A 29-year-old man presented with cutaneous hemangioma and a progressive paraparesis and paresthesia of the lower extremities. [ncbi.nlm.nih.gov]

This is the first case presented from an institution of Argentina. [surgicalneurologyint.com]

• Arm Pain

  We present a 16-year-old boy with undiagnosed Cobb syndrome who developed acute chest and right arm pain followed by severe quadriparesis and respiratory failure; the patient recovered substantially after microsurgical treatment without embolization. [surgicalneurologyint.com]

• Soft Tissue Mass

  Aboulafia Medicine BMJ Case Reports 2018 A 62-year-old woman was referred to the orthopaedic oncology service for evaluation of a bleeding and enlarging soft tissue mass along the right anterior abdominal wall, affecting the right lateral chest and right [semanticscholar.org]

  tissue mass along the right anterior abdominal wall. [casereports.bmj.com]

• Localized Pain

  The radiation of pain from the back to the stomach must make one think of aneurysm, gastric ulceration, cholecystitis, or appendicitis, knowing that younger patients have difficulty specifically localizing pain. [healio.com]

• Cutaneous Manifestation

  Cobb syndrome is an unusual entity in the adult population and should be considered when there is a constellation of cutaneous manifestation and underlying neurological deficit. [ncbi.nlm.nih.gov]

  The cutaneous manifestations of the syndrome are most often flat vascular lesions (port-wine stains) but angiokeratomas, angiolipomas and lymphangiomas have been reported. The medullary lesions are arteriovenous malformations. [orpha.net]

• Hyperreflexia

  Signs of neurological involvement include: Hyperreflexia (overactivity of physiological reflexes), and sensory and motor defects, including paralysis Morphological (structural) abnormalities of the central nervous system. [dermnetnz.org]

• Spinal Cord Syndrome

  It is stressed the importance of clinical suspicion of cutaneo-meningospinal angiomatosis based on a spinal cord syndrome in the presence of a vascular skin nevus of the same metameric level. [ncbi.nlm.nih.gov]

  It is stressed the importance of clinical suspicion of cutaneo-meningospinal angiomatosis based on a spinal cord syndrome in the presence of a vascular skin nevus of the same metameric level. top of page [minervamedica.it]

• Clonus

  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 [researcher-app.com]

• Foot Drop

  Examination of the bilateral lower extremity showed marked steppage gait with bilateral external rotation deformities and bilateral foot drop. [casereports.bmj.com]

• Cervical Cord Compression

  The cervical cord opposite C4 vertebra appears thinned and shows mild increased intramedullary signal, representing compressive cord myelopathy. [birpublications.org]

• Incontinence

  A 42-year-old man presented with an acute onset of gait disturbance, low-back pain, and urinary incontinence. [ncbi.nlm.nih.gov]

  Cauda equina syndrome can present with severe back pain with saddle anesthesia accompanied by a disturbance of bowel and bladder function (overflow incontinence and poor rectal tone). [healio.com]

• Urinary Incontinence

  A 42-year-old man presented with an acute onset of gait disturbance, low-back pain, and urinary incontinence. [ncbi.nlm.nih.gov]

• Overflow Incontinence

  Cauda equina syndrome can present with severe back pain with saddle anesthesia accompanied by a disturbance of bowel and bladder function (overflow incontinence and poor rectal tone). [healio.com]

Workup for each patient included pre- and postoperative contrast-enhanced MR imaging with vascular sequencing and spinal angiography. [ncbi.nlm.nih.gov]

On radiological workup, an intradural perimedullary complex spinal arteriovenous fistula was seen with presence of subarachnoid haemorrhage in the spinal canal and compressive myelopathy. [casereports.bmj.com]

A thorough workup is recommended by a vascular anomaly specialist after identification. [hemangioma.com]

In such cases, a brain subarachnoid hemorrhage workup is often the starting point. Head CT shows hemorrhage, and a bloody tap will show up if one does an LP. In these cases, a cerebral workup is negative. Do not miss a spinal source! [neuroangio.org]

• Periostal Elevation

  Cobb chisel Cobb curet Cobb gouge Cobb method Cobb osteotome Cobb periosteal elevator Cobb retractor Cobb spinal elevator Cobb syndrome - cutaneous angiomas, usually in a dermatomal distribution on the trunk, associated with vascular abnormality of the [medical-dictionary.thefreedictionary.com]

• Gliosis

  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. [researcher-app.com]

