Management of cervical myelopathy due to ossification of posterior longitudinal ligament in a patient with Alström syndrome.

European Spine Journal : Official Publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
Rishi Mugesh KannaBronek M Boszczyk

Abstract

Alström syndrome (AS) is a rare autosomal recessive genetic disorder with multisystemic involvement characterised by early blindness, hearing loss, obesity, insulin resistance, diabetes mellitus, dilated cardiomyopathy, and progressive hepatic and renal dysfunction. The clinical features, time of onset and severity can vary greatly among different patients. Many of the phenotypes are often not present in infancy but develop throughout childhood and adolescence. Recessively inherited mutations in ALMS1 gene are considered to be responsible for the causation of AS. Musculoskeletal manifestations including scoliosis and kyphosis have been previously described. Here, we present a patient with AS who presented with cervical myelopathy due to extensive flowing ossification of the anterior and posterior longitudinal ligaments of the cervical spine resulting in cervical spinal cord compression. The presence of an auto-fused spine in an acceptable sagittal alignment, in the background of a constellation of medical comorbidities, which necessitated a less morbid surgical approach, favored a posterior cervical laminectomy decompression in this patient. Postoperatively, the patient showed significant neurological recovery with improved fun...Continue Reading

References

Aug 1, 1987·The Journal of Bone and Joint Surgery. British Volume·P C McAfeeH H Bohlman
Aug 26, 1998·European Journal of Radiology·S EharaK Yamazaki
Jan 8, 1999·Spine·T J Albert, A Vacarro
Mar 30, 2005·Archives of Internal Medicine·Jan D MarshallPatsy M Nishina
Oct 18, 2007·European Journal of Human Genetics : EJHG·Jan D MarshallJürgen K Naggert
Dec 25, 2007·Orphanet Journal of Rare Diseases·Tisha JoyPaul N Durrington
Dec 3, 2009·Journal of Neurosurgery. Spine·David E GwinnMichael P Steinmetz

❮ Previous
Next ❯

Citations

May 1, 2012·European Spine Journal : Official Publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society·Wen Yuan

❮ Previous
Next ❯

Related Concepts

Related Feeds

Alstrom Syndrome

Alstrom syndrome is a rare disorder characterized by early obesity, loss of central vision, diabetes mellitus, hearing loss, and short stature. Here is the latest research.

Auditory Perception

Auditory perception is the ability to receive and interpret information attained by the ears. Here is the latest research on factors and underlying mechanisms that influence auditory perception.

Cardiomyopathy

Cardiomyopathy is a disease of the heart muscle, that can lead to muscular or electrical dysfunction of the heart. It is often an irreversible disease that is associated with a poor prognosis. There are different causes and classifications of cardiomyopathies. Here are the latest discoveries pertaining to this disease.