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Idiopathic hypertrophic pachymeningitis mimicking hemicrania continua: An unusual clinical case

Published on Apr 1, 2018in Cephalalgia 4.44
· DOI :10.1177/0333102417708773
Antonio Russo66
Estimated H-index: 66
,
Marcello Silvestro3
Estimated H-index: 3
+ 2 AuthorsGioacchino Tedeschi34
Estimated H-index: 34
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Abstract
BackgroundHemicrania continua (HC) is a primary headache syndrome characterized by a unilateral, moderate, continuous headache with exacerbations marked by migrainous and cranial autonomic symptoms. However, clinical phenotypes similar to primary HC may be subtended by several disorders.Case reportWe report the case of a 62-year-old man experiencing, over the previous year, a headache completely consistent with HC and its absolute responsiveness to indomethacin therapy. Later, the patient developed diplopia caused by sixth cranial nerve palsy ipsilateral to headache. In this frame, clinical, laboratory and neuroimaging characteristics supported the diagnosis of idiopathic hypertrophic pachymeningitis (IHP).ConclusionsIHP is a rare fibrosing inflammatory disorder leading to a localized or diffuse dura mater thickening. IHP clinical manifestations are a progressively worsening headache and signs related to cranial nerves involvement and venous sinus thrombosis. Here, we report, for the first time, a HC phen...
  • References (10)
  • Citations (4)
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References10
Newest
Published on Jul 1, 2014in Journal of Clinical Neuroscience 1.59
Mangsuo Zhao3
Estimated H-index: 3
(THU: Tsinghua University),
Tongchao Geng2
Estimated H-index: 2
(THU: Tsinghua University)
+ 6 AuthorsHuancong Zuo2
Estimated H-index: 2
(THU: Tsinghua University)
Abstract Hypertrophic pachymeningitis is a rare chronic inflammatory disorder characterized by marked fibrous thickening of the cerebral and/or spinal dura mater. Clinical, laboratory, neuroradiologic and therapeutic data from 12 patients with idiopathic hypertrophic pachymeningitis (IHP) from our department were retrospectively studied. There were four men and eight women with a mean age of 49 ± 15.3 years, and more than half of the patients (58%) were aged 40–60 years. Headache was the most co...
Published on Jun 1, 2014in JAMA Neurology 12.32
Lucy X. Lu1
Estimated H-index: 1
(Vandy: Vanderbilt University),
Emanuel Della-Torre13
Estimated H-index: 13
(UniSR: Vita-Salute San Raffaele University)
+ 1 AuthorsStephen W. Clark5
Estimated H-index: 5
(Vandy: Vanderbilt University)
Importance IgG4-related hypertrophic pachymeningitis (IgG4-RHP) is an increasingly recognized manifestation of IgG4-related disease, a fibroinflammatory condition that can affect virtually any organ. It is estimated that IgG4-RHP may account for a high proportion of cases of hypertrophic pachymeningitis once considered idiopathic. Objective To summarize the current knowledge on IgG4-RHP including its pathological, clinical, and radiological presentations. Particular emphasis is placed on diagnos...
Published on Jan 1, 2014in Journal of Neuroimmunology 2.83
Emanuel Della-Torre13
Estimated H-index: 13
(UniSR: Vita-Salute San Raffaele University),
Laura Galli1
Estimated H-index: 1
+ 7 AuthorsMaria Grazia Sabbadini35
Estimated H-index: 35
(UniSR: Vita-Salute San Raffaele University)
Abstract Diagnosis of IgG4-Related Hypertrophic Pachymeningitis (IgG4-HP) relies on meningeal biopsies, because cerebrospinal fluid (CSF) diagnostic biomarkers are lacking. Here, we determined whether IgG4 intrathecal production could distinguish IgG4-HP from other disorders presenting with HP (OHP). In patients with IgG4-HP, the median CSF IgG4 concentration, IgG4 Index and IgG4 Loc were significantly higher than in both controls and OHP. CSF IgG4 levels higher than 2.27 mg/dL identified 100% o...
Published on Oct 1, 2013in Headache 3.75
Arne May1
Estimated H-index: 1
Although severe short-lasting headaches are rare, they can be considered disabling conditions with a major impact on the quality of life of patients. These headaches can divided broadly in to those associated with autonomic symptoms, so called trigeminal autonomic cephalgias (TACs), and those with few or no autonomic symptoms. The TACs include cluster headache, paroxysmal hemicranias, hemicrania continua, and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms ...
