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Título

Pachymeningitis: A Case Report

RESUMO

Case-report:
A 48 years old female , with history of headache with phenotype of migraine with visual aura. In 2005 she developed headache with pattern change, suggestive of intracranial hypertension, and targeted propedeutics was performed. On clinical and imaging investigation, indirect signs of intracranial hypertension were found on skull MRI such as: partially empty sella turcica, enlargement of the perioptic liquoric spaces and of the cavum of meckel, in addition to regular and diffuse pachymeningeal thickening at the level of the cerebellar tentorium, mainly on the right, as well as of the interhemispheric sickle, in its posterior aspect without a clear nodular aspect, a nonspecific finding from the etiological point of view, but may be associated with diverse conditions, such as pachymeningitis. The CSF was altered, with increased opening pressure (29 and 30); and inflammatory features (cels: 11 (leucus 97%), no macrophages, glucose: 58, prot: 119 ldh: 23). Ophthalmology evaluation with campimetry was without significant changes. Rheumatological and serological tests were negative, except for a high serum IgG 4 value (260.1 - VR: 4-86). Thus, he started treatment for IgG4 hypertrophic pachymeningitis with 1mg/kg/day Prednisone and Azathioprine 2-3mg/kg/day. After starting the medication, there was a significant improvement in symptoms, but with a side effect attributed to the corticosteroid, which is currently still being weaned.

Discussion:
IgG4-related hypertrophic pachymeningitis is only one of many possible IgG4-related diseases, it denotes inflammation and thickening of the dura mater and often comes associated with systemic symptoms such as fever, weight loss or abdominal pain. Neurological disorders in these patients are often associated with nerve or vascular compression, leading to focal deficits, but diffuse inflammation of the dura can lead to more diffuse presentations such as headache, cervical stiffness, and seizures. Its diagnosis remains a great challenge because of the compendium of possible presentations and its indolent character, which ends up delaying the search for medical assistance by patients.

Final Comments:
The early diagnosis of hypertrophic pachymeningitis is essential to stop disease progression and brain damage. The management of the disease still is a challenging due to its recent description in 2003, with a pathophysiological picture not yet fully understood.

Palavras Chave

Spinal Cord Ischemia, Paresis