Clinical features and pathophysiological basis of sensory neuronopathies (ganglionopathies)

Abstract

Sensory ganglionopathies have a frequent association with neoplastic disorders (paraneoplastic subacute sensory neuronopathy, or SSN) or dysimmune disorders (Sjögren's syndrome, SS; Miller Fisher syndrome; and Bickerstaff's brainstem encephalitis, BBE), with drugs, such as cisplatin or pyridoxine, and with inherited disorders with degeneration of dorsal root ganglion cells. Unsteady gait and pseudoathetoid movements of the hand are the distinctive signs encountered in these disorders. The chronic disorders are characterized by non-length-dependent abnormalities of sensory nerve action potentials (SNAPs) and differ from other sensory neuropathies in showing a global, rather than distal, decrease in SNAP amplitudes. This review focuses on recent advances in defining the mechanisms involved in sensory ganglionopathies. Specific topics include a summary of their clinical features, pathological findings, and immunopathology. In SSN, early diagnosis by the detection of anti-Hu antibodies and early treatment of the cancer gives the best chance of stabilizing the disorder. In SS sensory ganglionitis, response to treatment has been disappointing, but immunomodulating treatments are emerging. The immunological profile common to BBE and Fisher syndrome supports a common pathogenesis. In toxic sensory neuronopathy, no treatment is available. The differential diagnosis involves separating sensory ganglionopathies from other ataxic polyneuropathies, such as infectious neuropathies, sensory neuropathies with various autoantibodies, and the neuropathies seen in celiac disease.

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

“…It presents with impaired kinesthetic awareness and proprioception, and leads to severe sensory ataxia with pseudoathetotic hand movements due to involvement of large ganglionic neurons [92]. A small case series reports successful management with IVIg (grade 3D) [93], while treatment with rituximab, plasmapheresis, infliximab, and interferon (IFN)-α were encouraging in other series (grade 3D) [94][95][96][97].…”

Ulnar sensory-motor amplitude ratio: a new tool to differentiate ganglionopathy from polyneuropathy

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Ganglionopathies (GNP), also known as sensory neuronopathies, are a group of conditions characterized by primary and selective damage to the dorsal root ganglia (DRG) of the spinal cord and sensory nuclei of the brainstem 1,2 . The etiologies are diverse and include immune-mediated diseases, vitamin deficiencies, drug toxicity, paraneoplastic syndromes and genetic causes, but many patients are yet defined as idiopathic 1,2 .

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“…The etiologies are diverse and include immune-mediated diseases, vitamin deficiencies, drug toxicity, paraneoplastic syndromes and genetic causes, but many patients are yet defined as idiopathic 1,2 . The clinical presentation is characterized by diffuse, often asymmetric, sensory deficits and marked ataxia due to loss of proprioception 1,2 .…”

“…The etiologies are diverse and include immune-mediated diseases, vitamin deficiencies, drug toxicity, paraneoplastic syndromes and genetic causes, but many patients are yet defined as idiopathic 1,2 . The clinical presentation is characterized by diffuse, often asymmetric, sensory deficits and marked ataxia due to loss of proprioception 1,2 .In neurological practice, it is important to differentiate GNP from polyneuropathies (PNP) because the etiologies, therapeutic strategies and prognosis are often diverse 3 . Clinically, GNP can be distinguished from PNP due to a purely sensory dysfunction and the absence of length-dependent gradient of involvement.…”

Neuropatia paraneoplásica associada ao mastocitoma canino

“…BRAUND et al (1987) demonstraram a presença de lesões degenerativas em nervos periféricos de 59% dos cães com carcinoma broncogênico, 59% com adenocarcinoma mamário, 48% com melanoma, 47% com insulinoma, 39% com osteossarcoma e 32% com mastocitoma. As principais alterações observadas nas fibras neuronais são desmielinização, vasculite e degeneração axonal (KUNTZER et al, 2004;BERGMAN, 2007).…”