|Year : 2015 | Volume
| Issue : 4 | Page : 531-533
Idiopathic hypertrophic pachymeningitis presenting with nasal mass: A rare case
Bhagwati Salgotra1, Kunal Kishore1, Dakshyani Nirhale2, Gaurav Sharma1
1 Department of Neurosurgical Sciences, Dhiraj Hospital, Sumandeep Vidyapeeth, Pipari, Vadodara, Gujarat, India
2 Depatment of Surgery, Dr. D.Y. Patil Medical College, Pimpri, Pune, Maharashtra, India
|Date of Web Publication||14-Jul-2015|
Department of Neurosurgical Sciences, E26 Staff Quarter, Dhiraj Hospital, Sumandeep Vidyapeeth University, Pipari, Vadodara, Gujarat
Source of Support: None, Conflict of Interest: None
Hypertrophic pachymeningitis (HP) is a rare disorder of diverse etiology. This case presented with a nasal mass. Magnetic resonance imaging of brain revealed HP. All other differential diagnosis was ruled out to term it as idiopathic. Patient was treated conservatively on steroids and nonsteroidal antiinflammatory drugs. Patient showed improvement of symptoms on treatment. We report here a case of idiopathic HP presenting with nasal mass.
Keywords: Nasal mass, pachymeningitis, steroids
|How to cite this article:|
Salgotra B, Kishore K, Nirhale D, Sharma G. Idiopathic hypertrophic pachymeningitis presenting with nasal mass: A rare case. Med J DY Patil Univ 2015;8:531-3
|How to cite this URL:|
Salgotra B, Kishore K, Nirhale D, Sharma G. Idiopathic hypertrophic pachymeningitis presenting with nasal mass: A rare case. Med J DY Patil Univ [serial online] 2015 [cited 2019 Aug 17];8:531-3. Available from: http://www.mjdrdypu.org/text.asp?2015/8/4/531/160829
| Introduction|| |
Hypertrophic pachymeningitis (HP) is a rare disorder of diverse etiology. It was first described by Charcot and later by Naffziger and Stern.  HP is a rare form of diffuse inflammatory disease that causes thickening of the dura mater. It can involve the cranial or the spinal dura or both. Until 2008, 60 treated cases of HP have been reported in the English literature. Three more cases were reported from India in 2009.  We present a case of HP extending into nose presenting with nasal congestion.
| Case Report|| |
A 22-year-young male came with chief complaints of headache since 6 months and nasal congestion since 2 months. Examination revealed a nasal mass. Patient was conscious oriented [Figure 1]. No history of seizures, loss of consciousness or vomiting was present. Glasgow Coma Scale score was 15/15 at presentation. On magnetic resonance imaging (MRI) (plain + contrast) [Figure 2]. Diffuse thickening of dura was present involving base of skull and frontoparietal area including fissures extending upto nose [Figure 3]. Intranasal mass removal and biopsy was taken which revealed nonspecific granulomatous inflammation [Figure 4]. Venereal disease research laboratory test was negative. Serum polymerase chain reaction for tuberculosis was negative. Serum calcium levels, angiotensin-converting enzyme levels were normal. Chest X-ray was within normal limits. So sarcoidosis was ruled out. perinuclear anti-neutrophil cytoplasmic antibodies and cytoplasmic anti-neutrophil cytoplasmic antibodies were negative, Wegener's granulomatosis and vasculitis were ruled out. Patient was kept on conservative treatment with steroids, nonsteroidal antiinflammatory drugs, antiepileptics (prophylactically) and was offered surgery later but patient relatives denied surgery. Patient was discharged on steroids.
