Dr Chamani Kodikara1, Dr Erin Roche1, Dr Arzoo Khalid1,2, Dr Timothy Pianta1,2
1The Northern Hospital, Epping, Melbourne, Australia, 2University of Melbourne, Parkville, Melbourne, Australia
Biography:
Biographies to come
Abstract:
Background: The term pachymeningitis describes an uncommon phenomenon of thickening or inflammation of the dura mater, typically visualised on Magnetic Resonance Imaging (MRI)¹. Clinical features commonly include headaches and visual or hearing changes but rarely encephalopathy and seizures². Most cases are idiopathic, but the differential diagnosis includes sarcoidosis, autoimmune conditions and immunoglobulin-G4 related disease (IgG4-RD)². Pachymeningitis has not been described in pregnancy or peripartum. There is no established link with HELLP syndrome, disseminated intravascular coagulopathy (DIC), complement-mediated thrombotic microangiopathy (CM-TMA), or acute fatty liver of pregnancy (AFLP).
Case: A 30-year-old primigravida presented at term with reduced foetal movements, abdominal pain and vomiting. She had hypertension, proteinuria, acute kidney injury, liver dysfunction and DIC. Following emergency Caesarean-section under general anaesthesia, a live female birthweight 3.63kg was delivered.
Post-operatively, she required re-intubation for prolonged unresponsiveness. Following a normal computed tomography brain and perfusion series at D1 post-partum, an MRI brain at D3 demonstrated diffuse dural thickening, T2 and FLAIR hyperintensity, and postcontrast enhancement representing pachymeningitis. ANA and serum IgG4 levels were elevated, complements C3 and C4 were reduced, however other markers of autoimmune conditions including ENA were normal.
Further complications included pancreatitis, intermittent hypoglycaemia, ileus, and rectus sheath haematoma. A diagnosis of AFLP was proposed. However, ongoing haemolysis, thrombocytopaenia and coagulopathy prompted empirical therapy for TMA transiently comprising plasma exchange and methylprednisolone at D2, and eculizumab at D8. Haemolysis and thrombocytopaenia rapidly improved thereafter.
The patient demonstrated substantial neurological recovery at D4. Repeat MRI at D11 demonstrated reduction in pachymeningeal enhancement. The patient and infant remain well 1-month post-partum. We plan withdrawal of eculizumab, repeat serological assessment, and testing for a genetic predisposition to CM-TMA and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.
Conclusion: We report peripartum pachymeningitis with neurological dysfunction in a critically unwell patient. The mechanism for this finding remains to be elucidated.
Keywords
Pachymeningitis, Acute Fatty Liver of Pregnancy (AFLP), Disseminated Intravascular Coagulopathy (DIC)
References
1. Matias TB, Cordeiro RA, Duarte JA, de Jarry VM, Appenzeller S, Villarinho L, Reis F. Immune-Mediated Hypertrophic Pachymeningitis and its Mimickers: Magnetic Resonance Imaging Findings. Acad Radiol. 2023;30(11):2696-2706.
2. Gilbert E, Al-Awqati M, Gunderson T, Berianu F. Pachymeningitis: The Mayo Clinic Experience. Arthritis Rheumatol. 2021;73 Suppl 9:1-4259.