MCID: IDP081
MIFTS: 17

Idiopathic Hypertrophic Pachymeningitis

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Idiopathic Hypertrophic Pachymeningitis

MalaCards integrated aliases for Idiopathic Hypertrophic Pachymeningitis:

Name: Idiopathic Hypertrophic Pachymeningitis 52
Idiopathic Hypertrophic Craniospinal Pachymeningitis 52
Idiopathic Hypertrophic Cranial Pachymeningitis 52
Idiopathic Hypertrophic Spinal Pachymeningitis 52

Classifications:



Summaries for Idiopathic Hypertrophic Pachymeningitis

NIH Rare Diseases : 52 Idiopathic hypertrophic pachymeningitis (IHP) is a rare disorder causing inflammation and thickening of the outer layer (dura) of the brain and/or spinal cord. IHP can be widespread or cause tumor -like lesions. Before IHP can be diagnosed, other conditions including sarcoidosis, tumors, meningioma, infections (syphilis, tuberculosis, and Lyme disease) , and autoimmune diseases ( rheumatoid arthritis , Sjogren's syndrome , Wegener's granulomatosis, and IgG4-related disease) must be ruled out. IHP often presents with headache and cranial nerve impairment . Treatment may involve prednisone and/or an immune suppressing drug. This treatment often improves symptoms, however complete recovery is rare. Surgery may be recommended for people with advanced or severe IHP. Some people with IHP have no symptoms and may not need treatment.

MalaCards based summary : Idiopathic Hypertrophic Pachymeningitis, also known as idiopathic hypertrophic craniospinal pachymeningitis, is related to igg4-related pachymeningitis and headache. Affiliated tissues include brain, spinal cord and neutrophil.

Related Diseases for Idiopathic Hypertrophic Pachymeningitis

Diseases related to Idiopathic Hypertrophic Pachymeningitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 98)
# Related Disease Score Top Affiliating Genes
1 igg4-related pachymeningitis 12.0
2 headache 10.6
3 meningioma, radiation-induced 10.5
4 meningioma, familial 10.5
5 spinal meningioma 10.5
6 secretory meningioma 10.5
7 lymphoplasmacyte-rich meningioma 10.5
8 tolosa-hunt syndrome 10.4
9 status epilepticus 10.4
10 brain edema 10.4
11 ataxia and polyneuropathy, adult-onset 10.3
12 cranial nerve palsy 10.3
13 thrombosis 10.3
14 uveitis 10.3
15 diabetes insipidus 10.3
16 autoimmune disease 10.3
17 syphilis 10.3
18 vasculitis 10.3
19 systemic autoimmune disease 10.3
20 rapidly involuting congenital hemangioma 10.3
21 quadriplegia 10.3
22 otitis media 10.2
23 sarcoidosis 1 10.2
24 3-methylglutaconic aciduria, type iii 10.2
25 igg4-related disease 10.2
26 hydrocephalus 10.2
27 pituitary apoplexy 10.2
28 neurosarcoidosis 10.2
29 papilledema 10.2
30 neuritis 10.2
31 optic nerve disease 10.2
32 anisocoria 10.1
33 migraine with or without aura 1 10.1
34 thrombophilia due to thrombin defect 10.1
35 yemenite deaf-blind hypopigmentation syndrome 10.1
36 pars planitis 10.1
37 atrioventricular block 10.1
38 horner's syndrome 10.1
39 intermediate uveitis 10.1
40 scleritis 10.1
41 polyneuropathy 10.1
42 transient cerebral ischemia 10.1
43 macular retinal edema 10.1
44 peripheral nervous system disease 10.1
45 thyroiditis 10.1
46 myocarditis 10.1
47 lymphocytic hypophysitis 10.1
48 dysphagia 10.1
49 trigeminal autonomic cephalalgia 10.1
50 lymphoma 10.1

Graphical network of the top 20 diseases related to Idiopathic Hypertrophic Pachymeningitis:



