Hypertrophic Spinal Pachymeningitis Research Articles

Hypertrophic Spinal Pachymeningitis in a Patient with Chronic Antineutrophil Cytoplasmic Antibody–associated Nephritis: A Case Report

Hypertrophic Spinal Pachymeningitis in a Patient with Chronic Antineutrophil Cytoplasmic Antibody–associated Nephritis: A Case Report

Idiopathic hypertrophic spinal pachymeningitis with compressive myelopathy: a case report

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare condition characterized by chronic progressive diffuse inflammatory fibrosis in the dura mater of the spinal cord. Depending on its extent or severity, IHSP may cause symptoms by compressing the spinal cord. We present a case of IHSP across the cervical dura mater in a 78-year-old male patient. The patient visited the emergency department for right shoulder pain a month before admission, weakness in the right upper extremity a week before admission, and sudden quadriparesis on the day of admission. A neurological examination of the motor power of the extremities indicated grade IV quadriparesis and grade I hand grasp power in both hands. Hypoesthesia under the T1 dermatome was evaluated. Cervical magnetic resonance imaging (MRI) with gadolinium enhancement showed diffuse thickening of the anterior and posterior dura through the C2 to C5 levels, causing central canal stenosis and compressive myelopathy. Immediate decompressive laminoplasty was performed. A biopsy specimen of the thickened dura mater was obtained during surgery, and IHSP was diagnosed on the basis of the results of the pathological examination. High-dose steroid therapy was administered after decompressive surgery, and follow-up MRI showed radiological improvement in the lesion area.

Clinical analysis of 15 cases with myeloperoxidase antineutrophil cytoplasmic antibody associated hypertrophic pachymeningitis

To study the clinical features of myeloperoxidase(MPO) antineutrophil cytoplasmic antibody (ANCA) associated hypertrophic pachymeningitis (HP). Clinical data of 15 cases diagnosed with MPO-ANCA vasculitis complicated with HP were retrospectively analyzed. Nine cases were males and the other 6 were females, with an average age of (58±8) years. All cases presented with chronic headache. Contrast-enhanced magnetic resonance imaging (MRI) scan showed local or diffused thickening of cerebral and/or spinal dura matter while brain parenchyma were normal. Nine cases developed multiple cranial nerve paralysis, with trigeminal nerve and auditory nerve involved most commonly. The main clinical manifestations were facial pain, hearing loss and tinnitus. Two cases were complicated with hypertrophic spinal pachymeningitis (HSP) and 4 cases were complicated with pulmonary diseases. Positive serum perinuclear pattern ANCA (pANCA) and MPO could be found in all cases, positive serum IgG4 was seen in two patients. erythrocyte sedimentation rate(ESR;25-116 mm/1h) and C-reactive protein (CRP;29.02-146.00 mg/L) were both elevated in 14 cases. Nine cases had elevated intracranial pressure[180-235 mmH2O (1 mmH2O=0.009 8 kPa)] and abnormal protein level (457.6-3710.0 mg/L) in cerebrospinal fluid. Six cases were treated with glucocorticoids (prednisone 20-60 mg/d) and 9 cased with glucocorticoids and immunosuppressants (methotrexate 15 mg/week or cyclophosphamide 100 mg/d po). All patients achieved remission. MPO-ANCA associated HP is a special type of central nervous system involvement in ANCA associated vasculitis (AAV). It rarely involves the lung or kidney. Steroids and immunosuppressive agents are effective. In HP with unknown underlying diseases, it is suggested to screen ANCA and IgG4 tests for AAV or IgG4-related disease.

Idiopathic hypertrophic pachymeningitis as a rare cause of spinal cord compression

Idiopathic hypertrophic pachymeningitis (IHP) is a rare inflammatory disease characterized by inflammation and hypertrophy of the dura mater. Spinal form of IHP is extremely rare. We report an idiopathic hypertrophic spinal pachymeningitis case, a 67-year-old female who presented with neck and upper back pain with progressive paraparesis and sensory level. Her magnetic resonance imaging cervico-dorsal spine showed abnormal epidural hypointensity on T2-weighted images from C5 to T4 with significant cord compression and intramedullary cord signal changes at T2 and T3 levels. The lesional biopsy was done suggestive of chronic inflammatory changes. She was treated with steroids and was able to walk unaided.

