We describe symptomatic spinal cord compression associated with pseudohypoparathyroidism (PHP) in a young female patient and reviewed similar cases previously reported in the literature. The characteristics of these cases were analyzed from etiology, clinical subtypes, symptoms, treatment, and prognosis. Neurological examination revealed functional upper extremities with bilateral lower extremity paraplegia. Laboratory tests showed hypocalcemia, hyperphosphatemia, and elevated parathyroid hormone; high-throughput sequencing showed a heterozygous GNAS mutation in exon 12, specifically c.1006C > T (p.R336W). Imaging findings showed multilevel spinal stenosis with significant spinal cord compression at the T2-T3 level. Seventeen cases with similar characteristics were reviewed. We found that the primary clinical manifestation of these patients was bilateral lower extremity spastic paraplegia. Multilevel spinal cord compression was commonly observed, especially at the lower cervical and upper thoracic spinal cord. Most of the patients had poor surgical treatment outcome and prognosis. Clinicians should be aware of paraplegia due to spinal cord compression as a rare neurological complication in patients with PHP. Early diagnosis and treatment of PHP is one basis for preventing severe spinal cord-related complications.

Compressive myelopathy in severe angular kyphosis is rare and challenging for surgical treatment. The goal of this retrospective study was to report a series of ten patients with compressive myelopathy in severe angular kyphosis and the results of surgical decompression and correction of kyphosis.
Between 2010 and 2014, 10 patients were surgically treated for severe angular kyphosis with a progressive onset or a sudden onset of paraplegia in investigator group. In these ten patients (seven males and three females), the etiologic diagnosis included eight cases of congenital kyphosis and two of neurofibromatosis; the distribution of spine level was from C5 to T11; the duration from onset until surgery ranged from 1 to 120 months; follow-up ranged from 12 to 26 months (mean 18.5 months); the kyphosis angle of the patients ranged from 50° to 180°. Magnetic resonance imaging demonstrated the spinal cord thinning and compression at apex in most of patients. All patients underwent decompressive surgery by single-stage posterior vertebral column resection or both anterior corpectomy fusion and posterior fixation. Neurological status was evaluated using the ASIA impairment classification and the motor score.
Postoperatively, all patients had different kyphosis correction rate from 24 to 100 %. Nine patients showed neurological improvement; one patient showed no improvement. Among them, one sudden onset ASIA A adolescent paraplegic patient improved to ASIA E within 1 year of follow-up. One ASIA C adolescent paraplegic patients deteriorated neurologically to ASIA A after surgery and improved to ASIA D with 12-month follow-up.
Compressive myelopathy in severe angular congenital kyphosis is usually occurred high incidence rate at apex of upper thoracic spine (T1-T4). The duration from onset of paraplegia until surgery and the severity of paraplegia before surgery are two key factors for neurological prognosis after surgery.