Churg-Strauss syndrome

Case contributed by Dalia Ibrahim

Presentation

Headache. Bilateral lower limb weakness and numbness.

Patient Data

Age: 45 years
Gender: Female
mri

Multifocal pachymeningeal thickening is seen at the high frontal parafalcine region and along the tentorial reflections. The pachymeningeal thickening elicits isointense/low signal on T1 and T2 WI with vivid post-contrast enhancement.

Multiple parenchymal foci and patches of an abnormal signal are seen at the middle cerebellar peduncles, pons, and midbrain eliciting a high signal on T2 & FLAIR WI with no diffusion restriction or post-contrast enhancement.

Conclusion:

  • Hypertrophic pachymeningitis which could be secondary to infection (TB), inflammatory/granulomatous process (Wegner, IgG4, Rosai Dorfman, sarcoid), or less likely neoplastic (metastatic)
  • Multiple foci of abnormal signal at the middle cerebellar peducnles, pons and midbrain, likely vasculitic.

Upon further questioning, the patient mentioned a chronic history of asthma. Clinical examination showed peripheral neuropathy - mononeuritis multiplex. CBC revealed peripheral eosinophilia >15%.

ct

Bilateral maxillary, frontal, and sphenoid chronic sinusitis showing diffuse sclerosis and mucosal thickening.

ct

CT scan of the chest, abdomen, and pelvis showed:

Bilateral pulmonary mainly upper lobar peripheral subpleural ground-glass patches. Smooth interlobuar thicknieng is also noted.

Normal CT scan of the abdomen and pelvis.

Conclusion:

Pulmonary findings are suggestive of eosinophilic lung disease, which in view of clinical and labratory data are likely representing Churg-Strauss syndrome.

Case Discussion

Our case demonstrated more than four out of the six criteria of Churg Strauss syndrome including asthma, hypereosinophilia (>15%), paranasal sinusitis, pulmonary infiltrates, and peripheral polyneuropathy.

The neurological radiographic manifestations were in the form of hypertrophic pachymeningitis, which was suggestive of a granulomatous process. Also, small parenchymal patches of abnormal signal are seen at the midbrain, pons and middle cerebellar peduncles, likely vasculitic.

Paranasal sinus CT revealed chronic sinusitis and chest CT showed pulmonary infiltrates with patchy ground-glass attenuation and smooth interlobular thickening which are suggestive of eosinophilic lung disease.

Churg Strauss syndrome is a multisystemic disorder characterized by necrotizing vasculitis of the small vessels, associated with extravascular eosinophilic granulomas that occur exclusively in patients with asthma and peripheral eosinophilia.

At least four of the following six criteria should be present; asthma, eosinophilia greater than 10% in peripheral blood, paranasal sinusitis, migratory or transient pulmonary infiltrates, polyneuropathy, and biopsy-proven extra-vascular eosinophils.

Chest CT in patients with Churg Strauss syndrome usually show ground glass opacities in a lobular distribution without zonal predilection and are predominantly peripheral, resembling those in chronic eosinophilic pneumonia, and can be transient, resembling findings of simple pulmonary eosinophilia. Other manifestations include smooth interlobular septal thickening, diffuse interstitial reticular or reticulonodular, and bronchial wall thickening.

Meningeal involvement in Churg Strauss disease is not common and has been reported a few times 1,2.

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