Rare Causes of Hypertrophic Spinal Pachymeningitis Primarily Identified on Spinal MRI.

Rare Causes of Hypertrophic Spinal Pachymeningitis Primarily Identified on Spinal MRI.

Hypertrophic spinal pachymeningitis caused by ANCA-associated vasculitis revealed by 18F-FDG PET/CT

Rationale:Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) can involve the central nervous system in estimatedly 15% of patients. Hypertrophic pachymeningitis causes inflammatory hypertrophy of the cranial or spinal dura mater and patients present with various neurological deficits. ANCA-associated hypertrophic spinal pachymeningitis has rarely been reported in literature. We report a case of AAV presenting with hypertrophic spinal pachymeningitis detected by 18F-FDG PET/CT.Patient concerns:A 66-year-old woman diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis developed back pain, bilateral lower limb weakness, dysuria, and dysporia 1 month ago.Diagnosis:Contrast-enhanced MRI showed thickening and enhancement of the dura mater in the thoracic cord. Intraspinal hypermetabolism in the corresponding region was observed on 18F-FDG PET/CT. The patient was finally diagnosed with ANCA-associated hypertrophic spinal pachymeningitis.Interventions:The patient was treated with a higher dose of prednisone and cyclophosphamide.Outcomes:After 2-week treatment, the patient's neurological symptoms improved rapidly and laboratory findings were ameliorated. A repeated contrast-enhanced MRI showed partial improvement of the disease in the thoracic cord.Lessons:18F-FDG PET/CT and contrast-enhanced MRI can aid in the clinical diagnosis and surveillance in AAV-associated hypertrophic spinal pachymeningitis and potentially facilitate early recognition and intervention to prevent irreversible neurological impairment.

The Idiopathic Hypertrophic Spinal Pachymeningitis

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory condition characterized by chronic inflammatory hypertrophy of the dura mater. It can involve the entire spine. However, most cases are reported in the cervical and thoracic spine. It can progress from local pain to radiculopathy and eventually develop myelopathy. The aetiology of IHSP is not known. However, it has been suggested to be associated with many diseases. Here we report a case of IHSP in 21-year-old female who presented with paraplegia. The diagnosis was made on MRI Spine and histopathological examination. It was treated with surgical decompression, steroid therapy and patient improved gradually.

Myeloperoxidase-Antineutrophil Cytoplasmic Antibody Positive Hypertrophic Spinal Pachymeningitis at the Cervicothoracic Junction: A Case Report.

Myeloperoxidase-Antineutrophil Cytoplasmic Antibody Positive Hypertrophic Spinal Pachymeningitis at the Cervicothoracic Junction: A Case Report.

Hypertrophic Pachymeningitis in Chinese Patients: Presentation, Radiological Findings, and Clinical Course.

Background Hypertrophic pachymeningitis (HP) is generally regarded as a rare inflammatory disease, which results in a diffuse thickening of the dura mater. We retrospectively collected data from patients with HP. Methods A total of 16 patients with HP were included in our study. The clinical features, laboratory evaluation, imaging findings, treatment, and outcome were reviewed. Results Of the 16 cases, half were male, with a mean age of 52.6 ± 13.2 years. The mean duration from onset to diagnosis was 8.6 months. The most frequent presenting symptoms in HP cases were a recurrently chronic headache (81.3%) and multiple cranial nerve injury (50%). Antineutrophil cytoplasmic antibody- (ANCA-) related HP was found in 5 cases and IgG4-related HP in 1 case. The intracranial pressure was elevated in 4 cases. The cerebrospinal fluid (CSF) had lymphocytosis in 5 cases and increased protein in 12 cases. Immunoglobulins (IgG, IgA, and IgM) and protein showed linear relationships in the CSF. On magnetic resonance imaging (MRI), localized or diffuse dura maters were thickened in all cases. HP combined with subacute subdural hemorrhage or hypertrophic spinal pachymeningitis was also observed in individual cases. Biopsy of the dura mater in one case showed amounts of inflammatory cells infiltrating, with an increased percentage of IgG4-positive plasma cells. Of all cases referring to glucocorticoid treatment, the symptoms have improved significantly in 10 cases. In other 6 cases, mycophenolate mofetil or azathioprine was added. All patients showed clinical improvement at the follow-up visits. Conclusion The clinical characters of HP are chronic onset, recurrently chronic headache, and multiple cranial nerves paralysis. Inflammatory changes in CSF caused by intrathecal synthesis of immunoglobulin, characteristic dural enhancement on MRI, and pathologic biopsy are all helpful for diagnosis. The addition of immunosuppressant, especially mycophenolate mofetil, is a good choice for steroid-resistance HP.

