A case report: Diagnosis and treatment of idiopathic hypertrophic pachymeningitis

Abstract

A 57‐year‐old man who suffered from a headache for 1 year, accompanied by blurred vision for 7 months and numbness in his left face for 1 week was admitted to the Affiliated Hospital of Zunyi Medical University on May 7, 2022. One year ago, the patient had no obvious precipitating factor of paroxysmal stabbing pain in the whole skull with dizziness, which could be relieved by oneself after lasting for 1–2 min each time, with about 20 episodes per day. The cranial magnetic resonance imaging revealed changes in bilateral frontal lobe ischemic foci, bilateral frontal, ethmoid, sphenoid and maxillary sinusitis, and retinal macular degeneration. After hormone shock treatment, the condition improved. He suffered from headaches again with blurred vision in the right eye 7 months ago and was initially diagnosed with multiple sclerosis. He then was discharged after improvement due to hormone shock therapy. Oral hormone therapy was continued outside the hospital, but he stopped it due to drug side effects (details remained unclear). After cutting off, he developed a headache and visited our hospital once more, the relevant tests were performed and the patient was diagnosed with idiopathic hypertrophic pachymeningitis (IHP). The symptoms were slightly abated after hormone therapy. We hope that through this case report, we can deepen the clinicians' understanding of IHP, and improve the diagnosis rate of the disease through relevant examinations in future clinical work, so that patients can receive timely treatment and the mental pressure and economic burden caused by the disease on patients are reduced.

An Incidental Finding of Idiopathic Hypertrophic Pachymeningitis: A Case Report

“…The etiology of hypertrophic pachymeningitis is complex, and in this particular case, a differential diagnosis of rheumatoid arthritis, tuberculosis, syphilis, IgG4-related diseases, and lymphoma were all considered before classifying this patient with idiopathic hypertrophic pachymeningitis [1,3,7]. The patient in this case did not have a medical history of any underlying autoimmune disease, infection, or systemic diseases, and no such findings were found on a thorough history and laboratory examination.…”

“…Only about 64 cases of IHP have been reported in the English medical literature from 1860 to 2010, with a current incidence rate of about 0.949 per 100,000 [3]. Early symptoms can be mild and non-specific, which commonly leads to their misdiagnosis.…”

“…The majority of cases of IHP initially presented with headache, loss of vision, diplopia, papilledema, cranial nerve involvement, ataxia, and seizures [7]. Cranial nerves commonly involved include the optic nerve (II), oculomotor nerve (III), trochlear nerve (IV), abducens nerve (VI), and facial nerve (VII), hence the predominantly visual symptoms are seen in many IHP patients [3]. However, this patient denied any symptoms that could be related to this pachymeningitis other than a sudden episode of speech arrest that could be associated with the underlying meningioma that was found on the MRI.…”

“…A study done in Japan showed that 87.2% of the 94 patients treated with corticosteroids had a complete resolution of their symptoms. For patients who did not respond to corticosteroids alone, a combination of immunosuppressive agents alleviated symptoms in 92.6% of the patients [3]. Some patients require surgery if all other treatment options fail.…”

“…A dural biopsy is essential to exclude secondary causes of pachymeningitis [2]. Idiopathic hypertrophic pachymeningitis has been shown to respond to corticosteroids; however, there is a high rate of recurrence when weaned or withdrawn over time [3,4]. As a distinct disease, IHP is certainly a rare disorder of diverse etiology.…”