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In Wegener's granulomatosis (WG), neurological involvement is rare at onset. We present an unusual case where headache was the initial, dominant presentation of WG.
A 34 year old white man presented with a three month history of headache. The headaches were migratory, throbbing, and were accentuated with head movement. Physical examination was normal. Computed tomography (CT) of the sinuses was normal. The patient was diagnosed with non-specific vascular headaches, and was prescribed pizotifen, which alleviated his headaches.
One month later, the patient developed a red right eye. Bilateral papilloedema was noted. He was now unable to work because of the headache. Magnetic resonance imaging (MRI) of the brain disclosed a normal ventricular system, but pronounced gadolinium enhancement of the meninges around the entire left hemisphere, most of the parieto-occipital region on the right, as well as the tentorium bilaterally (fig 1). Lumbar puncture disclosed a high cerebrospinal fluid (CSF) opening pressure of 27 cm (13–18 cm). A CSF examination was entirely normal and cultures were negative. The headache was partially relieved by CSF drainage, and acetazolamide was started.
One week later, the patient developed a red left eye and left knee arthritis. Over the course of the next week, his condition progressed rapidly with purpuric lesions appearing on his hands and feet, followed by pericarditis and pulmonary haemorrhage. Biopsy of the purpura disclosed leucocytoclastic vasculitis. Antineutrophil cytoplasmic antibody (cANCA) taken at the time that he complained of the red left eye was positive at a titre of 1/80, with specificity for proteinase-3. A week later, repeat testing showed that cANCA had risen to 1/320. There was also a mild normochromic, normocytic anaemia, and raised inflammatory markers. Urine analysis disclosed microscopic haematuria and mild proteinuria. No casts were identified. A CSF examination was again normal, but the opening pressure had risen to 36 cm. WG was diagnosed.
Treatment was started with a 1 g pulse of intravenous methylprednisone, followed by oral daily doses of 1 mg/kg prednisone and 2 mg/kg cyclophosphamide. A few days after the start of treatment, the headaches had resolved and the CSF opening pressure was normal. Six months later, the patient is symptom-free, the papilloedema has resolved, MRI is normal, and the patient has returned to full-time work.
It is rare for WG to present with neurological symptoms. Neurological presentations described include ataxia, ocular nerve palsies, seizures, and deteriorating mental status.1–4 Shiotani et al described a 37 year old man with chronic sinusitis, who presented with fever and headache for 10 days before CT disclosed subdural and paranasal masses with marked thickening of the nasal mucosa.5 Our patient presented far more insidiously, with significant headache that persisted and worsened with time. The headache had clear vascular features but, beyond this, was non-specific. It was only four months later that musculoskeletal, cutaneous, ophthalmic, and cardiorespiratory features developed. Although neurological involvement may eventually develop in 33.6% of patients with WG,6 meningeal involvement, as gauged by meningeal enhancement on MRI or by biopsy is particularly uncommon, being recorded in only a handful of case reports.2–5,7–10 There also seems to be no relation between CSF abnormalities, clinical symptoms, or extent of meningeal involvement on MRI.7 A CSF examination may show no abnormality3,4,7 or a pleocytosis.3,5,8,9 High opening pressures are unusual but have been described.9,10
In conclusion, we have presented a case of WG with extensive meningeal involvement. The exceptional feature in this case is the fact that headache was the sole symptom of the disease over several months, before a dramatic activation of the disorder with more typical features of WG.
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