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Year : 2015  |  Volume : 3  |  Issue : 2  |  Page : 105-107

Idiopathic hypertrophic pachymeningitis: An unusual masquerader of multiple cranial nerve palsies

1 Department of General Ophthalmology, Aravind Eye Hospital and PG Institute of Ophthalmology, Tirunelveli, Tamil Nadu, India
2 Department of Neuro-Ophthalmology, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Tirunelveli, Tamil Nadu, India
3 Department of Pediatric Ophthalmology, Sadguru Netra Chikitsalaya, Chitrakoot, Madhya Pradesh, India

Date of Submission21-Jul-2014
Date of Acceptance06-Nov-2014
Date of Web Publication7-May-2015

Correspondence Address:
Kaustubh B Harshey
Aravind Eye Hospital, S.N High Road, Tirunelveli - 62 7001, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2320-3897.156607

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Idiopathic hypertrophic pachymeningtis is a rare inflammatory disorder affecting the dura mater. A 39-year-old diabetic male presented with left sixth nerve paresis along with ophthalmic and hypoglossal nerve involvement. Neuroimaging is an important tool for the neuroophthalmologist in such cases where it can point to a diagnosis as well as exclude common conditions like ischemic neuropathy in the absence of more invasive diagnostic tests. Magnetic resonance imaging (MRI) in our case revealed features suggestive of hypertrophic pachymeningitis. Secondary causes were ruled out with extensive investigations and the patient improved completely with systemic corticosteroids.

Keywords: Corticosteroids, hypertrophic pachymeningitis, magnetic resonance imaging, multiple cranial neuropathy

How to cite this article:
Harshey KB, Maharajan P, Khilji S, Rengappa R. Idiopathic hypertrophic pachymeningitis: An unusual masquerader of multiple cranial nerve palsies . J Clin Ophthalmol Res 2015;3:105-7

How to cite this URL:
Harshey KB, Maharajan P, Khilji S, Rengappa R. Idiopathic hypertrophic pachymeningitis: An unusual masquerader of multiple cranial nerve palsies . J Clin Ophthalmol Res [serial online] 2015 [cited 2023 Mar 24];3:105-7. Available from: https://www.jcor.in/text.asp?2015/3/2/105/156607

Idiopathic hypertrophic pachymeningitis (IHP) is a rare disorder characterized by fibrosing inflammation that thickens the dura mater. The disorder is usually seen by neurophysicians, but little is known about it in the ophthalmic community despite frequent ocular manifestations. Ophthalmologists should be aware of this disease for a multidisciplinary approach, which may aid in its early diagnosis and treatment.

  Case Report Top

A 39-year-old diabetic male presented with sudden horizontal binocular diplopia since 2days, more on left gaze with headache. Two years previously he developed left-sided total external ophthalmoplegia with an enhancing lesion at the left orbital apex on neuroimaging which resolved with systemic corticosteroids. Ocular examination showed left face turn; left eye (LE) 15° esotropia, with secondary (40 prism diopters) more than primary deviation (28 prism diopters) and limitation of abduction in the LE [Figure 1]a.

Uncorrected visual acuity was 6/6 in both eyes (BE). Anterior segment was normal showing brisk pupillary reflexes in BE without relative afferent pupillary defect. Corneal sensation was diminished in the LE with deviation of tongue to the left [Figure 1]b. Diplopia charting showed uncrossed diplopia with maximum separation of images on levoversion, while Hess chart confirmed left lateral rectus paresis [Figure 2]. Total and differential leukocyte counts, erythrocyte sedimentation rate (ESR), and urinalysis were normal. Fasting and postprandial blood glucose was 153 and 298 mg/dl, respectively. Considering the multiple cranial neuropathy (left abducens and ophthalmic and hypoglossal nerve) and diabetes, magnetic resonance imaging (MRI) with contrast was repeated which revealed an abnormal thick plaque-like pachymeningeal enhancement in left orbital apex and anterior cavernous sinus [Figure 3], left occipital convexity, and posterior fossa including cerebellum and 9 th to 12 th nerve complexes bilaterally [Figure 4]a and b]; suggestive of hypertrophic cranial pachymeningitis. There was no involvement of falx cerebrum.
Figure 1: (a) Left eye abduction limitation on the gaze photograph of the patient (b) Tongue deviation to the left suggesting left hypoglossal nerve involvement

