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Table of Contents
Year : 2021  |  Volume : 17  |  Issue : 2  |  Page : 58-62

A case of idiopathic hypertrophic cranial pachymeningitis masquerading as Tolosa–Hunt syndrome in an elderly female

Department of Medicine, Indira Gandhi Government Medical College, Nagpur, India

Date of Submission03-Apr-2021
Date of Acceptance05-May-2021
Date of Web Publication09-Aug-2021

Correspondence Address:
Dr. Kizhakkemuriyil S Tony
Department of Medicine, Indira Gandhi Government Medical College, Nagpur.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/AMJM.AMJM_12_21

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Idiopathic hypertrophic cranial pachymeningitis (IHCP) is an extremely rare clinical entity of unknown etiology, characterized by a chronic inflammation causing thickening of the dura mater.[1] ICHP can produce similar presentation to Tolosa–Hunt syndrome (THS) if it involves cavernous sinus.[2] THS might represent a focal manifestation of IHCP.[2] The THS is a rare syndrome with an estimated annual incidence of one case per million per year.[3] It typically presents with orbital pain associated with palsy of the third, fourth, or sixth cranial nerve. We present an interesting case of a patient with IHCP associated with THS in an elderly female who responded well to high-dose steroids and in a few days had significant improvement in her retro-orbital pain and ocular movements. The importance of appropriate neuroimaging and serological investigation in patients with suspected THS, role of erythrocyte sedimentation rate in follow-up of these patients, and the relationship between IHCP and THS are discussed.

Keywords: Ophthalmoplegia, pachymeningitis, Tolosa–Hunt syndrome

How to cite this article:
Parate T, Tony KS, Chavan S, Khatri A. A case of idiopathic hypertrophic cranial pachymeningitis masquerading as Tolosa–Hunt syndrome in an elderly female. Amrita J Med 2021;17:58-62

How to cite this URL:
Parate T, Tony KS, Chavan S, Khatri A. A case of idiopathic hypertrophic cranial pachymeningitis masquerading as Tolosa–Hunt syndrome in an elderly female. Amrita J Med [serial online] 2021 [cited 2022 Aug 11];17:58-62. Available from: https://ajmonline.org.in/text.asp?2021/17/2/58/323542

  Introduction Top

Painful ophthalmoplegia is a condition associated with multiple cranial nerve palsies involving oculomotor, trochlear, abducent, and ophthalmic division of the trigeminal nerve. Various etiologies have been reported; these include infections, inflammations, sphenoid sinus mucocele, tumors, dural arteriovenous malformation, trauma, and diabetes mellitus. Tolosa–Hunt syndrome (THS), a rare cause of painful ophthalmoplegia, is characterized by non-specific granulomatous inflammation of the cavernous sinus, superior orbital fissure, and/or the apex of the orbit.[4] The disease is manifested by recurrent periorbital pain, headaches on the same periorbital side, and diplopia. Clinical signs include slight exophthalmos, extra ocular palsies involving the third, fourth, and sixth cranial nerves, and numbness in the area of V1 and V2 branch of the trigeminal nerve.[4],[5]

The disease shares histopathological features with idiopathic orbital pseudo tumor; however, owing to its anatomical location, it produces characteristic clinical manifestations. Clinically, immediate response to steroid therapy is a hallmark of the condition. The clinical presentation of THS has a wide differential diagnosis, and timely and appropriate imaging—as an adjunct to pertinent laboratory investigations—can greatly assist clinicians with early accurate diagnosis and management.[6]

  Case Report Top

Our patient, a 68-year-old female with no significant past history, presented with a 4-week history of left hemicranial headache and drooping of left eyelid following cataract surgery on right eye during the month of February 2020. She also complained of pain, redness, swelling, protrusion, watering from left eye, and numbness around the eye for the same duration. Diplopia, maximum on the left side and blurring of vision in the same eye, was also present. On clinical examination, her vitals were normal, she had left-sided ptosis [Figure 1] and restriction of movement of the left eye in all directions. All extraocular muscles were involved (left third, fourth, sixth cranial nerves) [Figure 2]. She also had decreased corneal sensation and corneal and conjunctival reflex (first part of trigeminal nerve involvement). Fundus examination of the right eye was normal and that of the left revealed attenuated blood vessels. No other cranial nerve involvement could be elicited. Sensory and motor system examination was normal. No long tract signs were present. Bladder and bowel habits were within normal limits. No other abnormalities were found.
Figure 1: Presenting features before steroid therapy showing left-sided ptosis

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Figure 2: Improvement after course of steroids

