Extensive metameric involvement in Cobb syndrome presenting as compressive myelopathy

Area of Science:

• Neurology
• Vascular Malformations
• Dermatology

Background:

• Cobb Syndrome (cutaneomeningospinal angiomatosis) is a rare, non-hereditary neurocutaneous disorder.
• It is characterized by spinal arteriovenous malformations (AVMs) and concurrent cutaneous vascular lesions within the same metamere.
• This condition often presents with progressive neurological deficits due to spinal cord compression.

Purpose of the Study:

• To report a rare case of Cobb Syndrome with extensive spinal involvement.
• To emphasize the diagnostic challenges and management strategies for this condition.
• To highlight the importance of recognizing cutaneous markers for early diagnosis.

Main Methods:

• Case presentation of Cobb Syndrome.
• Diagnostic workup included Magnetic Resonance Imaging (MRI) and angiographic studies.
• Surgical decompression was performed for spinal cord compression.

Main Results:

• The patient presented with progressive paraparesis due to dorsal compressive myelopathy.
• Extensive vascular malformations affected all spinal segments, a rare presentation.
• Vertebral hemangiomas and an epidural AVM were identified, causing significant spinal cord compression.
• Surgical decompression resulted in partial neurological recovery.

Conclusions:

• Early recognition of cutaneous markers is vital for diagnosing neurocutaneous syndromes like Cobb Syndrome.
• Prompt neurosurgical intervention is essential to prevent irreversible neurological deficits.
• This case underscores the variability in presentation and the need for comprehensive spinal evaluation.