Chronic immune sensory polyradiculopathy with cranial and peripheral nerve involvement

Abstract

Chronic immune sensory polyradiculopathy (CISP) is a recently described restricted form of chronic inflammatory demyelinating polyradiculopathy (CIDP) [1]. It preferentially affects large myelinated fibres of the posterior roots, is associated with normal peripheral nerve conduction studies, and may respond to immunomodulatory treatment. We describe a patient with clinical features of CISP and additional cranial nerve involvement. A 61 year old woman presented with a 10 year relapsing-remitting neurological disorder. She initially presented with nausea, headache and photophobia followed by diplopia and unilateral ptosis consistent with an oculomotor nerve palsy. Intracranial CT, MRI, MRA, catheter angiography and CSF were all normal. Symptoms resolved over 18 months but an inflammatory cause was suspected after a relapse and treatment with 2 g/kg intravenous immunoglobulin (IVIg) was effective. Further relapses with additional poor balance, facial numbness and distal sensory symptoms responded promptly to IVIg. Intravenous methylprednisolone worsened her symptoms. Neurological examination revealed a partial right oculomotor nerve palsy. Pin prick sensation was reduced in a right trigeminal nerve distribution. Limb power was normal but reflexes were reduced in the upper limbs and absent in the lower limbs with flexor plantars. Romberg’s test was positive. Pin prick was reduced on the ulnar aspect of the right forearm and up to the mid-shins bilaterally. Vibration sense was reduced up to both ankles and proprioception impaired up to the right ankle. Examination after IVIg revealed that ophthalmoplegia, ptosis and a timed Romberg’s test had all improved. Systemic inflammatory/autoimmune blood tests were unremarkable and these included full blood count, ESR, CRP, ANA, ANCA, complement, rheumatoid factor, ENA, protein electrophoresis, immunofixation, acetylcholine receptor antibodies, immunoglobulins, ganglioside serology, lyme serology, treponemal serology and anti-neuronal antibodies. CSF was acellular with normal glucose and a raised protein level of 0.71 g/L (normal 0.15–0.65 g/L). Oligoclonal bands were absent. Neurophysiological data are presented in the appendix. In summary, the absolute values of all neurophysiological investigations listed were normal. However, the ulnar nerve SNAPs were significantly asymmetrical and the motor F-waves prolonged. Thermal thresholds were abnormal in the feet and right hand. Somatosensory evoked potentials were poorly formed in the right lower limb. Asymmetry of motor evoked potential latencies following cervical spine stimulation was consistent with a lesion distal to the intervertebral foramina. Electronic supplementary material The online version of this article (doi:10.1007/s00415-011-6326-0) contains supplementary material, which is available to authorized users.