title: Management of cervical myelopathy due to ossification of posterior longitudinal ligament in a patient with Alström syndrome.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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 function. Follow-up MRI showed substantial enlargement of the spinal canal with improved space available for the spinal cord. The rarity of the syndrome, cervical myelopathy due to ossified posterior longitudinal ligament as a disease phenotype and the treatment considerations for performing a posterior cervical decompression have been discussed in this Grand Rounds' case presentation.
authors.affiliation: Centre for Spinal Studies and Surgery, Nottingham University Hospitals, Queen's Medical Centre, Nottingham, NG7 2UH, UK. rishiortho@gmail.com
authors.forename: Rishi Mugesh
authors.initials: RM
authors.lastname: Kanna
authors.forename: Daniela
authors.initials: D
authors.lastname: Gradil
authors.forename: Bronek M
authors.initials: BM
authors.lastname: Boszczyk
mesh_terms: D056769:Alstrom Syndrome / Q000150:complications*; D002574:Cervical Vertebrae; D019299:Decompression, Surgical; D006801:Humans; D008297:Male; D008875:Middle Aged; D017887:Ossification of Posterior Longitudinal Ligament / Q000150:complications* / Q000601:surgery; D013117:Spinal Cord Compression / Q000209:etiology / Q000601:surgery; D013118:Spinal Cord Diseases / Q000209:etiology*