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Figure 2: Hess chart showing left lateral rectus underaction

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Additional investigations ruled out differential diagnoses. Rheumatoid factor, antinuclear antibodies, anticytoplasmic antibodies, Mantoux and tuberculosis IgG and IgM, Venereal Disease Research Laboratory titers, and human immunodeficiency virus serology were within normal limits. MRI of the chest and abdomen was also normal. Patient was therefore diagnosed as a case of IHP and started on intravenous methylprednisolone 1g/day for 3days followed by tapering oral prednisolone under systemic insulin cover. Two weeks later, he improved completely without diplopia, full ocular movements [Figure 5], a normal corneal sensation and nondeviated tongue. Neuroimaging showed resolution of pachymeningitis [Figure 6]a and b]. Six months later, left sixth nerve paresis recurred twice and resolved completely after oral steroid taper. Further, he reported 2months later again with recurred left sixth nerve paresis and a new left pupil sparing partial third nerve paresis. Low-dose oral steroids were restarted, following which the paresis improved completely within 3 weeks. Currently, the patient is asymptomatic and off steroids since 3 months without any diplopia or neurological deficit.
Figure 3: T1-weighted fat suppressed post-contrast axial section magnetic resonance imaging showing enhancement in the left orbital apex and anterior cavernous sinus

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Figure 4: (a) Meningeal enhancement involving the 9th-12th cranial nerves complex (bilateral) on T1-weighted fat suppressed post-contrast axial section magnetic resonance imaging. (b) Plaque-like meningeal enhancement seen in the posterior fossa and cerebellum on T1-weighted fat suppressed post-contrast axial section magnetic resonance imaging

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Figure 5: Nine gaze picture showing full ocular movements after treatment

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Figure 6: (a) T1-weighted axial scan with contrast showing absence of enhancement at the left orbital apex and anterior cavernous sinus after therapy (b) T2-weighted axial scan of the posterior fossa showing resolution of the plaque-like pachymeningitis after therapy

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  Discussion Top

IHP is an inflammatory disorder involving dura mater of the skull base, cerebrum, tentorium and falx cerebrii, and spinal cord. [1] Hypertrophic pachymeningitis may be primary or secondary, although these associations in secondary cases may be controversial. [2]

The etiopathogenesis is unknown, but it may be an autoimmune disorder or occurring directly due to infectious or infiltrative pathology. [3],[4] Rossi et al., demonstrated fibrosis and prominent CD4 + T-cell inflammatory infiltrate on dural biopsy, suggesting a role for cell-mediated immunity. [1] Riku et al., showed that it may be a dural lesion of IgG4-related systemic disease. [4] Tolosa-Hunt syndrome may be its focal manifestation or it may be a localized manifestation of multifocal fibrosclerosis. [5]

IHP predominantly affects males [6] with age ranging 20-78 years. [5] Predominant clinical features are headache, progressive cranial nerve palsies, and cerebellar dysfunction; [1],[2] resulting from compression of adjacent structures by hypertrophic pachymeninges. [6] Our patient demonstrated similar features sans cerebellar signs. Riku and Kato described two patterns of involvement: Cavernous sinus to superior orbital fissure and falcotentorial to posterior fossa dural involvements. [7] Our patient had features suggestive of both. Spinal pachymeningitis either occurs alone or as a craniospinal form. [2]

