Abstract
Cutaneous vertebral medullary angiomatosis, also known as Cobb syndrome, is a rare segmental neurocutaneous syndrome. This syndrome is considered to be a non-hereditary congenital disease that is usually associated with arteriovenous malformations in the skin and spine. The clinical manifestations are complex because the lesions can involve the spine, spinal cord, skin, and even the viscera. Here, we present the case of a 10-year-old girl who was admitted to hospital due to headache with two episodes of convulsions. Previous examination at another hospital found no evidence of any abnormalities on either cranial or intracranial vascular magnetic resonance imaging (MRI). However, the patient had a history of subcutaneous hemangioma. Following exhaustive tests at our hospital, she was diagnosed with Cobb syndrome. She received surgery, treatment for decreasing intracranial pressure, and hormonal and nutritional support. She subsequently remained stable, with no recurrence of convulsions over a 9-year follow-up period. Here, we expand upon the clinical manifestations of Cobb syndrome and propose mechanisms for the underlying pathogenesis. We hope that our experience can help avoid missed diagnoses and misdiagnosis and provide more clinical evidence for early diagnosis.
Highlights
A 10-year-old girl was admitted to our hospital due to two episodes of seizures within the previous 3 months
The patient presented with a generalized tonic-clonic seizure that was followed by a headache of unknown cause
Known as cutaneous vertebral medullary angiomatosis, is generally considered to be a non-hereditary congenital disease. This syndrome is characterized by segmental vascular malformation involving the skin, soft tissue, spine, spinal cord, and even the viscera of the same body segment
Summary
A 10-year-old girl was admitted to our hospital due to two episodes of seizures within the previous 3 months. Three days prior to admission, the patient had experienced pharyngalgia, without fever, vomiting or diarrhea Later that day, she had experienced a second seizure which was similar to the first episode; this occurred during the night and was followed by headache, vomiting and rigidity in the neck. She had experienced a second seizure which was similar to the first episode; this occurred during the night and was followed by headache, vomiting and rigidity in the neck Her head was subsequently scanned by computed tomography (CT) in a local hospital, which revealed ventriculomegaly and showed that prominent. The patient regained conscious following the postoperative administration of hemostatic drugs, intravenous fluid therapy and 100 mg of phenobarbital (intramuscular, once daily) for 8 days She was seizure free, physically stable, and was subsequently discharged on day 35. This study was approved by the Ethics Committee of First Medical Center of PLA General Hospital, China (No 2018267)
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