Table 1

Demographic features of the 159 patients with hypertrophic pachymeningitis

Basic demographicsInitial symptomsNumber (%)
 Number of cases analysed159 Headache56 (35.2)
 Sex ratio (male:female)1:0.91 Visual loss21 (13.2)
 Age at onset (mean±SD, years)58.3±15.8 Double vision20 (12.6)
 Age at first visit to physician (mean±SD, years)59.7±15.2 Otological symptoms15 (9.4)
 Disease duration (mean±SD, months)48.3±50.9Symptoms during entire courseNumber (%)
Underlying diseasesNumber Headache113 (71.1)
 ANCA-related angiitis21 Back pain6 (3.8)
 Wegener's granulomatosis19 Fever42 (26.4)
 Multifocal fibrosclerosis6* Consciousness disturbance21 (13.2)
 IgG4-related disease9 Convulsion14 (8.8)
 Otitis media3 Memory disturbance and other higher brain dysfunction11 (6.9)
 Cholesteatoma of the middle ear1 Visual loss52 (32.7)
 Sinusitis1 Double vision46 (28.9)
 Sjögren syndrome2 Dysphagia13 (8.2)
 Tuberculosis2 Dysarthria11 (6.9)
 Aspergillosis2 Weakness (facial and/or extremities)38 (23.9)
Site of dural hypertrophyNumber (%) Sensory disturbance45 (28.3)
 Cranial135 (84.9) Bladder disturbance10 (6.3)
 Spinal14 (8.8) Bowel disturbance7 (4.4)
 Cranial and spinal7 (4.4)Neurological findingsNumber (%)
Mode of onsetNumber (%) Cranial nerve palsy99 (62.3)
 Acute41 (25.8) Neck stiffness7 (4.4)
 Subacute75 (47.2) Motor impairment26 (16.4)
 Chronic27 (17.0) Abnormal tendon reflexes44 (27.7)
Clinical course Number (%) Pathological reflexes15 (9.4)
 Monophasic51 (32.1) Limb and/or truncal ataxia6 (3.8)
 Progressive28 (17.6) Decreased sensation31 (19.5)
 Relapsing-remitting62 (39.0) Sphincter dysfunction5 (3.1)
 Unknown18 (11.3)
  • *Includes five cases of MFS-related HP and one case assigned to ANCA-related HP.

  • ANCA, antineutrophil cytoplasmic antibody; HP, hypertrophic pachymeningitis; MFS, multifocal fibrosclerosis.