Diseases related to Idiopathic Hypertrophic Pachymeningitis

Symptoms & Phenotypes for Idiopathic Hypertrophic Pachymeningitis

Drugs & Therapeutics for Idiopathic Hypertrophic Pachymeningitis

Search Clinical Trials , NIH Clinical Center for Idiopathic Hypertrophic Pachymeningitis

Genetic Tests for Idiopathic Hypertrophic Pachymeningitis

Anatomical Context for Idiopathic Hypertrophic Pachymeningitis

MalaCards organs/tissues related to Idiopathic Hypertrophic Pachymeningitis:

40
Brain, Spinal Cord, Neutrophil, Pituitary

Publications for Idiopathic Hypertrophic Pachymeningitis

Articles related to Idiopathic Hypertrophic Pachymeningitis:

(show top 50) (show all 110)
# Title Authors PMID Year
1
Dark as night: Spelunking for spinal solitary fibrous tumors/hemangiopericytomas in the differential of T2 hypointensity. 61
32507710 2020
2
Intracranial Inflammatory Pseudotumor Associated with Idiopathic Hypertrophic Pachymeningitis Mimicking Malignant Tumor or High-Grade Meningioma. 61
31704361 2020
3
Idiopathic Hypertrophic Spinal Pachymeningitis: A Diagnostic Challenge: A Case Report and Review of the Literature. 61
32140024 2020
4
Headache and Hypoglossal Nerve Palsy in a Child With Idiopathic Hypertrophic Pachymeningitis. 61
31294820 2019
5
Idiopathic Hypertrophic Spinal Pachymeningitis: A Rare Cause of Spinal Cord Compression. 61
31744986 2019
6
Idiopathic Hypertrophic Pachymeningitis Mimicking Meningioma with Occlusion of Superior Sagittal Sinus: Case Report and Review of Literature. 61
30965168 2019
7
Cerebrospinal fluid cytokine/chemokine/growth factor profiles in idiopathic hypertrophic pachymeningitis. 61
30784775 2019
8
Letter to the editor: citations for the case report on idiopathic hypertrophic pachymeningitis by Kosuke Tsuchida et al. 61
30673832 2019
9
Rapidly progressive fatal idiopathic hypertrophic pachymeningitis with brainstem involvement in a child. 61
29730790 2018
10
Hypertrophic Pachymeningitis of the Internal Auditory Canal: A Rare Case of Unilateral Sudden Sensorineural Hearing Loss. 61
29938521 2018
11
Diplopia With Dural Fibrotic Thickening. 61
29961529 2018
12
Ocular involvement in idiopathic hypertrophic pachymeningitis associated with eosinophilic angiocentric fibrosis: a case report. 61
29924200 2018
13
Marked effectiveness of low-dose oral methotrexate for steroid-resistant idiopathic hypertrophic pachymeningitis: Case report. 61
29510265 2018
14
Idiopathic hypertrophic pachymeningitis mimicking hemicrania continua: An unusual clinical case. 61
28474987 2018
15
Idiopathic hypertrophic pachymeningitis as a rare cause of cervical compressive myelopathy. 61
29403257 2017
16
Bacterial meningitis due to Streptococcus pneumoniae following untreated idiopathic hypertrophic pachymeningitis. 61
28870586 2017
17
Concomitant idiopathic hypertrophic spinal pachymeningitis and Guillain-Barré syndrome in a patient: coincidence or a triggering mechanism? 61
28644069 2017
18
A case of idiopathic hypertrophic pachymeningitis presenting with chronic headache and multiple cranial nerve palsies: A case report. 61
28723776 2017
19
Letter re: Teaching NeuroImages: Idiopathic hypertrophic pachymeningitis. 61
28583934 2017
20
Author response: Teaching NeuroImages: Idiopathic hypertrophic pachymeningitis. 61
28583935 2017
21
Rituximab Treatment for Idiopathic Hypertrophic Pachymeningitis. 61
28271643 2017
22
Central Nervous System Inflammatory Myofibroblastic Tumor Masquerading as Chronic Suppurative Otitis Media. 61
28904584 2017
23