ANCA-Associated Hypertrophic Spinal Pachymeningitis Presenting with Longitudinally Extensive Transverse Myelitis: A Case Report.

ANCA-Associated Hypertrophic Spinal Pachymeningitis Presenting with Longitudinally Extensive Transverse Myelitis: A Case Report.

Idiopathic Hypertrophic Dorsal Pachymeningitis - Imaging Features

Idiopathic Hypertrophic Spinal Pachymeningitis (IHSP) is a rare disease causing chronic inflammatory hypertrophy of the dura mater. It may be idiopathic or secondary to other diseases. 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 dorsal spine. IHSP is a diagnosis of exclusion since it is associated with trauma, infection, and autoimmune diseases. We describe a rare case of Idiopathic Hypertrophic Dorsal Pachymeningitis (IHDP) in a 42 -year -old woman presenting with progressive weakness of both lower limbs for the past 2 years and outline the important clinical and radiographic features of the disease.

Comparative analysis between hypertrophic spinal pachymeningitis and epidural meningioma

Comparative analysis between hypertrophic spinal pachymeningitis and epidural meningioma

Effective Response of Methotrexate for Recurrent Idiopathic Hypertrophic Spinal Pachymeningitis.

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a chronic progressive and diffuse inflammatory fibrosis of the spinal dura mater. Though treatment of IHSP is surgical decompression with steroid therapy, treatment for recurrent IHSP is controversial. Our patient was diagnosed with IHSP based on magnetic resonance imaging (MRI) and underwent laminectomy for decompression following steroid pulse therapy. Despite maintenance of steroid therapy, the patient experienced 3 recurrences. As an alternative immunosuppressant medication, methotrexate was introduced with low-dose steroid. Fortunately, the symptom was resolved, and a decrease of dura thickening was revealed on MRI. We present the case and suggest that methotrexate might be an effective treatment modality for recurrent IHSP.

Teaching Neuro Images : Idiopathic hypertrophic spinal pachymeningitis mimicking epidural lymphoma

A 41-year-old man presented with bilateral leg numbness and paraparesis for 3 months. MRI showed a circumferential long epidural mass extending from T2 to T4 level (figure 1). Histologic examination demonstrated dense fibrous tissue with inflammatory infiltrate (figure 2). By exclusion of trauma, infectious diseases, and autoimmune diseases, idiopathic hypertrophic spinal pachymeningitis (IHSP) was diagnosed.

Idiopathic Hypertrophic Spinal Pachymeningitis.

A 63-year-old man revealed a four-month history of muscle weakness of the lower limbs, hypoesthesia of the L5 and S1 area and ischuria. On MRI, the spinal cord was compressed by an encircled mass, which showed hypointensity on T1- and T2-weighted images with gadolinium enhancement at the Th11 to Th12 vertebra. Because of the rapid progression of myelopathy, posterior decompression was performed and idiopathic hypertrophic spinal pachymeningitis (HSP) was finally diagnosed. The patient's neurological signs markedly improved with postoperative corticosteroid treatment. Idiopathic HSP is a clinical emergency and early surgical intervention is essential to prevent irreversible damage to the nervous system.

