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Cobb syndrome: A rare cause of paraplegia.

Pal P, Ray S, Chakraborty S, Dey S, Talukdar A - Ann Neurosci (2015)

Bottom Line: These concurrent findings led to the diagnosis of Cobb's syndrome.Cobb's syndrome is a rare disease entity and literature search revealed only a few case reports and series mentioning this condition to date.The importance lies in the recognition that cutaneous vascular lesions may clue to an associated spinal cord angioma or AVM that may lead to weakness or paralysis.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Calcutta National Medical College and Hospital, Kolkata.

ABSTRACT
Cobb syndrome is an exceedingly rare clinical condition defined by the presence of a vascular skin nevus and an angioma in the spinal canal at the same metamere. We report the case of a 14-year-old boy who presented with sudden onset paraplegia. Physical examination showed port-wine stains over buttock and thigh. Magnetic resonance (MR) angiogram of the dorso-lumbar spine revealed a large arteriovenous malformation (AVM) at D11-D12 to L2-L3 levels. These concurrent findings led to the diagnosis of Cobb's syndrome. The patient received orally administered prednisolone therapy and underwent endovascular embolization of spinal angioma. Cobb's syndrome is a rare disease entity and literature search revealed only a few case reports and series mentioning this condition to date. The importance lies in the recognition that cutaneous vascular lesions may clue to an associated spinal cord angioma or AVM that may lead to weakness or paralysis.

No MeSH data available.


Related in: MedlinePlus

Large port-wine stains on his buttock (A) and over the anterior aspect of right thigh (B).
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fig_1: Large port-wine stains on his buttock (A) and over the anterior aspect of right thigh (B).

Mentions: A 14-year-old boy presented with sudden-onset weakness of the lower limbs associated with loss of sensation of all modalities up to the pelvis and with marked bladder/fecal dysfunction. There was a history of frequent cramps in both legs since 5 years of age. On examination, there were conspicuous port-wine stains in the buttock, measuring 60 × 45 cm and over the right thigh, measuring 40 × 25 cm (Figure 1). There was no palpable thrill or audible bruit over this area. These spots were initially blue after birth which gradually faded with time to evolve into the current appearance. Neurologically, the patient had marked flaccid weakness of both upper and lower limbs (grade 1/5). Deep tendon reflexes were absent, with plantar and cremasteric reflexes being non responsive. Definite sensory level was present corresponding to L1 segment of spinal cord. Fundoscopic examination was normal.


Cobb syndrome: A rare cause of paraplegia.

Pal P, Ray S, Chakraborty S, Dey S, Talukdar A - Ann Neurosci (2015)

Large port-wine stains on his buttock (A) and over the anterior aspect of right thigh (B).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4481555&req=5

fig_1: Large port-wine stains on his buttock (A) and over the anterior aspect of right thigh (B).
Mentions: A 14-year-old boy presented with sudden-onset weakness of the lower limbs associated with loss of sensation of all modalities up to the pelvis and with marked bladder/fecal dysfunction. There was a history of frequent cramps in both legs since 5 years of age. On examination, there were conspicuous port-wine stains in the buttock, measuring 60 × 45 cm and over the right thigh, measuring 40 × 25 cm (Figure 1). There was no palpable thrill or audible bruit over this area. These spots were initially blue after birth which gradually faded with time to evolve into the current appearance. Neurologically, the patient had marked flaccid weakness of both upper and lower limbs (grade 1/5). Deep tendon reflexes were absent, with plantar and cremasteric reflexes being non responsive. Definite sensory level was present corresponding to L1 segment of spinal cord. Fundoscopic examination was normal.

Bottom Line: These concurrent findings led to the diagnosis of Cobb's syndrome.Cobb's syndrome is a rare disease entity and literature search revealed only a few case reports and series mentioning this condition to date.The importance lies in the recognition that cutaneous vascular lesions may clue to an associated spinal cord angioma or AVM that may lead to weakness or paralysis.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Calcutta National Medical College and Hospital, Kolkata.

ABSTRACT
Cobb syndrome is an exceedingly rare clinical condition defined by the presence of a vascular skin nevus and an angioma in the spinal canal at the same metamere. We report the case of a 14-year-old boy who presented with sudden onset paraplegia. Physical examination showed port-wine stains over buttock and thigh. Magnetic resonance (MR) angiogram of the dorso-lumbar spine revealed a large arteriovenous malformation (AVM) at D11-D12 to L2-L3 levels. These concurrent findings led to the diagnosis of Cobb's syndrome. The patient received orally administered prednisolone therapy and underwent endovascular embolization of spinal angioma. Cobb's syndrome is a rare disease entity and literature search revealed only a few case reports and series mentioning this condition to date. The importance lies in the recognition that cutaneous vascular lesions may clue to an associated spinal cord angioma or AVM that may lead to weakness or paralysis.

No MeSH data available.


Related in: MedlinePlus