Idiopathic Hypertrophic Spinal Pachymeningitis: A Rare Cause of Spinal Cord Compression

Kutty, Raja K; Sreemathyamma, Sunilkumar Balakrishnan; Sivanandapanicker, Jyothish Laila; Peethambaran, Anilkumar

doi:10.4103/0028-3886.271262

Cited by 2 publications

(6 citation statements)

References 38 publications

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“…Conservative management alone in the form of steroid therapy was tried in four cases and all of them completely improved; however, 50% of them showed recurrence of disease (Table 3) (Supplementary Material 1, www.journalmc.org). Presence of myelopathy features or UMN signs could explain the rationale behind their relapse [4,17]. On the contrary, steroids alone can be an effective management for patients with symptoms of radiculopathy including current case.…”

Section: Treatment Outlines and Relapsesmentioning

confidence: 78%

“…We did a literature search on PubMed for published cases of IHSP since 2009 and found a total of 22 cases. Findings from these studies have been described in Supplementary Material 1 (www.journalmc.org) [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22]. We analyzed a total of 23 cases including ours.…”

Section: Review Of Current Literaturementioning

confidence: 99%

“…During our comprehensive review, we identified total of four and five cases with elevated CSF proteins [16,17,21] and lymphocyte count [4,16,17,21] respectively, including our case. Severity of CSF abnormalities including CSF protein and cell count is positively related to disease progression, as stated by Qin et al in their study [16].…”

Section: Laboratory Featuresmentioning

confidence: 99%

“…Relapse of IHSP remains a critical aspect of disease management with poor understanding of its implications and underlying mechanism. From our review of cases, six patients experienced relapse with an average 9-week period [4,8,14,16,17,20]. Some studies suggest patients with elevated inflammatory markers and leukocytosis have increased relapse rate in comparison to those without these findings [5,16].…”

Section: Treatment Outlines and Relapsesmentioning

confidence: 99%

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Idiopathic Hypertrophic Spinal Pachymeningitis

Gupta,

Um,

Samant

et al. 2023

J Med Cases

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Hypertrophic pachymeningitis (HP) is a rare presentation with duramater thickening and fibrosis which can result in cranial or spinal compressive disease. Most cases of spinal HP require surgical management. We present an uncommon case of idiopathic hypertrophic spinal pachymeningitis (IHSP) in a 40-year-old male who showed complete improvement to steroids without any further relapses. The patient presented with bilateral upper limb weakness with magnetic resonance imaging (MRI) spine showing diffuse dural thickening of the entire spine with cervical cord compression. He had an extensive workup for underlying etiology and worsening symptoms until he was diagnosed with IHSP. Later, he was started on high-dose steroids with good response and no relapse after 2 years. A descriptive analysis of IHSP cases since 2009 including ours showed that it usually occurs after 50s with female preponderance. Weakness and sensory loss are the most common complaints with 50% patients showing clinical signs of myelopathy like hyperreflexia, clonus, Babinski sign and sensory level. Cerebrospinal fluid (CSF) and inflammatory markers like erythrocytic sedimentation rate (ESR) and C-reactive protein (CRP) can be used to assess disease progression and prognosis. Surgical removal of HP followed by steroids is the best line of management while steroids alone can be tried in cases where clinical signs of myelopathy are absent.

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Section: Treatment Outlines and Relapsesmentioning

confidence: 78%

Section: Review Of Current Literaturementioning

confidence: 99%

Section: Laboratory Featuresmentioning

confidence: 99%

Section: Treatment Outlines and Relapsesmentioning

confidence: 99%

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Idiopathic Hypertrophic Spinal Pachymeningitis

Gupta,

Um,

Samant

et al. 2023

J Med Cases

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“…The parenchymal manifestations of HP include seizures, hemiparesis, tremors, cognitive impairment, and localized brain edema with "pseudotumor" presentation 7,12,13 . HP is a rare cause of spinal cord compression 14 .…”

Section: Clinical Featuresmentioning

confidence: 99%

A clinical approach to hypertrophic pachymeningitis

Abrantes

Moraes

Filho

et al. 2020

Arq. Neuro-Psiquiatr.

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Importance: Hypertrophic pachymeningitis (HP) is a non-usual manifestation of rheumatologic, infectious, and neoplastic diseases. Etiological diagnosis is a challenge, but when made promptly it creates a window of opportunity for treatment, with the possibility of a total reversal of symptoms. Observations: HP is an inflammatory process of the dura mater that can occur as a manifestation of sarcoidosis, granulomatosis with polyangiitis, and IgG4-related disease. The HP case evaluation is extensive and includes central nervous system imaging, cerebrospinal fluid analysis, serology, rheumatologic tests, and systemic survey for other manifestations sites. After systemic investigation, meningeal biopsy might be necessary. Etiology guides HP treatment, and autoimmune disorders are treated with corticosteroids alone or associated with an immunosuppressor. Conclusion: HP is a manifestation of several diseases, and a precise etiological diagnosis is crucial because of the difference among treatments. An extensive investigation of patients with HP helps early diagnosis and correct treatment.

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Idiopathic Hypertrophic Spinal Pachymeningitis: A Rare Cause of Spinal Cord Compression

Cited by 2 publications

References 38 publications

Idiopathic Hypertrophic Spinal Pachymeningitis

Idiopathic Hypertrophic Spinal Pachymeningitis

A clinical approach to hypertrophic pachymeningitis

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