Misleading Rare Case of Idiopathic Hypertrophic Pachymeningitis

Idiopathic hypertrophic pachymeningitis (IHP) is a rare disease with diffuse thickening of the dura mater that has no specific clinical symptoms and manifestations and it causes neurosurgeons to misdiagnose. A 4-year-old girl presented at the emergency room of our hospital with speech difficulty and...

Full description

Saved in:
Bibliographic Details
Main Authors: Ahmad Rezaee Azandaryani, Amir Mohammad Salehi
Format: Article
Language:English
Published: Wiley 2024-01-01
Series:Case Reports in Medicine
Online Access:http://dx.doi.org/10.1155/2024/5561686
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1832546253704003584
author Ahmad Rezaee Azandaryani
Amir Mohammad Salehi
author_facet Ahmad Rezaee Azandaryani
Amir Mohammad Salehi
author_sort Ahmad Rezaee Azandaryani
collection DOAJ
description Idiopathic hypertrophic pachymeningitis (IHP) is a rare disease with diffuse thickening of the dura mater that has no specific clinical symptoms and manifestations and it causes neurosurgeons to misdiagnose. A 4-year-old girl presented at the emergency room of our hospital with speech difficulty and severe headache. Head computed tomography scans (CT scan) on admission revealed a large fluid collection over the right temporoparietal region with mass effect, and the neurosurgeon drained it with the initial diagnosis of subdural hematoma. However, the postoperative CT scan demonstrated the failure of surgical drainage; therefore, magnetic resonance imaging (MRI) was requested for the patient. MRI identified diffuse nodular dural thickening with internal septations and different internal hemorrhagic stages on the right side with no evidence of brain parenchymal involvement and according to the serology and autoimmune screening tests, and IHP was diagnosed for the patient. The patient underwent craniotomy. There was an immediate improvement of neurologic symptoms. The patient had good clinical and radiologic outcome at 3 -months follow-up. IHP should be part of the differential diagnosis of some cases of noncommunicating hydrocephalus; however, the rarity of the disease and the absence of specific clinical symptoms make the diagnosis difficult.
format Article
id doaj-art-2e5e5ab0bd794c629a0ead7fce55f05b
institution Kabale University
issn 1687-9635
language English
publishDate 2024-01-01
publisher Wiley
record_format Article
series Case Reports in Medicine
spelling doaj-art-2e5e5ab0bd794c629a0ead7fce55f05b2025-02-03T07:23:24ZengWileyCase Reports in Medicine1687-96352024-01-01202410.1155/2024/5561686Misleading Rare Case of Idiopathic Hypertrophic PachymeningitisAhmad Rezaee Azandaryani0Amir Mohammad Salehi1Department of RadiologyStudent Research CommitteeIdiopathic hypertrophic pachymeningitis (IHP) is a rare disease with diffuse thickening of the dura mater that has no specific clinical symptoms and manifestations and it causes neurosurgeons to misdiagnose. A 4-year-old girl presented at the emergency room of our hospital with speech difficulty and severe headache. Head computed tomography scans (CT scan) on admission revealed a large fluid collection over the right temporoparietal region with mass effect, and the neurosurgeon drained it with the initial diagnosis of subdural hematoma. However, the postoperative CT scan demonstrated the failure of surgical drainage; therefore, magnetic resonance imaging (MRI) was requested for the patient. MRI identified diffuse nodular dural thickening with internal septations and different internal hemorrhagic stages on the right side with no evidence of brain parenchymal involvement and according to the serology and autoimmune screening tests, and IHP was diagnosed for the patient. The patient underwent craniotomy. There was an immediate improvement of neurologic symptoms. The patient had good clinical and radiologic outcome at 3 -months follow-up. IHP should be part of the differential diagnosis of some cases of noncommunicating hydrocephalus; however, the rarity of the disease and the absence of specific clinical symptoms make the diagnosis difficult.http://dx.doi.org/10.1155/2024/5561686
spellingShingle Ahmad Rezaee Azandaryani
Amir Mohammad Salehi
Misleading Rare Case of Idiopathic Hypertrophic Pachymeningitis
Case Reports in Medicine
title Misleading Rare Case of Idiopathic Hypertrophic Pachymeningitis
title_full Misleading Rare Case of Idiopathic Hypertrophic Pachymeningitis
title_fullStr Misleading Rare Case of Idiopathic Hypertrophic Pachymeningitis
title_full_unstemmed Misleading Rare Case of Idiopathic Hypertrophic Pachymeningitis
title_short Misleading Rare Case of Idiopathic Hypertrophic Pachymeningitis
title_sort misleading rare case of idiopathic hypertrophic pachymeningitis
url http://dx.doi.org/10.1155/2024/5561686
work_keys_str_mv AT ahmadrezaeeazandaryani misleadingrarecaseofidiopathichypertrophicpachymeningitis
AT amirmohammadsalehi misleadingrarecaseofidiopathichypertrophicpachymeningitis