Title:Cutaneomeningospinal Angiomatosis (Cobb Syndrome) in a Young Patient
Volume: 20
Issue: 10
Author(s): Marina Putilina*, Nataliya Teplova and Anton Dvornikov
Affiliation:
- Chair of Clinical Pharmacology, Chair of Dermatology and Venereology, Russian National Research Medical University Named After Pirogov N.I., Ostrovityanov St., 1, 117997, Moscow,Russian Federation
Keywords:
Cobb syndrome, skin hemangiomas, arteriovenous malformation, stroke, young patient, endovascular embolization.
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.
