Cutaneomeningospinal Angiomatosis (Cobb Syndrome) in a Young Patient

Abstract

: Cobb Syndrome (Spinal Arteriovenous Metameric Syndrome 1-31 (SAMS 1-31)) is a rare, non-hereditary disorder. Approximately 100 cases of CS have been described to date. The actual incidence may be much higher since only symptomatic patients were documented. In particular, post mortem studies suggest a possibly higher incidence of this syndrome. The main clinical manifestations of this syndrome include skin stains of vascular nature on the torso, in combination with spinal vascular malformations localized in one and the same metameric or spinal segment. A rare diagnosis of this syndrome in patients over 18 is probably related to the fact that the disease may be asymptomatic throughout a long period of time, while patients may tend to disregard the skin lesions. As a result, most publications on this pathology are based on separate case reports. : Significant variability of clinical manifestations as well as prolonged progress of the disease often cause errors in diagnosis. What follows is a case report of a young patient with Cobb Syndrome, who was admitted to a regional vascular centre with a misdiagnosis of stroke. : 20 patients of young age (from 20 to 35 years old), with a diagnosis of stroke, who were admitted to a University Clinic (of the Russian National Research Medical University Named After Pirogov N.I., Moscow). Among this group of patients, a patient with Cobb syndrome was identified. Patient P., of 22 years, presented with acute, intensive cervical spinal pain, predominantly on the right, numbness and weakness in the arms and legs. About 3 weeks before admission to the hospital, the patient had ARVI with a fever of up to 37.5°C: two weeks before the onset of symptoms, he had undergone extirpation of 2 teeth, for which reason he spent over 2 hours in a forced position with his head thrown back (prolonged overextension in the cervical spine). Multiple skin angiomas on the chest spreading to the shoulder and scapula region. Tetraparesis up to 4 points: tetraparesis in hands with low muscle tone, low reflexes, tetraparesis in legs with high muscle tone, high reflexes, foot clonus when causing Achilles reflexes, tremor in the extremities and no plantar reflex pathology were detected, sensitivity disorders in the hands “the high gloves” and no pelvic disorders were detected. Given the presence and exacerbation of neurological symptoms and cutaneous angiomas, MRI with a contrast agent of the cervical spine was recommended. MR-image of an advanced arteriovenous malformation (AVM) of the cervical spinal cord with signs of gliosis and spinal cord oedema at the C2-C7 level. Endovascular embolization of the AVM in the cervical spinal cord was performed. The treatment led to the complete reversal of neurological symptoms. In the presence of skin lesions, the diagnosis of CS does not present particular difficulties, so in children and young patients with skin angiomatosis, it is advisable to conduct a comprehensive examination using selective spinal angiography or MR angiography to exclude arteriovenous malformations in the spinal cord.