ABSTRACT:
Idiopathic hypertrophic pachymeningitis is rare cause of neurological symptoms
with myelopathy due to spinal cord compression. We report a case of
pachymeningitis, which was manifested primarily by tetraparesis after low-energy
trauma and recurrence the myelopathy symptoms after 5 years of surgery. The
patient, a 19-year-old woman, was subjected to extensive investigation without
evidence of any underlying disease. A meningeal biopsy was performed and showed
an unspecific inflammatory process with extensive fibrosis of the dura mater.
These findings, associated with the exclusion of other causes, suggest
idiopathic hypertrophic pachymeningitis.
Keywords: spinal cord; meningitis/ pathology; meningitis/ physiopathology; hypertrophy.
RESUMO:
A paquimeningite hipertrófica idiopática é uma causa rara de sintomas
neurológicos apresentando mielopatia por compressão da medula espinhal.
Relatamos um caso de paquimeningite com manifestação primária de tetraparesia
após trauma de baixa energia e recorrência dos sintomas de mielopatia 5 anos
após a cirurgia. A paciente, uma mulher de 19 anos, foi submetida a extensa
investigação sem evidências de qualquer doença de base. Uma biópsia da meninge
revelou processo inflamatório inespecífico com extensa fibrose da dura máter,
também visualizado no período perioperatório. Esses achados, associados à
exclusão de outras causas, sugerem o diagnóstico de paquimeningite hipertrófica
idiopática.
Palavras-chave: medula espinal; meningite/patologia; meningite/fisiopatologia; hipertrofia.
FIGURES
| Citation: Melo LLA, Daher MT, Gonçalves MVM, Freitas MB. Idiopathic Hypertrophic Spinal Pachymeningitis: A Case Report. 57(03):521. doi:10.1055/s-0041-1724087 |
| Financial Support There was no financial support from public, commercial, or non-profit sources. |
|
Conflict of Interests The authors have no conflict of interests to declare. |
| Received: October 12 2020; Accepted: December 01 2020 |
INTRODUCTION
Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory autoimmune disorder that leads to spinal cord compression. This disease, which may be related to IgG4 is an immune-mediated condition characterized by lesions with inflammatory reaction associated with fibrosis and lymphoplasmocyte infiltration, composing a spectrum of lymphoproliferative diseases. We present a case with this pathology and the magnetic resonance imaging (MRI) and meningeal biopsy findings.
CASE REPORT
A 19-year-old woman presented with acute tetraparesis after cervical whiplash injury. The image of the arrival examination (►Figure 1)showed signs of cervical stenosis at the C3 to C6 level, suggestive of epidural hematoma in the differential diagnosis. Initially, a C3 to C6 laminoplasty was performed. After the surgery, there was no improvement in the neurological status, and postoperative imaging still showed cervical stenosis. It was decided to perform a corpectomy of the C3 level with anterior arthrodesis of the C2 to C4 levels (►Figure 2).
The patient showed a progressive motor and sensitive improvement after surgery and physiotherapy. Five years later, the patient displayed abrupt worsening of gait without trauma and motor worsening in the upper and lower limbs (asymmetrical, worse on the right side). Magnetic resonance imaging noted cervicothoracic stenosis at the C7 to T4 levels (►Figure 3). A C7 to T4 laminoplasty was performed. During the surgery, we observed a posterior thickening of the meninge (►Figura 4), which led to the decision of performing a duraplasty with dura mater substitute and posterior arthrodesis of C7 to T5 (►Figure 5). In the biopsy, it was noted a nonspecific chronic inflammatory process of the meninges, with lymphoplasmocyte infiltrate, suggestive of hypertrophic pachymeningitis (►Figure 6). The patient evolved with improvement of the gait and motor symptoms after surgery. Secondary causes were excluded during clinical investigation and the histological clinical diagnosis was IHSP.
DISCUSSION
Hypertrophic pachymeningitis is a rare disease arising from a chronic inflammatory course evolving with fibrosis of the duramater.1,2 It may be idiopathic or secondary to systemic diseases, such as sarcoidosis, tuberculosis, fungal meningitis, rheumatoid arthritis, carcinomatosis, among other inflammatory diseases.
This disease may have intracranial and spinal involvement. A spinal form, IHSP, is extremely rare, and it might be associated with myelopathy, radiculopathy, or both. This disease is included in the spectrum of diseases related to IgG4, which present with diffuse fibrosis combined with lymphoplasmacytic infiltration rich in IgG4-positive tissue plasma cells.3 The spinal canal stenosis occurs in this case, and the patient may present abrupt neurological worsening after the trauma. In MRI, it is common to find thickening of the dura mater with hypointensity in T2 and T1, affecting multiple levels with strong contrast enhancement.4,5 The clinical treatment includes use of long-term immunosuppressants , such as corticosteroids. Surgical treatment is performed inpatients with myelopathy or radiculopathy associated with neurological changes for decompression of the spinal cord. In some cases, it is necessary to perform duraplasty for adequate decompression.
CONCLUSION
Hypertrophic pachymeningitis is a rare disease that can develop with spinal canal stenosis with signs of myelopathy. In these cases, surgical decompression may be one of the first therapeutic options implemented, combined with the use of immunosuppressants, which can reduce neurological sequelae in these patients.
