Morgellon’s Disease and Neurocutaneous Syndrome (NCS): Cases and Recovery

The acronym PHACES summarizes the most important manifestations of a rare neurocutaneous syndrome. Specifically, “P” accounts for malformation of the brain in the region of the posterior fossa, “H” stands for haemangiomas, “A” is for arterial anomalies, and “C” is for coarctation of the aorta along with cardiac defects, “E” is for abnormalities of the eye, and “S” for clefting of the sternum, and/or a supraumbilical abdominal raphe. Our objective is to introduce the syndrome to paediatric cardiologists. Our patient has stenosis of the aortic arch, multiple malformations of the great vessels arising from the aortic arch, intracranial vascular abnormalities, a sternal malformation with a supraumbilical raphe, and facial haemangiomas. We stress that it is important always to consider the existence of this syndrome in all patients with facial haemangiomas.

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Encephalocraniocutaneous lipomatosis: An uncommon neurocutaneous syndrome

Journal of the American Academy of Dermatology ◽

10.1016/0190-9622(95)90411-5 ◽

1995 ◽

Vol 32 (2) ◽

pp. 387-389 ◽

Cited By ~ 25

Author(s):

Dolores Nosti-Martínez ◽

Victoria del Castillo ◽

Carola Durán-Mckinster ◽

Lourdes Tamayo-Sánchez ◽

Maria de la Luz Orozco-Covarrubias ◽

...

Keyword(s):

Encephalocraniocutaneous Lipomatosis ◽

Neurocutaneous Syndrome

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Occlusive arteriopathy and brain tumor

Journal of Neurosurgery ◽

10.3171/jns.1978.49.1.0022 ◽

1978 ◽

Vol 49 (1) ◽

pp. 22-35 ◽

Cited By ~ 53

Author(s):

Koreaki Mori ◽

Fuji Takeuchi ◽

Masatsune Ishikawa ◽

Hajime Handa ◽

Mitsuo Toyama ◽

...

Keyword(s):

Brain Tumor ◽

Cerebral Angiography ◽

Mass Effect ◽

Clinical Analysis ◽

Sudden Onset ◽

Circle Of Willis ◽

Ischemic Lesion ◽

Neurocutaneous Syndrome ◽

The Brain ◽

Occlusive Arteriopathy

✓ Four cases with the association of occlusive arteriopathy and brain tumor are presented. A clinical analysis of these cases and cases reported in the literature revealed that occlusive arteriopathy at the base of the brain was often associated with a slowly growing basal tumor in children. Possible causes of occlusive arteriopathy in these cases were compression of the circle of Willis by a slowly growing basal tumor, secondary arterial occlusive changes by radiation therapy for a basal tumor, or vasculopathy associated with neurocutaneous syndrome. Symptoms of sudden onset or episodic nature suggest the presence of occlusive arteriopathy rather than the mass effect of a tumor. Cerebral angiography is mandatory whenever computerized tomography (CT), performed to rule out recurrence of a basal tumor, shows an ischemic lesion with low-density areas without any evidence of mass effect of the tumor. Cerebral angiography is also necessary when a basal tumor is suspected in children, particularly in cases associated with neurocutaneous syndrome and a basal tumor. Care should be taken not to scarify the abnormal vascular network at the base of the brain at the time of operation, because it is considered to be functioning as collateral circulation. The potential hazards of radiotherapy to radiation-induced occlusive changes of the circle of Willis must be considered in treating a benign basal brain tumor in children. Even in adults, repeated large doses of irradiation could cause occlusive arteriopathy.

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Neurocutaneous syndrome: A prospective study

Indian Journal of Dermatology ◽

10.4103/0019-5154.84721 ◽

2011 ◽

Vol 56 (4) ◽

pp. 375 ◽

Cited By ~ 8

Author(s):

Tryambak Samanta ◽

Sandipan Dhar ◽

Radheshyam Purkait ◽

Sachin Thakur

Keyword(s):

Prospective Study ◽

Neurocutaneous Syndrome ◽

A Prospective Study

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Hypomelanosis of Ito: Case report of a rare neurocutaneous syndrome in a neonate and review of the literature.

Nigerian Journal of Paediatrics ◽

10.4314/njp.v42i3.12 ◽

1970 ◽

Vol 42 (3) ◽

pp. 231-233

Author(s):

M Mukhtar-yola ◽

LI Audu ◽

AT Otuneye ◽

AB Mairami ◽

EC Otubelu ◽

...

Keyword(s):

Case Report ◽

High Index ◽

Review Of The Literature ◽

Cutaneous Lesions ◽

Hypomelanosis Of Ito ◽

The Third ◽

Neurocutaneous Syndrome ◽

Paediatric Practice ◽

High Index Of Suspicion

Hypomelanosis of Ito (HI) though said to be the third most common neurocutaneus disorder, is rarely reported in paediatric practice in Africa. A high index of suspicion must be maintained in children with cutaneous lesions as a seizure may be the first symptom that may bring the child to attention. A case of HI in a neonate is hereby reported to sensitize clinicians about this relatively uncommon disorder

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Neurocutaneous Syndrome

Pediatric Oncall ◽

10.7199/ped.oncall.2014.44 ◽

2014 ◽

Vol 11 (3) ◽

Author(s):

Spoorthi Jagadish ◽

Tanavi Ghadage

Keyword(s):

Neurocutaneous Syndrome

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Encephalocraniocutaneous lipomatosis: a review of its clinical pathology and neurosurgical indications

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.6.peds11138 ◽

2011 ◽

Vol 8 (3) ◽

pp. 316-320 ◽

Cited By ~ 8

Author(s):

Robert E. Ayer ◽

Alexander Zouros

Keyword(s):

Spinal Cord ◽

Clinical Pathology ◽

Tethered Cord ◽

Male Infant ◽

Review Of The Literature ◽

Encephalocraniocutaneous Lipomatosis ◽

Neurocutaneous Syndrome ◽

Brain And Spinal Cord ◽

The Brain ◽

Cervicomedullary Compression

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome whose hallmark lesions are benign lipomas of the brain and spinal cord. The authors present a case of a male infant with ECCL who had extensive brainstem and spinal cord lipomas. The management of this patient's hydrocephalus, cervicomedullary compression, tethered cord, and scoliosis over the course of his first 2 years of life is described. This case report and review of the literature is presented to provide a synopsis of the problems likely to be encountered by neurosurgeons who treat patients with this syndrome.

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