Cutis verticis gyrata, underrecognized neurocutaneous syndrome

Abstract

I report two patients with cutis verticis gyrata. Characteristic of this neurocutaneous syndrome is the scalp tissue folds that generally run in an anterior-posterior direction. Although the etiology of the scalp abnormality is unclear, patients may have associated static encephalopathy, seizure disorder, or minor ocular abnormalities. The prognosis is benign. Other organ systems are spared and there is no malignant transformation of scalp tissue or brain parenchyma.

Magnetic Resonance Imaging of Cutis Verticis Gyrata

“…The essential primary CVG is not associated with any disease; however, the nonessential form is associated with mental deficiency, epilepsy, seizures, and different ophthalmic abnormalities; hence, it is considered a neurocutaneous syndrome. 6 The secondary form of CVG on the other hand can occur at any age and usually has asymmetric distribution. Several diseases have been reported in association with secondary CVG including local scalp conditions such as eczema, psoriasis, neurofibromas, and congenital nevus (cerebriform intradermal nevus) as well as systemic diseases such as acromegaly, myxedema, amyloidosis, leukemia, tuberous sclerosis, diabetes, and pachydermoperiostosis.…”

“…2,6 Some authors believe that the higher incidence in men is related to easier detection because of shorter hair cut. 6 Primary nonessential CVG was found in 0.2% to 12.5% of institutionalized psychiatric patients, and several theories have been proposed to explain this finding. 2 The diagnosis of CVG is primarily a clinical one; however, incidental cases are often identified on computed tomography (CT) and MRI.…”

“…The hair overlying CVG is usually normal but, in some cases, may be scanty or thickened. 6 On MRI, there are 3 distinctive layers in the scalp. Epidermis and dermis (skin) are seen as one layer, subcutaneous fat as one layer, and galea/subgalea/periosteum complex as a third layer.…”

Cutis verticis gyrata secondary to cerebriform intradermal nevus

“…Basically, it has been classified as primary (essential and non-essential) and secondary cutis verticis gyrata. The primary essential form evolves from normal skin, on the other hand secondary forms can be associated with neoplasms, inflammatory diseases, systemic and congenital diseases [3]. Cutis verticis gyrata associated with cerebriform intradermal nevi is among the rare secondary forms.…”

Essential primary cutis verticis gyrata

“…A CVG pode ser classificada em duas formas: primária (essencial e não-essencial) e secundária 4 . A forma primária não-essencial responde por 0,5% dos pacientes com retardo mental; paralisia cerebral, epilepsia, catarata e cegueira podem estar presentes 1,5 . Já a forma primária essencial não está associada com alteração neurológica e oftalmológica, ocorre somente formação de dobras no couro cabeludo, que mimetizam os giros cerebrais; aparece na puberdade e ocorre exclusivamente em homens; o tipo de herança é incerta 1,4,5 .…”