Hypertrophic Spinal Luetic Pachymeningitis: Neurosurgical Treatment. Case Report and Review of the Literature

Case descriptions of hypertrophic spinal pachymeningitis with compressive myelopathy are relatively rare in the literature. In recent years their number tends to increase as a result of the wider application of magnetic resonance imaging, the gold diagnostic standard for spinal pathology. Meningeal hyperplasia with reduction of the spinal subdural space is usually related to infectious diseases or considered idiopathic. Neurosurgical interventions performed in similar cases not only have a decompressive therapeutic effect, but also contribute to the diagnostic process by providing material for neuropathological assessment. The optimal surgical technique should be determined according to the specific clinical case. Methods of choice include laminectomy with excision of adhesions, aiming at spinal and root decompression, microsurgical technique (adhesiolysis) under optic magnification, and endoscopic subarachnoepidurolysis. We present the case of a 45-year-old woman with hypertrophic spinal luetic pachymeningitis and compressive myelopathy at the thoracic level, who underwent neurosurgical treatment: laminectomy at levels C7 to Th4 with excision of subdural adhesions, decompression of the spinal cord, and plastic reconstruction of the dura mater. Very good recovery of bladder functions and gait was achieved. The beneficial effect persisted on follow-up after 6 and 12 months.

A case of overlap syndrome with systemic sclerosis and Sjögren syndrome presenting acute progressive quadriplegia due to hypertrophic spinal pachymeningitis

A case of overlap syndrome with systemic sclerosis and Sjögren syndrome presenting acute progressive quadriplegia due to hypertrophic spinal pachymeningitis

Idiopathic Hypertrophic Spinal Pachymeningitis: A Rare Entity

We describe a case of idiopathic hypertrophic spinal pachymeningitis in a 62 -year -old gentleman presenting with progressive myelopathy of two years. The imaging and corresponding histological findings are discussed and correlated.

Hypertrophic Spinal Pachymeningitis With Thoracic Myelopathy

A retrospective case review combined with a review of current literature. We describe a case of antineutrophil cytoplasmic antibodies (ANCA)-related systemic vasculitis, with nearly 4 years of clinical and radiographic follow-up, initially presenting as hypertrophic spinal pachymeningitis (HSP). The diagnosis, surgical, and medical treatment of HSP are discussed in the context of a literature review. HSP is a rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. HSP has been associated with infectious, inflammatory, autoimmune, and traumatic conditions. Surgical decompression and/or corticosteroid therapy have been shown effective at managing this disorder, but identifying associated diseases can be essential to a favorable patient outcome. The medical, pathologic, and radiographic records of this case were reviewed. A computer-based search of the PubMed database was used to perform a comprehensive literature review of HSP. We report a 66-year-old male with a history of renal cell carcinoma and bladder cancer who presented with upper abdominal pain, thoracic myelopathy (progressive weakness and numbness of the lower extremities, and gait disturbances), and weight loss. A diagnosis of HSP was subsequently shown to be the initial presentation of ANCA-related systemic vasculitis. The possibility of an ANCA-related systemic vasculitis presenting as HSP without systemic signs is a diagnostic and therapeutic challenge for the spinal surgeon. The diagnosis of HSP should initiate a search for an associated disease process and close follow-up after initial treatment. With interdisciplinary collaboration between medicine, radiology, pathology, and orthopedics, the diagnosis was made and a treatment initiated which halted disease progression and has maintained remission for more than 4 years.

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a comparatively rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. We report a case of IHSP followed up for 20 years in a 46-year-old man. Expansive laminoplasty was performed in 1991, and this case has been previously reported by a co-author. After 17 years, the patient's gait disturbance returned. Physical examination and imaging confirmed IHSP that had developed into syringomyelia at the T2-L1 conus level. This case was diagnosed as adhesive spinal arachnoiditis due to pachymeningitis caused by syringomyelia. T1-T4 laminectomy, a syringo-subarachnoid shunt (S-S shunt), and L2-L3 laminectomy were performed. The patient again developed dysesthesia and gait disturbance 3 years after the second operation. Most reports of IHSP have limited their focus to short-term follow-up after initial treatment with no long-term results. At present, there are only five reports referring to long-term results of greater than 5 years. All but one case needed additional surgery. To the best of our knowledge, this is the first case in which syringomyelia occurred in a patient with IHSP. It is important to note that syringomyelia may be a cause of symptom recrudescence during long-term follow-up in IHSP patients.