Idiopathic hypertrophic cervical pachymeningitis – A rare report
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.190229
Source of Support: None, Conflict of Interest: None
Spinal idiopathic hypertrophic pachymeningitis (IHP) is a rare disease causing chronic inflammatory hypertrophy of the dura mater. It is an extremely rare cause of spinal cord compression. This process can be present throughout the entire spine but is commonly reported in the cervical and thoracic spine., This entity is more commonly described in the cranium, mainly in the base of the skull and posterior fossa, and often presents with multiple cranial nerve defects. Spinal IHP commonly presents in the 6th and 7th decades and can be either solitary or multiple, and diffuse or nodular. The natural history is progression from local pain to radiculopathy and an eventual myelopathy. The etiology is unknown, but trauma, infection, tumors, and various autoimmune diseases have been implicated.
We report the cases of a
42-year-old male patient who presented with neck pain radiating to the outer aspect of the left arm for the last 2 years. Gradually, he also developed ascending paresthesia of both the upper and lower limbs. This was accompanied by weakness of the distal muscles of both upper and lower limbs and stiffness, along with slowing of gait. There was no sphincteric involvement. On examination, he had gross wasting of intrinsic muscles of both hands with symmetrical spastic quadriparesis Medical Research Council grade 4/5. The deep tendon jerks were exaggerated and planters were extensor. There was reduced sensation of all modalities below the C4 level. A clinical diagnosis of a high cervical compressive myelopathy was made and the patient was investigated.
Contrast magnetic resonance imaging (MRI) of the cervical spine showed diffuse circumferential, thickened, and mildly enhancing pachymeninges extending from the C2 to C5 level compressing the cord. There was a patchy hyperintense signal within the cord parenchyma from C3–C6 segments on T2 weighted imaging suggestive of myelomalacia [Figure 1].
The routine blood tests, X-ray of the chest, serum angiotensin converting enzyme levels, erythrocyte sedimentation rate, cerebrospinal fluid routine examination, a denosine deaminase level and polymerase chain reaction for tuberculosis, and Venereal Disease Research Laboratory fluorescent treponemal antibody absorption were normal. MRI of the brain showed no meningeal involvement and was grossly normal.
A diagnosis of idiopathic hypertrophic cervical spinal pachymeningitis was made and the patient was taken up for surgery. A C2–C5 laminoplasty with durotomy was done. The dura was grossly thickened causing compression of the underlying spinal cord. The sleeve of the thickened dura was excised, and subsequently water-tight lax duroplasty was done [Figure 2].
During the immediate postoperative period, the patient noticed significant improvement in his paresthesia and neck pain. However, his stiffness improved gradually and he was discharged on the 5th postoperative day.
Postoperative MRI of the cervical spine showed adequate decompression of the spinal cord [Figure 3]. Pathological sections of the dura showed dense fibrous tissue with focal aggregates of lymphocytes and plasma cells [Figure 4]. There were no granulomas or any evidence of vasculitis. Special stains were negative for fungi, bacteria, and mycobacteria. These histological features supported the diagnosis of spinal IHP.
Spinal IHP is a chronic inflammatory fibrosis of the dura mater and is an extremely rare cause of nerve root and spinal cord compression. The first description of IHP was given by Charcot and Joffroy in 1869. The cranium and spinal cord can be involved concomitantly;
however, in our case there was an isolated spinal cord involvement.
Spinal IHP is a diagnosis of exclusion. Various collagen and autoimmune disorders need to be ruled out as a cause of thickened dura. Therefore, a thorough workup is required before labelling it as IHP. Radiographic and pathological confirmation, along with exclusion of other known etiologies, are the cornerstone for its diagnosis.
The presentation of spinal IHP can vary from radicular symptoms to progressive myelopathy and involvement of sphincters in the later stages. At present, decompressive surgery for symptomatic compressive lesions and corticosteroid therapy for the inflammatory cascade remain the mainstay of treatment. Surgery serves the dual purpose of achieving an immediate decompression of the spinal cord and in obtaining a tissue diagnosis.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]