Idiopathic Hypertrophic Spinal Pachymeningitis : Report of Two Cases and Review of the Literature

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory disease characterized by hypertrophic inflammation of the dura mater and various clinical courses that are from myelopathy. Although many associated diseases have been suggested, the etiology of IHSP is not well understood. The ideal treatment is controversial. In the first case, a 55-year-old woman presented back pain, progressive paraparesis, both leg numbness, and voiding difficulty. Initial magnetic resonance imaging (MRI) demonstrated an anterior epidural mass lesion involving from C6 to mid-thoracic spine area with low signal intensity on T1 and T2 weighted images. We performed decompressive laminectomy and lesional biopsy. After operation, she was subsequently treated with steroid and could walk unaided. In the second case, a 45-year-old woman presented with fever and quadriplegia after a spine fusion operation due to lumbar spinal stenosis and degenerative herniated lumbar disc. Initial MRI showed anterior and posterior epidural mass lesion from foramen magnum to C4 level. She underwent decompressive laminectomy and durotomy followed by steroid therapy. However, her conditions deteriorated gradually and medical complications occurred. In our cases, etiology was not found despite through investigations. Initial MRI showed dural thickening with mixed signal intensity on T1- and T2-weighted images. Pathologic examination revealed chronic nonspecific inflammation in both patients. Although one patient developed several complications, the other showed slow improvement of neurological symptoms with decompressive surgery and steroid therapy. In case of chronic compressive myelopathy due to the dural hypertrophic change, decompressive surgery such as laminectomy or laminoplasty may be helpful as well as postoperative steroid therapy.

Idiopathic Hypertrophic Spinal Pachymeningitis: A Case Report

Idiopathic hypertrophic pachymeningitis (IHP) is a rare, chronic nonspecific and granulomatous inflammatory disorder of the dura with unknown etiology. The diagnosis can be established by open biopsy and exclusion of all other specific granulomatous and infectious diseases. We report a typical case of spinal IHP occurring in a long segment of cervical and thoracic dura from C6 to T8. The patient was 56-yr-old female, who had been suffered from pain on her upper back and both arms for 3 months and recent onset motor weakness of both legs. During the 9 months of follow-up period, she experienced the improvement of her neurologic symptoms with combined therapy of partial excision and corticosteroid medication. Since early surgical intervention and subsequent pulse ste-roid therapy are mandatory for this disease to avoid irreversible damage of nervous system, the identification of this unique disease entity is essential on frozen diagnosis. A few cases have been reported in Korean literature.

Nonspecific inflammation in the face / 대한구강악안면방사선학회지

Patient with compalints of swelling, pain in the maxillary region and discomfort visited Seoul National University Dental Hospital in August last year. Clinical examination and diagnostic imagings implied he was suffered from fungal h yphal infection but no causative fungus was found by the histopathologic and microbiologic investigation. Therefore he w as diagnosed with nonspecific inflammation. But as yet, we do think this case is very similar to some kinds of mucomycos is. So we presented this case for more thorough discussion. Following are founded in the examination. 1. Patient had sufferd from Diabetes mellitus and complained of stuffness, headache, swelling in buccal cheeks and pare sthesia. And we found more maxillary bony destruction and ulcer with elevated margin in the palate by clinical examinati on. 2. In the first visit, Plain films revealed general bony destruction of the maxilla, radiopaqueness in the sinonasal ca vities. CT and MRI showed soft tissue mass filled in the paranasal sinus except frontal sinus and bony destruction in in volved bones. 3. No causative bacteria and fungus was found in the biopsy and microbiologic cultures. 4. Caldwell-Luc operation and curettage were carried and antibiotics were taken for 4 months. But now he was worse than in the past. 5. In the second visit, involvement of orbit, parapharyngeal sinus, clivus, cavernous sinus and middle cranial fossa we re seen clearly in the CT and MRI.

Nonspecific inflammation in the face / 대한구강악안면방사선학회지

Patient with compalints of swelling, pain in the maxillary region and discomfort visited Seoul National University Dental Hospital in August last year. Clinical examination and diagnostic imagings implied he was suffered from fungal h yphal infection but no causative fungus was found by the histopathologic and microbiologic investigation. Therefore he w as diagnosed with nonspecific inflammation. But as yet, we do think this case is very similar to some kinds of mucomycos is. So we presented this case for more thorough discussion. Following are founded in the examination. 1. Patient had sufferd from Diabetes mellitus and complained of stuffness, headache, swelling in buccal cheeks and pare sthesia. And we found more maxillary bony destruction and ulcer with elevated margin in the palate by clinical examinati on. 2. In the first visit, Plain films revealed general bony destruction of the maxilla, radiopaqueness in the sinonasal ca vities. CT and MRI showed soft tissue mass filled in the paranasal sinus except frontal sinus and bony destruction in in volved bones. 3. No causative bacteria and fungus was found in the biopsy and microbiologic cultures. 4. Caldwell-Luc operation and curettage were carried and antibiotics were taken for 4 months. But now he was worse than in the past. 5. In the second visit, involvement of orbit, parapharyngeal sinus, clivus, cavernous sinus and middle cranial fossa we re seen clearly in the CT and MRI.