title: Clinical presentation and management of proatlas segmentation defect presenting with palatal myoclonus: case report.
abstract: Clinical presentation of craniovertebral junction disorders may range from acute catastrophic neurological deficits to insidious signs and symptoms that may mask the underlying etiology. Prompt recognition and treatment is essential to avert long-term neurological morbidity. Proatlas segmentation disorders are a rare group of developmental disorders involving the craniocervical junction. Abnormal bony segmentation leads to malformed bony structures that can in turn lead to neurological deficits through bony compression of the cervicomedullary junction. This report details a proatlas segmentation defect presenting as palatal myoclonus, a rare movement disorder. The clinical presentation, surgical management, and neuroanatomical basis for the disorder is presented. This report highlights the myriad clinical presentations of craniovertebral disorders and emphasizes a rare but treatable etiology for palatal myoclonus.
authors.affiliation: Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa.
authors.forename: Raheel
authors.initials: R
authors.lastname: Ahmed
authors.affiliation: Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa City, Iowa.
authors.forename: Arnold H
authors.initials: AH
authors.lastname: Menezes
mesh_terms: D001270:Cervical Atlas / Q000002:abnormalities*; D002648:Child; D003251:Constriction, Pathologic / Q000150:complications / Q000601:surgery; D019299:Decompression, Surgical; D004569:Electroencephalography; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009207:Myoclonus / Q000209:etiology*; D009809:Odontoid Process / Q000002:abnormalities*; D010160:Palate, Soft / Q000503:physiopathology*; D012886:Skull / Q000002:abnormalities; D013115:Spinal Canal / Q000473:pathology* / Q000601:surgery*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
keywords: CVJ = craniovertebral junction; EPT = essential palatal tremor; GMT = Guillain-Mollaret triangle; SPT = symptomatic palatal tremor; craniovertebral junction; palatal myoclonus; proatlas segmentation anomaly; spine; symptomatic palatal tremor