrome

“…However, late onset until the sixth and seventh decades of life has also been described 1 3. Our patient showed a comparatively late progression, more than 40 years after disease onset.…”

“…Cerebral atrophy was accompanied by contralateral cerebellar atrophy (F). Intrathecal IgG-synthesis as well as parenchymal and prominent perivascular T-cell infiltration in a brain biopsy (G) confirmed neuroinflammation as a potential pathophysiological correlate of PRS 1 2. Glucocorticoid treatment subsequently stabilised the late clinical progression.…”

“…Our patient showed a comparatively late progression, more than 40 years after disease onset. As a neurocutaneous disorder, PRS is characterised by PHA, which shows a clinical overlap with localised scleroderma (morphoea en coup de sabre) as well as by central nervous system involvement, the most common extracutaneous finding 1 3. Epilepsy, often refractory to anticonvulsive medication, and headache, are the most common neurological manifestations.…”

Relapsing–remitting lesions in a woman with progressive hemifacial atrophy and chronic hepatitis B virus infection

“…Sometimes, the condition may affect the underlying bony structures. [1,2] In the majority of patients, the disease stabilizes over a period of 2 to 10 years after its first presentation; a small proportion of patients (26%), however, may experience sustained progression. [1] Neurological complications such as epilepsy, migraine, hemiplegia, and trigeminal neuralgia may develop in 15% of all PHA patients.…”

Intracranial germinoma causing cerebral haemiatrophy and hypopituitarism

“…Parry-Romberg syndrome3 and linear scleroderma4 are possibly related, with the common feature of progressive atrophy of the facial skin and underlying soft tissue. In both disorders, focal unilateral atrophy (mostly facial in Parry-Romberg syndrome and frontal in linear scleroderma) may involve deep ipsilateral structures such as the skull, brainstem and brain parenchyma.…”