Cobb syndrome

Cobb syndrome
Cobb syndrome
Classification and external resources
DiseasesDB 32587
eMedicine article/1086037

Cobb syndrome is a rare congenital disorder characterized by visible skin lesions with underlying spinal angiomas or arteriovenous malformations (AVMs).[1] The skin lesions of Cobb syndrome typically are present as port wine stains or angiomas, but reports exist of angiokeratomas,[2] angiolipomas, and lymphangioma circumscriptum.[3] The intraspinal lesions may be angiomas or AVMs and occur at levels of the spinal cord corresponding to the affected skin dermatomes. They may in turn produce spinal cord dysfunction and weakness or paralysis.

The disorder was first described by Berenbruch in 1890, but became widely known only after Cobb's report in 1915.[4] Cobb syndrome is thought to be more common in males and have no racial prediliction, but only a few dozen cases are known. It is believed to be due to a sporadic mutation, since parents of affected children usually have no evidence of the disease.

Signs & Symptoms

The disease is present at birth, but clinical manifestations are often not seen until later in life. Patients typically experience the sudden onset of pain, numbness, or weakness in their extremities as children or young adults. These symptoms may remit or remain stable and often can be localized below a specific dermatome. Symptoms tend to worsen over time either by discrete steps or continuously. Early development of weakness may portend a more aggressive course. Less commonly, weakness or bowel and bladder dysfunction may be presenting symptoms.

The major debility from Cobb syndrome is the onset of weakness, paresis, sensory loss, and loss of bowel and bladder control. A possible complication if treatment is delayed is Foix-Alajouanine disease[5] or subacute necrotic myelopathy due to thrombosis in the spinal angioma.

Cutaneous lesions may be distributed anywhere in the dermatome, from midline back to abdomen. Midline back lesions may be associated with spina bifida. The cutaneous lesion may be very faint and may be more pronounced when the patient performs a Valsalva maneuver which increases abdominal pressure and causes preferential filling of the cutaneous angioma. Neurological examination will reveal weakness or paralysis and numbness or decreased sensation with a sharp upper cutoff.

See also

References

  1. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0. 
  2. ^ Clinton TS, Cooke LM, Graham BS. Cobb syndrome associated with a verrucous (angiokeratomalike) vascular malformation. Cutis. Apr 2003;71(4):283-7.
  3. ^ Shim JH, Lee DW, Cho BK. A case of Cobb syndrome associated with lymphangioma circumscriptum. Dermatology. 1996;193(1):45-7.
  4. ^ Cobb S. Haemangioma of the spinal cord associated with skin naevi of the same metamere. Annals Surgery. 1915;62:641-9.
  5. ^ Wirth FP, Post KD, Di Chiro G. Foix-Alajouanine disease. Spontaneous thrombosis of a spinal cord arteriovenous malformation: a case report. Neurology. 1970;20:1114-18.

Wikimedia Foundation. 2010.

Игры ⚽ Нужно решить контрольную?

Look at other dictionaries:

  • Syndrome post-commotionnel — Classification et ressources externes CIM 10 F07.2 CIM 9 310.2 eMedicine …   Wikipédia en Français

  • syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… …   Medical dictionary

  • Cobb — Stanley, U.S. neuropathologist, 1887–1968. See C. syndrome …   Medical dictionary

  • Síndrome de Cobb — Este artículo o sección tiene un estilo difícil de entender para los lectores interesados en el tema. Si puedes, por favor edítalo y contribuye a hacerlo más accesible para el público general, sin eliminar los detalles técnicos que interesan a… …   Wikipedia Español

  • Thoracic outlet syndrome — Classification and external resources The right brachial plexus, viewed from in front. In thoracic outlet syndrome there is compressio …   Wikipedia

  • Post-concussion syndrome — Classification and external resources ICD 10 F07.2 ICD 9 310.2 …   Wikipedia

  • Second-impact syndrome — (SIS) is an extremely rare condition in which the brain swells rapidly and catastrophically after a person suffers a second concussion before symptoms from an earlier concussion have subsided. SIS may develop when a second blow to the head occurs …   Wikipedia

  • Liste der Syndrome — Diese Seite listet in alphabetischer Reihenfolge und ohne Anspruch auf Vollständigkeit Syndrome und Komplexe aus unterschiedlichen medizinischen Fachgebieten auf. Bitte nur Verweise auf den tatsächlichen Titel des Beitrags und keine… …   Deutsch Wikipedia

  • King Cobb Steelie (album) — Infobox Album Name = King Cobb Steelie Type = studio Artist = King Cobb Steelie Released = 1993 Recorded = Genre = Length = Label = Raw Energy Records Producer = Reviews = Last album = This album = Next album = King Cobb Steelie is the self… …   Wikipedia

  • List of cutaneous conditions — This is an incomplete list, which may never be able to satisfy particular standards for completeness. You can help by expanding it with reliably sourced entries. See also: Cutaneous conditions, Category:Cutaneous conditions, and ICD 10… …   Wikipedia

Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”