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Complete blood count and C-reactive protein were normal. Erythrocyte sedimentation rate (ESR) was 110 mm at the end of 1 h. Cerebrospinal fluid (CSF) examination showed lymphocytosis with slightly raised protein. CSF adenosine deaminase was 2.9 U/L. HbA1C was normal. Thyroid function test was normal. Viral hepatitis markers were negative. An enzyme-linked immunosorbent assay test for HIV was negative. Serum calcium was normal. Tuberculosis was ruled out. Venereal Disease Research Laboratory (VDRL) test was negative. Computed tomography (CT) scan brain was normal. Ophthalmology consultation was done, and it revealed decreased visual acuity in the left eye. Magnetic resonance imaging (MRI) orbit showed asymmetrical mild enhancing altered ill-defined signal intensity lesion in the left anterior cavernous sinus, left superior orbital fissure, and left orbital apex and short-TI inversion recovery (STIR) hyperintensity involving lateral retrobulbar fat, superior rectus muscle, and lateral muscle [Figure 1]. MRI brain was suggestive of dural thickening with pachymeningeal enhancement along left fronto-parietal and bilateral basitemporal regions [Figure 3] and [Figure 4].
Figure 3: MRI showing pachymeningitis

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Figure 4: Hypertrophic pachymeningitis in anterior basitemporal region and left frontoparietal region

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Neurology consultation was done. Anti-nuclear antibody and anti-neutrophlic cytoplasmic antibody were done and negative. Angiotensin-converting enzyme levels were also estimated and were found to be optimal. On the basis of these findings, a diagnosis of Idiopathic hypertrophic cranial pachymeningitis (IHCP) was made, even though a histopathological diagnosis could not be achieved. The patient was started on methylprednisolone 1 g i.v. OD, followed by oral steroids. The patient clinically improved after 3 days [Figure 5] after initiation of steroids. The patient was started on oral steroids and was referred to a neurosurgeon for further evaluation and management and was kept on periodic follow-up and evaluation.
Figure 5: Improvement after initiation of steroids

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  Discussion Top

IHCP is a diffuse inflammatory disease that causes thickening of the dura mater.[2]

Clinical features

Headache and cranial nerve palsies are the most common manifestations of the disease followed by cerebellar symptoms and diabetes insipidus.[2],[7] Among the cranial nerves, extraocular nerves, lower cranial nerves, and optic nerve are involved in that order. Cranial neuropathy is due to fibrous encasement of the nerves near the foramina or ischemia.[8] Occasionally, patients can present with dural venous sinus thrombosis or hydrocephalus.[2]

Radiological diagnosis

The hallmark of IHCP is the enhancement of the dura that is evident both on CT and MRI. However, because of its superior imaging characteristics, contrast-enhanced MRI is the procedure of choice and it reveals enhancement of the involved dura. As dural enhancement can occur in a number of other conditions such as neurosarcoidosis, spontaneous intracranial hypotension, malignancy, plasmocytoma, and en plaque meningioma, the diagnosis of IHCP is one of the exclusions.[2] Thallium-201 SPECT has been shown to be useful in detecting inflammatory activity that is not detected by MRI.[9]

ESR and CSF finding IHCP

In a series of 12 patients, Kupersmith et al.[10] described an elevated ESR in 41%. CSF examination was variable and non-diagnostic, but proteins were elevated in 50% of their patients.[11] But in case of our patient, ESR was elevated and CSF showed lymphocytosis.

Differential diagnosis of IHCP

The diagnosis of IHCP is one of the exclusions. The diseases to be ruled out include (1) neurosarcoidosis, (2) tuberculosis, (3) syphilitic and fungal pachymeningitis, (4) rheumatoid pachymeningitis, (5) Wegener’s granulomatosis, (6) en plaque meningioma, (7) dural carcinomatosis and intracranial plasmacytoma, and (8) spontaneous intracranial hypotension.

Treatment of IHCP

Corticosteroids constitute the mainstay in the treatment of IHCP. However, some patients experience recurrence following cessation of corticosteroid therapy. When the condition does not respond to corticosteroids, other drugs such as azathioprine and cyclophosphamide can be tried. Other suggested treatments include antituberculous treatment when the patient hails from an endemic area[12] or lymphocytapheresis in resistant cases.[13] Surgical treatment is indicated in two cases: (1) to establish the histological diagnosis and (2) in those occasional cases of IHCP that do not adequately respond to steroid therapy, surgical decompression by excision of the involved dura provides lasting relief, or when pachymeningitis leads to obstructive hydrocephalus or compression of the craniocervical junction.[2]

Relationship between IHCP and THS

Both are chronic granulomatous conditions which commonly present with headache and cranial nerve palsies with good response to steroid therapy and are prone for recurrence. However, whether they are different manifestations of the same disease or two independent disease entities with similar clinical presentation remains to be evaluated. It is important to remember that THS is a constellation of symptoms and signs and has been reported in association with a number of conditions.[2] Miwa et al.[14] proposed that THS might represent a focal manifestation of idiopathic pachymeningitis. Even though our experience is anecdotal, from our experience and from the review of the literature, we feel that the nosologic entity of THS might represent one manifestation of IHCP and further studies are required to clarify its etiology.

