Retrospective Analysis Of The Similarities Between Tolosa-Hunt Syndrome And Hypertrophic Pachymeningitis

Objective: Both Tolosa-Hunt syndrome(THS) and hypertrophic pachymeningitis(HP) are very rare diseases due to a chronic non-specific granulomatous inflammation which commonly present with headache, cranial nerve palsies and are prone for recurrence. The aim of this retrospective study was to discuss whether there is essential difference between THS and HP.Methods: We conducted a search for the data of 186 inpatients with chief complaint of headache and ophthalmoplegia, or cerebral MRI showing pachymeningitis change in the department of Neurology and Ophthalmology of First Affiliated Hospital of Fujian Medical University from January 2005 to March 2014. A total of 9 HP inpatients and 12 THS inpatients have been confirmed finally after screening. The demographic information, clinincal findings, Laboratory findings, MRI findings and therapeutic efficacy between HP and THS groups were compaired respectively, and the the relationship and difference between HP and THS were analyzed.Result: THS and HP can affect people of virtually any age, with no sex predilection. The main clinical features at presentation of both groups were headache and cranial nerves paresis. The pain mostly located in peri-orbital, frontal and temporal regions. In THS patients, the most frequently involved cranial nerve was oculomotor nerve(66.67%), followed by abducens nerve(50.00%), trochlear nerve(25.00%), optic nerve(16.67%) and trigeminus nerve(8.33%). Cranial nerves paresis also was the common symptom in HP, occurring in approximately 88.89% of patients. The most frequently involved cranial nerve was oculomotor nerve(44.44%), trigeminus nerve(44.44%), abducens nerve(44.44%), followed by trochlear nerve(22.22%) and optic nerve(11.11%). There were no significant differences found between two groups in terms of cranial nerve involvement, CSF tests, laboratory findings including WBC, CRP and ESR, Neuroimaging showed abnormal dural enhancement in 2 of 12 THS patients, and cavernous sinus involvement in 7 HP patients. Follow-up MRI scaning showed marked improvement of the dural lesions in 2 HP patients whom clinical symptoms had recovered. Both two groups were well response to glucocorticoid treatment.Conclusion: The demographic information, clinincal findings, Laboratory findings and therapeutic efficacy were highly similar between THS and HP, which support the hypothesis that THS and HP, also probably including inflammatory orbital pseudotumor and orbit eosinophilic granuloma be variants of a common pathogenesis. More pathologic evidence is needed to be consciously collected in clinical practice for further research. THS patients with negative MRI findings is very common, ICHD-3 standard which required MRI finding(or pathologic finding) as necessary conditions, should set up an appropriate formulation for those MRI negative patients. We suggest that the similar disease entity including THS, HP, inflammatory orbital pseudotumor and orbit eosinophilic granuloma be a new unified classification, then ranking the diagnostic criteria and categorizing the diagnosis as definite, probable and possible THS, which may be helpful to clinicians whose task is to understand and master the essence of THS, and to treated it more timely.