Being a diagnosis of exclusion; [3],[5] infective, autoimmune, and neoplastic diseases should be excluded. [2] ESR is usually elevated; [2] however, it was normal in our patient. Cerebrospinal fluid analysis shows lymphocytic pleocytosis with moderately elevated proteins, but can be normal in one-fourth of the cases. [5] MRI with contrast is the definitive modality for radiodiagnosis. [7] Thickened dura appears isointense to hypointense on T1- and T2-weighted images, with uniform dense enhancement on contrast [3] better appreciated on coronal and sagittal images in the interhemispheric fissure, tentorium, and basal dura. [5] Our patient's imaging correlated with the lateral rectus paresis, corneal hypoesthesia, headache, and left hypoglossal nerve involvement and helped us to arrive at a plausible diagnosis explaining the noncontiguous cranial nerve involvement and excluded diabetic neuropathy. However, the absence of cerebellar signs and contralateral multiple cranial nerve involvement on MRI without signs was intriguing as was the absence of other signs of orbital apex syndrome. Dural biopsy is the benchmark for establishing the diagnosis. [6],[7] Biopsy from an accessible site is more likely to be positive. [5],[7] Pathologically, thick fibrous dura associated with chronic inflammatory cell infiltrate of lymphocytes and plasma cells is noted. [1],[6],[7] Being invasive, our patient did not consent for a biopsy or lumbar puncture and in such situations neuroimaging is necessary. [8]

The differential diagnoses are extensive, which include tubercular meningitis, rheumatoid arthritis, Wegener's granulomatosis, meningeal carcinomatosis, syphilitic pachymeningitis, sarcoidosis, etc.; and need careful exclusion.

Optimal treatment protocols do not exist and disease may spontaneously improve or deteriorate without therapy. [2] Systemic corticosteroids are the cornerstone of treatment and are often effective in halting progression. [1],[2],[7] Relapses and recurrences are common with frequent need for immunosuppressive therapy. [1],[2]

Clinical presentation, neuroimaging characteristics, normal laboratory investigations, and recurrence and improvement with systemic steroids strongly suggested IHP in our case.

IHP is a rare cause of multiple cranial neuropathies. Ophthalmologists have a key role in identifying and treating the disease because of its ophthalmic manifestations. Long-term systemic corticosteroids with or without immunosuppressants achieve remission and may prevent sequelae.

  Acknowledgement Top

Dr. Gopinath A, MD (Consultant Radiologist, Barani Scans, Palayamkottai, Tirunelveli, Tamil Nadu).

  References Top

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.  Back to cited text no. 1
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.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
Goyal M, Malik A, Mishra NK, Gaikwad SB. Idiopathic hypertrophic pachymeningitis: Spectrum of the disease. Neuroradiology 1997;39:619-23.  Back to cited text no. 3
Riku S, Hashizume Y, Yoshida M, Riku Y. Is hypertrophic pachymeningitis a dural lesion of IgG4-related systemic disease? Rinsho Shinkeigaku 2009;49:594-6.  Back to cited text no. 4
Sylaja PN, Cherian PJ, Das CK, Radhakrishnan VV, Radhakrishnan K. Idiopathic hypertrophic cranial pachymeningitis. Neurol India 2002;50:53-9.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
Rojana-udomsart A, Pulkes T, Viranuwatti K, Laothamatas J, Phudhichareonrat S, Witoonpanich R. Idiopathic hypertrophic cranial pachymeningitis. J Clin Neurosci 2008;15:465-9.  Back to cited text no. 6
Riku S, Kato S. Idiopathic hypertrophic pachymeningitis. Neuropathology 2003;23:335-44.  Back to cited text no. 7
Pareja-Esteban J, Gutiérrez-Solana S, Cedazo M, Sánchez-Cordón B, Gamo-Gallego M, Teus MA. Hypertrophic pachymeningitis and ophthalmological disturbances: Description of two case reports. Arch Soc Esp Oftalmol 2008;83:497-500.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

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