  Conclusions Top

IHCP can present as THS. Appropriate neuroimaging and serological investigation are mandatory in cases of THS. The condition usually responds to steroid therapy. However, long-term follow-up of these patients is necessary as recurrence can occur after cessation of steroid therapy. ESR can be used to monitor the response to treatment and recurrence of the disease.[15]

Financial support and sponsorship

The authors have not declared a specific grant for this research from any funding agency in the public, commercial, or not-for-profit sector.

Conflicts of interest

None declared.

  References Top

Sachdeva V, Gupta N, Mittal V, Chandrasekharan A, Kekunnaya R, Suresh T Idiopathic hypertrophic cranial pachymeningitis presenting as recurrent multiple cranial nerve palsies: Case report and review of literature. Delhi J Ophthalmol2015;25:179-82.  Back to cited text no. 1
Muthukumar N, Senthilbabu S, Usharani K Idiopathic hypertrophic cranial pachymeningitis masquerading as Tolosa–Hunt syndrome. J Clin Neurosci 2005;12:589-92.  Back to cited text no. 2
Iaconetta G, Stella L, Esposito M, Cappabianca P Tolosa–Hunt syndrome extending in the cerebello-pontine angle. Cephalalgia 2005;25:746-50.  Back to cited text no. 3
Arar ZV, Janjetović Z, Marinić M, Sekelj S, Lezaić Z, Dikanović M [Painful ophthalmoplegia—Tolosa–Hunt syndrome]. Acta Med Croatica2007;61(4):395-8.  Back to cited text no. 4
Ranjan R, Banerjee A, Sarkar R, Sarkar N Tolosa-Hunt syndrome: A rare case report with uncommon imaging findings and discussion. Int J Res Med Sci 2014;2:1795-8.  Back to cited text no. 5
Mendez JA, Arias CR, Sanchez D, Pesci LM, Lopez BS, Lopez R, et al. Painful ophthalomoplegia of the left eye in a 19-year-old female, with an emphasis on Tolosa-Hunt syndrome: a case report. Cases J. 2009;2:827.  Back to cited text no. 6
Takuma H, Shimada H, Inoue Y, Ishimura E, Himuro K, Miki T, et al. Hypertrophic pachymeningitis with anti-neutrophil cytoplasmic antibody (p-ANCA), and diabetes insipidus. Acta Neurol Scand 2001;104:397-401.  Back to cited text no. 7
River Y, Schwartz A, Gomori JM, Soffer D, Siegal T Clinical significance of diffuse dural enhancement detected by magnetic resonance imaging. J Neurosurg 1996;85: 777-83.  Back to cited text no. 8
Nishioka H, Ito H, Haraoka J, Yamada Y, Nojima H Idiopathic hypertrophic cranial pachymeningitis with accumulation of thallium-201 on single-photon emission CT. AJNR Am J Neuroradiol 1998;19: 450-3.  Back to cited text no. 9
Kupersmith MJ, Martin V, Heller G, Shah A, Mitnick HJ Idiopathic hypertrophic pachymeningitis. Neurology 2004;62:686-94.  Back to cited text no. 10
Mathew RG, Hogarth KM, Coombes A Idiopathic hypertrophic cranial pachymeningitis presenting as acute painless visual loss. Int Ophthalmol 2012;32:195-7.  Back to cited text no. 11
Parney IF, Johnson ES, Allen PB “Idiopathic” cranial hypertrophic pachymeningitis responsive to antituberculous therapy: Case report. Neurosurgery 1997;41:965-71.  Back to cited text no. 12
Yamamoto T, Goto K, Suzuki A, Nakamura N, Mizuno Y Long-term improvement of idiopathic hypertrophic cranial pachymeningitis by lymphocytapheresis. Ther Apher 2000;4: 313-6.  Back to cited text no. 13
Miwa H, Koshimura I, Mizuno Y Recurrent cranial neuropathy as a clinical presentation of idiopathic inflammation of the dura mater: A possible relationship to Tolosa-Hunt syndrome and cranial pachymeningitis. J Neurol Sci 1998;154:101-5.  Back to cited text no. 14
Alzu’bi AA Tolosa-Hunt syndrome: Increased severity on second attack. J Gen Pract2016;4:226.  Back to cited text no. 15


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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