• Ischemic Changes

  AVMs and arterio-venous fistula) may present with pain, ulceration, ischemic changes, bleeding, congestive heart failure, warm pink patches on the skin with an underlying vascular murmur or thrill and treated with surgical resection or embolization. [ncbi.nlm.nih.gov]

Management and treatment Treatment of the osteomuscular malformations involves embolisation (endovascular navigation and occlusion of the arteries feeding the malformation using a biological glue) and/or surgery. [orpha.net]

Her symptoms improved by combined treatment with liquid embolization and corticosteroid therapy. Although Cobb syndrome has been reported in older children, it is extremely rare in infants. [ncbi.nlm.nih.gov]

A systematic review of the literature on Cobb syndrome, with a focus on treatment and outcomes, is presented. Current treatment options include combinations of embolization, neurosurgical intervention, corticosteroid therapy, and radiotherapy. [pedneur.com]

We emphasize the role of microsurgery because it is the only treatment modality that offers (in selected cases of compact AVMs) complete removal of the malformation and can achieve very good results if diagnosis is followed by treatment in a timely manner [surgicalneurologyint.com]

Prognosis likely depends on the severity of the spinal pathology and the timeliness of diagnosis and intervention. Patients who present with rapidly progressing neurological deficits may have a worse prognosis. Early diagnosis is critical. [emedicine.medscape.com]

Prognosis The disease course is unpredictable and the lesions may remain asymptomatic for long periods of time. The documents contained in this web site are presented for information purposes only. [orpha.net]

Treatment and prognosis Patients should be referred to a neurosurgeon and an interventional radiologist for embolization and decompression. [radiopaedia.org]

[…] symptoms of this disorder may undergo several imaging studies such as MRI, Radiography, Angiography, and CT scans, as well as a thorough work-up by specialists in the fields of Neurology, Neurosurgery, and Interventional Radiology to ensure the best prognosis [nicklauschildrens.org]

Etiology The syndrome is not familial or hereditary and no chromosomal anomaly has been described. [orpha.net]

Summary Epidemiology Less than 100 cases of Cobb syndrome have been reported in the literature. There is no sex predilection. Cobb syndrome represents less than 15% of cases of spinal cord arteriovenous malformations. [orpha.net]

You might also be interested in Vascular medicine Occurrence/Epidemiology This section has been translated automatically. So far, < 150 cases have been described in the literature. Both sexes are equally frequently affected. [altmeyers.org]

Epidemiology Less than 100 cases of Cobb syndrome have been reported in the literature. There is no sex predilection. Cobb syndrome represents less than 15% of cases of spinal cord arteriovenous malformations. [rarediseases.info.nih.gov]

Although pathophysiology of this Syndrome is poorly understood and the correct management remains unknown, endovascular treatment has become the first choice for spinal arteriovenous malformations. [idexlab.com]

The pathophysiology is similar to spinal pial fistulas, presenting with cord venous congestion. Right T10 injection demonstrates premature opacification of a spinal vein (blue) via a fistula (purple) supplied by the anterior cerebral artery (red). [neuroangio.org]

However, selective spinal angiography facilitates understanding of the complex angioarchitecture and pathophysiology as well as the embolization procedure.11,12 Lumbar puncture is contraindicated in suspected spinal mass lesion especially spinal vascular [ncbi.nlm.nih.gov]

[…] anatomical variations are hypothesized to predispose to a “flap-valve” occlusion of the natural maxillary sinus ostium wherein the sinus becomes completely occluded allowing gas resorption and development of negative pressure in the sinus antrum. [11] Pathophysiology [eyewiki.aao.org]

Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge-Weber syndrome. Neurologist 2011;17(4):179–184. Crossref, Medline, Google Scholar 25 Welty LD. Sturge-Weber syndrome: a case study. [pubs.rsna.org]

Recognition of the associated cutaneous lesions with spinal lesions will allow for prevention and treatment of neurological symptoms. [casereports.bmj.com]

Multiple embolizations failed to prevent neurological deterioration, and the patient eventually became wheelchair dependent. [ncbi.nlm.nih.gov]

The spinal vascular lesions can cause neurologic deficits, including paralysis. [2, 3] Early recognition of the association between the vascular skin lesions and associated spinal lesions may prevent or minimize neurological sequela. [emedicine.medscape.com]

The embolization that occurred was to stop the continued bleeding to prevent a mass effect, and to reduce/eliminate the artereovenous malformation to prevent further embolic complications. [healio.com]

Preventive (prophylactic) treatment of migraines and headaches may be recommended and may include medications such as propranolol or verapamil. [rarediseases.org]