A rare association of dysphagia and cervical compressive myelopathy in diffuse idiopathic skeletal hyperostosis
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.198231
Source of Support: None, Conflict of Interest: None
A 45-year-old female patient came to our hospital, with the chief compliants of numbness in both the upper limbs for the last 3 months and weakness in all four limbs, along with difficulty in swallowing for the last 1 month.
Neurological examination revealed dysphagia with no cranial nerve abnormality. The power in both the upper limbs was 3/5 and in lower limbs was 4/5, as per the Medical Research Council (MRC) scale. The tone was increased in all four limbs with a spastic gait. Touch, pain, temperature, and vibratory sensations were decreased in all the four limbs. Deep tendon reflexes were exaggerated in all the four limbs with bilateral extensor planter reflexes. The bladder and bowel functions were normal. The investigations were within normal range.
Lateral X-ray and computed tomography (CT) of the cervical spine revealed bony ankylosis from C2 to C6 vertebrae without any fractures or dislocation, along with ossification of the anterior as well as posterior longitudinal ligament [Figure 1] and [Figure 2]. X-ray of the sacroiliac joint was normal.
Magnetic resonance imaging (MRI) of the cervical spine showed cervical stenosis from C2 to C6 level, with both anterior and posterior compression and hyperintense signal in the cervical cord at C6 level suggestive of myelopathy. Large anterior osteophytes, most prominent at the C2-C3 and C5-C6 levels, were seen, causing significant impingement over the esophagus [Figure 3].
According to the radiographic criteria of Forestier, Resnick, and Niwayama, the diagnosis of diffuse idiopathic skeletal hyperostosis (DISH) with cervical myelopathy was made.
In view of progressive neurological deterioration, the patient was subjected to surgery. Posterior decompressive laminectomy along with anterior drilling of osteophytes was done. There was no complication during surgery or in the postoperative period. The neurological function remarkably improved 1 month after surgery. Dysphagia, bilateral clumsiness in fingers, and spastic gait improved significantly; the patient was able to carry out her daily activities without assistance.
DISH or Forestier's disease is an idiopathic spondyloarthropathy of the spine. It is characterized by spine ankylosis and enthesopathy, i.e. ossification of the ligaments and entheses. Involvement of the cervical spine is less frequent than involvement of the lumbar or thoracic spine (78%, 90%, and 97% respectively).
DISH, also known as ankylosing hyperostosis, is characterized by calcification and ossification of soft tissues, mainly ligaments and entheses. This condition was described by Forestier and Rotes-Querol in the 1950s. DISH is most prevalent in persons over 50 years of age. Studies showed that prevalence is as high as 15% in women and 25% in men over the age of 50 years, and 26% in women and 28% in men over the age of 80 years. Most patients with DISH are asymptomatic unless they experience a precipitating trauma. The exact etiology is not known but it can be associated with various metabolic disorders, especially obesity and insulin-dependent diabetes mellitus.
Forestier, and Resnick and Niwayama, described the following three criteria for the radiological diagnosis of DISH: 1. Flowing ossification along the anterolateral aspect of at least four contiguous vertebrae; 2. Preservation of the disc height in the involved vertebral segment; the relative absence of significant degenerative changes, such as marginal sclerosis in the vertebral bodies, or vacuum phenomenon; and, 3. Absence of facet-joint ankylosis; absence of sacroiliac erosion, sclerosis, or intra-articular osseous fusion.
The findings in this case were consistent with these criteria. The thoracic spine, especially in the middle and lower part, is the most frequently involved section, followed by the lumbosacral spine. The disease rarely affects the cervical spine. In our case, however, the cervical spine was involved.
This disease is usually asymptomatic and is diagnosed incidentally during the imaging studies. The most frequent clinical manifestation in the cervical spine is dysphagia, and other symptoms are neck pain, back pain, and gait disturbances., All these features were present in our case.
Neurological manifestations in cervical DISH are rare. Neurological deficits occur due to myelopathic changes caused by cervical canal stenosis., In our case, the cause of neurological deficit was cervical canal stenosis at the C2-C6 level resulting in myelopathy at the C6 level.
The management of patients with DISH is mostly conservative including nonsteroidal anti-inflammatory drugs and steroid therapy. Surgery is indicated in case of progressive dysphagia or neurological deficit, and surgical approaches depend on the location of pathology. If osteophytes are impinging on anterior structures, surgical resection can be palliative. In case of multilevel cervical stenosis and myelopathy with preserved lordosis, laminoplasty or laminectomy may be indicated. In other cases, anterior decompression and stabilization may be necessary. In our case, in view of the cervical canal stenosis and myelopathic changes, the patient was subjected to surgery. After the surgery, neurological functions improved significantly and the patient was able to carry out her daily activities without assistance.
Epstein  reported two cases of DISH with dysphagia and/or cervical myelopathy. Here, only one case of DISH presented with simultaneous dysphagia and cervical myelopathy. To the best of our knowledge, this is only the second report of simultaneous occurrence of dysphagia and cervical myelopathy in DISH requiring surgical intervention. Symptomatic cervical DISH causing dysphagia is fairly common; however, the simultaneous presence of dysphagia and compressive cervical myelopathy is rare. Prompt and appropriate diagnosis and treatment is necessary to avoid worsening of neurological deficits in these patients.
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[Figure 1], [Figure 2], [Figure 3]