Published on Feb 1, 2013in Journal of Headache and Pain 3.92
Michele Viana15
Estimated H-index: 15
,
Cristina Tassorelli37
Estimated H-index: 37
(UNIPV: University of Pavia)
+ 3 AuthorsFabio Antonaci29
Estimated H-index: 29
(UNIPV: University of Pavia)
Trigeminal autonomic cephalalgias (TACs) and hemicrania continua (HC) are relatively rare but clinically rather well-defined primary headaches. Despite the existence of clear-cut diagnostic criteria (The International Classification of Headache Disorders, 2nd edition - ICHD-II) and several therapeutic guidelines, errors in workup and treatment of these conditions are frequent in clinical practice. We set out to review all available published data on mismanagement of TACs and HC patients in order...
Published on Jan 1, 2012in Headache 3.75
Jane W. Chan1
Estimated H-index: 1
(UNR: University of Nevada, Reno)
This is the first report of 2 patients presenting with short-lasting unilateral neuralgiform headache with autonomic symptoms as the initial manifestation of idiopathic hypertrophic cranial pachymeningitis. They both had acute retro-orbital pain ipsilateral to the dural thickening on magnetic resonance imaging of brain, and one had transient miosis as an additional parasympathetic feature. Short-lasting unilateral neuralgiform headache with autonomic symptoms syndrome may be associated with seco...
Published on May 1, 2009in Journal of the Neurological Sciences 2.65
Sanjay Prakash12
Estimated H-index: 12
(Baroda Medical College),
Nilima D. Shah8
Estimated H-index: 8
(Baroda Medical College),
Ritu Kanchan Soni1
Estimated H-index: 1
Hemicrania continua (HC) is an indomethacin responsive primary headache disorder. Secondary or symptomatic HC is associated with another neurological or non-neurological disease. We report three patients with secondary HC. We also review the literature to identify the clinical predictors of an underlying disease entity. Intracranial structural lesion, head and neck vessel pathology, and carcinoma lung should be suspected in every patient. The factors that may suggest a secondary pathology are: e...
Published on Mar 9, 2004in Neurology 8.69
Mark J. Kupersmith47
Estimated H-index: 47
,
V. Martin1
Estimated H-index: 1
+ 2 AuthorsH.J. Mitnick1
Estimated H-index: 1
Background: Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater and has been associated with rheumatoid arthritis, syphilis, Wegener’s granulomatosis, tuberculosis, and cancer. Few series of the idiopathic variety have been described, particularly with respect to MRI correlation to clinical outcome and treatment. Objective: To investigate the clinical and laboratory evaluation, course, and treatment of patients with idiopathic hype...
Published on Feb 1, 1998in Headache 3.75
Fabio Antonaci29
Estimated H-index: 29
,
Juan Antonio Pareja Grande38
Estimated H-index: 38
+ 1 AuthorsOttar Sjaastad38
Estimated H-index: 38
The interval between indomethacin administration and clinical response may be clinically relevant in the assessment of chronic paroxysmal hemicrania and hemicrania continua and other unilateral headache disorders with which they can be confounded. Eight patients with chronic paroxysmal hemicrania (6 women and 2 men) and 12 patients with hemicrania continua (8 women and 4 men) were entered into the study. The patients were given 50 mg of indomethacin intramuscularly (IM) on day 1 and some of them...
Cited By4
Newest
Published on Apr 1, 2019in World Neurosurgery 1.72
Anhui Yao , Liyun Jia + 3 AuthorsBainan Xu11
Estimated H-index: 11
Background Idiopathic hypertrophic pachymeningitis is a rare inflammatory condition with diffuse thickening of the dura mater, which may cause a compressive effect or vascular compromise. Case Description A 40-year-old Chinese man presented with persistent headache for 6 months and a sudden epileptic seizure 2 days ago. Magnetic resonance imaging demonstrated a large (71 × 34 × 27 mm) extra-axial mass at the right frontal convexity with severe edema mimicking meningioma. The lesion and periphera...
Published on Feb 1, 2019in Neurological Sciences 2.48
Hao Chen3
Estimated H-index: 3
,
Wei Zhang1
Estimated H-index: 1
+ 7 AuthorsLiguo Dong1
Estimated H-index: 1
Objective To explore the clinical and imaging features of patients with hypertrophic cranial pachymeningitis (HCP).
Published on Sep 27, 2018in Case Reports
We report the rare case of an 80-year-old male patient with hypertrophic pachymeningoencephalitis that may be associated with temporal arteritis. The patient presented to our neurological department with a 2-week history of latent paresis and ataxia affecting his right hand. He had been diagnosed with temporal arteritis 12 years earlier. Brain MRI showed an enhancement of the left-sided frontoparietal meninges with oedema of the adjacent tissue of the precentral and postcentral cortex. A leptome...