Early Aggressive Immunotherapy Improves Functional Outcome in Chronic Immune Sensory Polyradiculopathy

Abstract

Chronic immune sensory polyradiculopathy (CISP) is an uncommon and treatable inflammatory disorder of the proximal sensory nerve roots. Patients typically present with severe sensory ataxia, normal motor examination, unsteady gait, and normal nerve conduction studies (NCS). We describe an elderly man who presented with a two-week history of progressive numbness of both legs and recurrent falls. He had hyporeflexia, normal strength, severe proprioceptive, and vibration sense loss in both lower limbs and was unable to stand or walk because of severe sensory ataxia. e NCS and MR scan of the spine were normal. Tibial somatosensory evoked potentials revealed proximal conduction defect and localized the pathology to the lumbar sensory nerve roots proximal to the dorsal root ganglion. Cerebrospinal fluid showed cytoalbuminergic dissociation suggestive of inflammation. CISP was diagnosed; he was given aggressive immunotherapy consisting sequentially of corticosteroids with mycophenolate mofetil and three cycles of intravenous immunoglobulin after which he regained independent mobility. Unlike previous reports where patients presented months-years after symptom onset and improved after single-line immunotherapy, our patient presented fairly acutely and made dramatic improvement only after aggressive combination therapy. We urge physicians to recognize this uncommon neurologic cause of sensory ataxia where early aggressive treatment is crucial for better functional outcomes.

Chronic immune polyradiculopathies: Three clinical variants of one disease?

“…Subsequently, cranial neuropathies, 4 use of SSEP in monitoring therapy, 10 and response to intravenous immunoglobulin (IVIg) 8 and to aggressive immunotherapy 11 have been documented in CISP reports.…”

“…Five available MRIs showed thickening of rootlets and patients responded to immunotherapy. Subsequently, cranial neuropathies, 4 use of SSEP in monitoring therapy, 10 and response to intravenous immunoglobulin (IVIg) 8 and to aggressive immunotherapy 11 have been documented in CISP reports. In contrast, Rajabally and Wong 9 described an unusual patient who initially deteriorated with corticosteroids and later improved with IVIg, adding a note of caution regarding use of corticosteroids.…”

“…Involvement limited to proximal segments can be largely sensory (chronic immune sensory polyradiculopathy, or CISP), 1 motor (chronic immune motor polyradiculopathy, or CIMP), 2 or mixed (chronic immune sensory motor polyradiculopathy, or CISMP). 3 A few case reports [3][4][5][6][7][8][9][10][11] and only one case series each of CISP 1 and CISMP 12 have been published. This study was undertaken to identify differences and similarities between the clinical, electrophysiological, and radiological features and therapeutic responses of all three types.…”