Unilateral optic disc swelling associated with idiopathic hypertrophic pachymeningitis: a rare cause for a rare clinical finding. 61
28348267 2017
24
Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease. 61
27592235 2017
25
Effective Response of Methotrexate for Recurrent Idiopathic Hypertrophic Spinal Pachymeningitis. 61
28127378 2016
26
Teaching NeuroImages: Idiopathic hypertrophic pachymeningitis. 61
27895252 2016
27
Report of a rare case of atypical lymphoplasmacyte-rich meningioma in the tentorium mimicking idiopathic hypertrophic pachymeningitis. 61
26902101 2016
28
[Review of ear and nose and throat involvement in IgG4-RD]. 61
26911076 2015
29
[IgG4- and MPO-ANCA-associated hypertrophic pachymeningitis]. 61
26553176 2015
30
Idiopathic Hypertrophic Pachymeningitis in the Craniocervical Junction. 61
26512276 2015
31
Idiopathic hypertrophic pachymeningitis mimicking prolactinoma with recurrent vision loss. 61
26238133 2015
32
Infiltrative mass of the skull base and nasopharynx: A diagnostic conundrum. 61
25905016 2015
33
[Lymphocytic hypophysitis and hypertrophic pachymeningitis: description of a possible case associated to IgG4 pathologies]. 61
26005074 2015
34
Idiopathic hypertrophic spinal pachymeningitis: a case report and review of literature. 61
25906379 2015
35
Teaching NeuroImages: Idiopathic hypertrophic spinal pachymeningitis mimicking epidural lymphoma. 61
25732370 2015
36
Idiopathic hypertrophic pachymeningitis presenting with occipital neuralgia. 61
25834565 2015
37
Clinico-radiological spectrum and outcome in idiopathic hypertrophic pachymeningitis. 61
25703277 2015
38
[Optic neuropathy in a case of recurrent idiopathic hypertrophic pachymeningitis unresponsive to steroids and immunosuppressants]. 61
25443460 2015
39
[A case of bilateral ophthalmoplegia caused by focal idiopathic hypertrophic pachymeningitis on the anterior cranial fossa]. 61
25672863 2015
40
Recurrent cerebral aneurysm formation and rupture within a short period due to invasive aspergillosis of the nasal sinus; pathological analysis of the catastrophic clinical course. 61
26722566 2015
41
Idiopathic Hypertrophic Spinal Pachymeningitis. 61
26234238 2015
42
Interval progression of idiopathic hypertrophic pachymeningitis. 61
29443163 2014
43
Arachnoid involved in idiopathic hypertrophic pachymeningitis. 61
25199674 2014
44
Pachymeningitis in a young child responded to antitubercular therapy: a case report. 61
24092891 2014
45
Idiopathic hypertrophic spinal pachymeningitis with an osteolytic lesion. 61
25328657 2014
46
Idiopathic hypertrophic pachymeningitis: clinical, laboratory and neuroradiologic features in China. 61
24589555 2014
47
IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. 61
24733677 2014
48
Idiopathic hypertrophic pachymeningitis at King Chulalongkorn Memorial Hospital. 61
24964678 2014
49
Peace sign in calcified idiopathic hypertrophic pachymeningitis. 61
24534041 2014
50
See no evil, hear no evil…. 61
23911152 2014

Variations for Idiopathic Hypertrophic Pachymeningitis

Expression for Idiopathic Hypertrophic Pachymeningitis

Search GEO for disease gene expression data for Idiopathic Hypertrophic Pachymeningitis.

Pathways for Idiopathic Hypertrophic Pachymeningitis

GO Terms for Idiopathic Hypertrophic Pachymeningitis

Sources for Idiopathic Hypertrophic Pachymeningitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
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35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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