Flare-ups may be attributed to, or exacerbated by growth spurts, stress, exhaustion, exercise, standing or walking for too long, illness, infection, being accidentally knocked/hurt or injured, after surgery/anaesthetics, cold weather, electrical storms, and sudden changes in barometric pressure.CED may also affect internal organs, the liver and spleen, which may become enlarged. A loss of vision and/or hearing can occur if bones are adversely affected by the hardening in the skull. Hence proactive specialist check-ups, X-rays, diagnostic tests/scans, and regular blood tests are recommended on an annual basis to monitor the CED bony growth and secondary medical issues that may arise from this condition. Cause
Camurati-Engelmann disease is caused by autosomal dominant mutations in the gene TGFB1, localized at chromosome 19q13. Diagnosis
Classification
There are two forms:

Type 1 is associated with TGFB1
Type 2 is not associated with TGFB1Type 1 Camurati-Engelmann Disease is associated with an error occurring in the TGFB1 protein. Affected individuals shared a haplotype between D19S881 to D19S606. TGFB1 protein is encoded by the TGF-B1 gene, which occurs on chromosome 19q13.1-13.3. This protein is responsible for a multitude of functions, one of which includes regulating the function of osteoblasts and osteoclasts, which decreases bone resorption and increases bone formation. These functions can be affected by a series of mutations that occur on exon 4, near the carboxyl terminus of the latency associated peptide, or LAP. TGFB1 is expressed as a latent form, a mature form and a B1-LAP. Mutations to R218H affect the association of the B1-LAP and the mature form of TGFB1 by conformational changes to B1-LAP.
Ossification of the posterior longitudinal ligament (OPLL) is a process of fibrosis, calcification, and ossification of the posterior longitudinal ligament of the spine, that may involve the spinal dura. Once considered a disorder unique to people of Asian heritage, it is now recognized as an uncommon disorder in a variety of patients with myelopathy. Causes
The causes of OPLL are unknown. However, genetic and environmental factors appear to play a role in pathogenesis. OPLL may also be associated with diffuse idiopathic skeletal hyperostosis

Diagnosis
Myeolography, including post-myelographic CT is likely the most effective imaging study an accurate diagnosis. Treatment
Surgical management options include extensive cervical laminectomy with or without an additional posterior arthrodesis, anterior decompression and arthrodesis, and posterior cervical laminoplasty. Treatment decisions can be made based on a grading systems devised by Hirabayashi et al., supplemented by the Nurick myelopathy classification system. Prognosis
Most patients suffer from only mild symptoms. Symptoms typically last approximately 13 months. Of patients without myelopathy at initial presentation, only 29% of them will develop myelopathy within 30 years. Epidemiology
The age range of patients with OPLL is from 32 to 81 years (mean = 53), with a male predominance. Prevalence is higher in those of Japanese or Asian ancestry (2-3.5%)  and rarer in other racial groups (0.16%). Schizophrenia patients in Japan may have as high as 20% incidence. References


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