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Wegener's granulomatosis presenting with multiple cranial nerve palsies and pachymeningitis
  1. Ahmed Mohammed Iqbal,
  2. Daniel Blackburn,
  3. Muhammad Rafiq,
  4. Basil Sharrack
  1. Department of Neurology, Sheffield Teaching Hospitals,  Sheffield, UK
  1. Correspondence to Dr Ahmed Mohammed Iqbal, Department of Neurology, Sheffield Teaching Hospitals, Royal Hallamshire Hospital, Glossop Road, Sheffield S10 2JF, UK; ahmed742{at}doctors.org.uk

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A previously healthy 27-year-old male professional singer presented with a 1-month history of difficulty in singing, fever and night sweats. He had been given a 1-week course of oral corticosteroids and antibiotics for a presumed diagnosis of acute laryngitis, with some clinical improvement.

Six weeks later, he presented again with diplopia, difficulty in swallowing and prominent dysphonia. There was no headache, epistaxis or nasal symptoms.

Neurological examination showed left-sided VI, IX and X cranial nerve palsies.

His erythrocyte sedimentation rate was 72 mm/1st h (<15) and serum C reactive protein was 46.2 mg/l (<10).

HIV, hepatitis B, hepatitis C and trepenomal serology were negative.

Initial immunological screen for anti-nuclear and anti-centromere antibodies was negative. Cerebrospinal fluid (CSF) examination was normal and viral PCR negative. MRI brain showed asymmetrical pachymeningeal enhancement and inflammatory change in the left mastoid and petrous temporal bones, extending into the left middle ear (figure 1A).

Figure 1

(A) T1-weighted axial magnetic resonance image of the brain showing asymmetric pachymeningeal enhancement with inflammatory change in the left mastoid and petrous bones extending to the left middle ear cavity. (B) Magnetic resonance venography demonstrating absent flow within the left transverse …

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