|Figure 2: Magnetic resonance imaging brain with contrast (saggital view)-showing hyperintensity along the base of skull + frontoparietal region + fissures|
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|Figure 3: Magnetic resonance imaging paranasal sinus view-extension of lesion to nose|
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|Figure 4: Histopathological examination of nasal lesion shows nonspecific granulomatous infl ammation|
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| Discussion|| |
Nonspecific symptoms of headache, nausea, and vomiting at the onset of the disease are the most common clinical presentations or are secondarily manifested with serous otitis and retro-orbital pain with later development of vision loss and hearing impairment.  Until 2008, 60 treated cases of HP have been reported in the English literature. Three more cases were reported from India in 2009.  There are few cases reported in literature involving visual disturbances but this is probably the first case reported with nasal mass. Cranial pachymeningitis typically cause progressive cranial nerve palsies, headaches, and cerebellar dysfunction. Cranial nerve VIII is the most frequently involved cranial nerve, and the next most frequently involved are cranial nerves V, VII, IX, X, and XII, with equal frequency.  Seizure at initial presentation is rare.  Hypertrophic cranial pachymeningitis is best identified by MRI.  The diagnosis is established by excluding all other granulomatous and infectious diseases.  The cause of idiopathic hypertrophic pachymeningitis (IHCP) is still speculative at this moment, from infectious agent, mucopolysaccharidosis, and intrathecal toxin to fibrosclerotic disease.  The diagnosis of IHCP relies on the exclusion of other possible causes of pachymeningitis, from granulomatous disease, such as neurosarcoidosis, neurosyphyllis, tuberculosis and rheumatoid pachymeningitis, and Wegener's granulomatosis to primary tumor of the meninx, en plaque meningioma, or dural carcinomatosis.  The pathologic finding of a biopsy specimen is also diagnostic because it shows proof of chronic inflammatory response.
The optimal treatment of IHCP is unknown.  Untreated, the clinical course is usually marked by severe headache and progressive neurologic detorioration and vision loss.  Steroid is the mainstay of therapy and is often effective in arresting disease progression,  suggesting that the mechanism underlying pachymeningitis may be an immunologic process.
In our case patient presented with a nasal mass whose biopsy showed nonspecific granulomatous inflammation. After ruling out all granulomatous disease and on MRI findings diagnosis of pachymeningitis was made and patient treated conservatively with steroids. Patient showed improvement with steroids.
| Conclusion|| |
We presented a very rare case of pachymeningitis extending into nose presenting with a nasal mass which was treated conservatively and patient was discharged with relieve of symptoms.
| References|| |
Mamelak AN, Kelly WM, Davis RL, Rosenblum ML. Idiopathic hypertrophic cranial pachymeningitis. Report of three cases. J Neurosurg 1993;79:270-6.
Hassan KM, Deb P, Bhatoe HS. Idiopathic hypertrophic cranial pachymeningitis: Three biopsy-proven cases including one case with abdominal pseudotumor and review of the literature. Ann Indian Acad Neurol 2011;14:189-93.
Lee YC, Chueng YC, Hsu SW, Lui CC. Idiopathic hypertrophic cranial pachymeningitis: Case report with 7 years of imaging follow-up. AJNR Am J Neuroradiol 2003;24:119-23.
Rossi S, Giannini F, Cerase A, Bartalini S, Tripodi S, Volpi N, et al.
Uncommon findings in idiopathic hypertrophic cranial pachymeningitis. J Neurol 2004;251:548-55.
Hatano N, Behari S, Nagatani T, Kimura M, Ooka K, Saito K, et al.
Idiopathic hypertrophic cranial pachymeningitis: Clinicoradiological spectrum and therapeutic options. Neurosurgery 1999;45:1336-42.
Phanthumchinda K, Sinsawaiwong S, Hemachudha T, Yodnophaklao P. Idiopathic hypertrophic cranial pachymeningitis: An unusual cause of subacute and chronic headache. Headache 1997;37:249-52.
Friedman D, Flanders A, Tartaglino L. Contrast-enhanced MR imaging of idiopathic hypertrophic craniospinal pachymeningitis. AJR Am J Roentgenol 1993;160:900-1.
Choi IS, Park SC, Jung YK, Lee SS. Combined therapy of corticosteroid and azathioprine in hypertrophic cranial pachymeningitis. Eur Neurol 2000;44:193-8.
Kupersmith MJ, Martin V, Heller G, Shah A, Mitnick HJ. Idiopathic hypertrophic pachymeningitis. Neurology 2004;62:686-94.
Bhatia R, Tripathi M, Srivastava A, Garg A, Singh MB, Nanda A, et al.
Idiopathic hypertrophic cranial pachymeningitis and dural sinus occlusion: Two patients with long-term follow up. J Clin Neurosci 2009;16:937-42.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]