Person:

Chwalisz, Bart

We present a case of a 60-year-old Caucasian woman with a 23-year history of rheumatoid arthritis, who presented with a 2-week history of headache and cognitive/behavioural changes. On the basis of clinical features, radiology, laboratory data and meningeal biopsy, a diagnosis of rheumatoid meningitis was performed. High-dose intravenous methylprednisolone was used as initial treatment followed by oral prednisone. The patient’s symptoms improved and repeat MRI scans confirmed resolution of the meningeal lesions. The clinical diagnosis of rheumatoid meningitis is difficult, but it must be considered in patients with long- standing rheumatoid arthritis presenting with neurological symptoms. Glucocorticoids or other immunomodulatory therapy are the mainstay of treatment.

The term small-fiber neuropathy (SFN) refers to the type of polyneuropathies that preferentially damage the small unmyelinated and thinly myelinated sensory or autonomic neurons [1]. Skin biopsy to determine the epidermal nerve-fiber density (ENFD) is the most de- finitive method in the diagnosis of SFN, but autonomic function testing can also be useful [2]. Importantly, electromyography and surface nerve-conduction studies (EMG/NCS) do not capture the small scattered action potentials of small fibers, and thus are insensitive to small-fiber restricted neuropathies. Quantitative sensory testing is a subjective test that depends on patient volition, so it is not recommend- ed or reimbursed for clinical use [3]. Most generalized polyneuropathies first affect the distal parts of the limbs, such as the feet. They only rarely present proximally and the diagnosis of a non-length dependent SFN is usually associated with symptoms beginning in the hands, face, or torso. Onset in the pelvic region is rare and the diagnosis can be missed. We report on two patients with an unexplained syndrome of somatic and visceral uro-genital sensory deficit with histologic evidence of SFN.