Abstract Headache is a common symptom in patients with granulomatosis with polyangiitis GPA mainly due to chronic sinusitis or orbital disease. Meningeal involvement may thus remain unrecognized for a long time. This can lead to a significant delay in accurate diagnosis, serious local damage of the central nervous system and high relapse rates. New diagnostic techniques such as contrast MRI allow one to identify inflammation of the dura mater in the course of GPA more frequently.
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Introduction Idiopathic hypertrophic pachymeningitis is a rare inflammatory condition characterised by fibrosis and thickening of the dura mater. Diagnosis of idiopathic hypertrophic pachymeningitis requires a high index of suspicion, as its initial manifestation could be subtle clinically and radiologically.
Idiopathic hypertrophic pachymeningitis has posed considerable diagnostic challenges to attending clinicians, including radiologists, neurologists, and ophthalmologists because of its highly variable presentation. Apart from clinical subtlety and variability, idiopathic hypertrophic pachymeningitis is also a great imposter because it can mimic other common and important neurological conditions such as prolactinoma.
Case report A year-old Chinese woman with a history of granulomatous mastitis 7 years previously was noted to have oligomenorrhoea, nausea, headache, and raised prolactin level of Her baseline hormone profile was otherwise normal. Plain magnetic resonance imaging MRI scan showed an enlarged pituitary gland of up to 1. At that time she was diagnosed with prolactinoma and treated with bromocriptine; her prolactin level was well-controlled subsequently.
Figure 1. Magnetic resonance images showing generalised increased pachymeningeal enhancement in the bilateral frontal-parietal-temporal regions just before steroid treatment in June Non-contrast a coronal and b sagittal scans showing an enlarged pituitary gland of up to 1. Humphrey visual field test showed superior field loss in the right eye, while the left eye was full field loss.
Other slit-lamp and fundal examinations were unremarkable. There was no papilloedema. There was no involvement of other cranial nerves.
Magnetic resonance imaging with contrast showed increased contrast enhancement and inflammation over the dura of the sella and cavernous sinus Figs 1c and 1d. Her symptoms resolved and visual function recovered quickly with high-dose steroid. However, she developed steroid dependency since repeated attacks developed when the steroid was stopped.
Serial MRI scan showed progressive diffuse thickening of the pachymeningitis with disappearance of pituitary apoplexy, together with chronic otitis media Figs 1c and 1d. Lumbar puncture showed 13 cm H2O and lymphocytosis without organisms. Open biopsy of the meninges was performed and histology showed features of inflammatory infiltrates and vasculitis, but was negative for malignancy Fig 2. Gene rearrangement polymerase chain reaction assay for immunoglobulin Ig heavy chain, T-cell receptor TCR —beta and TCR-gamma all showed no clonal peak.
Complete blood count showed normal haemoglobin, white blood cell, and platelet levels. Liver and renal function tests, serum calcium, creatine kinase, and lactate dehydrogenase levels were normal. C-reactive protein was raised at In coagulation profile, activated partial thromboplastin time was slightly prolonged to Autoimmune markers, including anti-nuclear antibodies, anti-neutrophil cytoplasmic antibodies, anti-DNA immunofluorescence test, anti-extractable nuclear antigen, anti-cardiolipin antibodies, and rheumatoid factors are all negative except for the presence of lupus anticoagulant.
Complement C3 was normal while complement C4 was slightly raised to 0. Virology screening—including human immunodeficiency virus, hepatitis B virus, and hepatitis C virus—was negative.
Serum and cerebrospinal fluid Venereal Disease Research Laboratory tests were negative. Chest X-ray was clear with no features of tuberculosis or sarcoidosis. Figure 2. She remained symptom-free 6 months after biopsy. Thickened dura mater with local mass effect may be pathognomonic of this condition. This pressure effect may serve as a mechanistic explanation of the observed neurological defect.
In this patient, these tests were all negative, so the diagnosis of idiopathic hypertrophic pachymeningitis was made. Demographically, the median age of patients with idiopathic hypertrophic pachymeningitis is There are few data on ethnicity due to the rarity of the disease. Idiopathic hypertrophic pachymeningitis is extremely rare in Chinese people.
The exact aetiopathophysiology of idiopathic hypertrophic pachymeningitis is not known. It is believed to be autoimmune in origin. Only the presence of lupus coagulants might suggest that idiopathic hypertrophic pachymeningitis is a form of vasculitis, which might share some common phenomenon with other autoimmune diseases, although the true relationship is controversial.
Clinically, headache is by far the most common feature and the optic nerve is one of the most common cranial nerves to be involved, which was the case for this patient. The posterior lobe was spared in their patient, who had a normal hormonal profile, unlike our patient.
The initial enlarged pituitary gland with raised prolactin was more likely to result from the stalk effect than from true prolactinoma. Granulomatous mastitis is a rare idiopathic chronic benign breast condition, which is believed to be autoimmune in origin, and mainly affects women of child-bearing age.
To the best of our knowledge, this is the first case of idiopathic hypertrophic pachymeningitis reported with the association of granulomatous mastitis, possibly related to the scarcity of cases affecting menstruating women.
Idiopathic hypertrophic pachymeningitis is a rare condition with a highly variable clinical presentation making accurate and timely diagnosis difficult. Therefore the attending clinician should maintain high vigilance in the event of an atypical presentation of a presumably typical disease. Early diagnosis and prompt therapeutic intervention such as high-dose steroid may be the key to preserving vision as well as life.
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Neurology ; Crossref 7. Pachymeningitis in a young child responded to antitubercular therapy: a case report. J Child Neurol ;NP Crossref 8. Inflammatory hypertrophic cranial pachymeningitis. Presse Med ; Antiphospholipid antibody testing for the antiphospholipid syndrome: a comprehensive practical review including a synopsis of challenges and recent guidelines. Pathology ; Crossref Idiopathic granulomatous mastitis: a rare entity with a variable presentation.
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Idiopathic hypertrophic pachymeningitis