Sensory ganglionopathy

Kyoko Saida MD (Dr. Saida of Kyoto Hakuaikai Hospital in Kyoto, Japan, has no relevant financial relationships to disclose.)
Raymond P Roos MD, editor. (Dr. Roos of the University of Chicago owns stock in Amgen, Best Doctors, Express Scripts, Isis, and Merck.)
Originally released May 22, 2001; last updated October 1, 2015; expires October 1, 2018

This article includes discussion of sensory ganglionopathy, sensory ataxic polyneuropathy, sensory ataxic ganglionopathy, ataxic polyneuropathy, ataxic neuronopathy, sensory ataxic neuronitis, sensory neuronopathy, sensory ganglionitis, acute sensory neuropathy, acute sensory neuronopathy syndrome, acute autonomic sensory neuropathy, sensory variant of Guillain-Barré syndrome, and non-length-dependent small fiber ganglionopathy. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Sensory ganglionitis, variably called ganglionopathy, is a disease of sensory neurons in dorsal root ganglia. Major forms of these diseases are associated with neoplasm, Sjögren syndrome, and paraproteinemia or polyclonal gammopathy with or without known autoantibodies. Most cases follow subacute courses, but there are forms that develop chronically and acutely as well. Clinical signs seen include sensory ataxia exhibited by gait unsteadiness, a positive Romberg sign, reduced deep tendon reflexes, poor coordination, and pseudo-athetoid movements in the hands. Axonal degeneration warrants the treatment as early as possible. Early cases of immunologic origin that are immune-mediated may respond to plasmapheresis and immunosuppression. Differential diagnoses include environmental and industrial intoxication and adverse effects of antineoplastic and antibiotic drugs. The term sensory neuronopathy or ganglionitis refers to disorders of small neurons, larger neurons, and/or neurons of both sizes in the sensory ganglia. This article will focus on the ganglionopathy involving larger neurons.

Key points

 

• Axonal degeneration in sensory ganglionitis warrants treatment as early as possible.

Historical note and terminology

In parenchymal peripheral nerve diseases, there are 2 major categories: (1) axonal and (2) neuronal neuropathy (Schroeder 1975). The former results from abnormalities in axons and is also called axonopathy; the latter, from abnormalities in neuronal cell bodies, and is also called neuronopathy (Spencer and Schaumburg 1976). As disease progresses, axonal neuropathy induces central chromatolysis of the neuronal cell body and neuronal neuropathy induces central-peripheral distal axonopathy of a dying back type (Spencer and Schaumburg 1976). In the peripheral nervous sensory system, sensory neurons are grouped in the dorsal root ganglia at the spinal level and gasserian (trigeminal) ganglia in the cranium. When inflammatory processes target the sensory ganglia, it is called sensory ganglionitis. Alternative descriptive terms are sensory (sensory ataxic or dorsal root) neuronitis or ganglioneuronitis. When it is uncertain if the disease is derived from inflammation, it is designated more vaguely as ganglionopathy or neuronopathy.

There are 4 major forms of sensory ganglionitis or ganglionopathy: (1) paraneoplastic sensory neuronopathy (Horwich et al 1977); (2) subacute sensory neuronopathy associated with Sjögren syndrome (Malinow et al 1986); (3) chronic ataxic neuropathy associated with paraproteinemia or polyclonal gammopathy with or without known autoantibodies (Dalakas 1986; Sobue et al 1988); and (4) acute sensory neuronopathy syndrome (Sterman et al 1980; Smith et al 1993), which is variably called acute sensory neuropathy (Windebank et al 1990) or acute autonomic sensory neuropathy (Colan et al 1980). Sensory ganglionitis occurs mostly in postinfectious conditions and resembles a sensory variant of Guillain-Barré syndrome (Asbury 1981; Dawson et al 1988).

A focal sensory ganglionitis may occur in viral or bacterial infections such as Herpes zoster (Gilden et al 2003), (possibly) HIV, and Borrelia burgdorferi. The inflammatory changes in vessels, Schwann cells, myelin, or the interstitium of the ganglia or nearby structures secondarily induce a sensory ganglionitis.

Table 1. Classification of Sensory (Autonomic) Neuronopathy or Ganglionopathy by Disease Onset

Inflammatory or idiopathic (probably autoimmune)

Acute

Acute sensory neuronopathy with or without ganglioside autoantibodies
Autoimmune autonomic ganglionopathy
Acute vestibular neuronitis or ganglionitis

Subacute

Carcinomatous neuronopathy
Sensory neuronopathy associated with Sjögren syndrome
Idiopathic sensory neuronopathy

Chronic

Paraproteinemic sensory neuronopathy
CANOMAD (chronic ataxic neuropathy with ophthalmoplegia, IgM paraprotein, cold agglutinins, and anti-GD1B (disialosyl) antibodies)
Idiopathic sensory neuronopathy
Autoimmune autonomic ganglionopathy

Toxic, metabolic, hereditary (gene mutations)

Acute

Toxic sensory neuronopathy

Subacute

Toxic sensory neuronopathy

   

Sensory neuronopathy associated with vitamin E deficiency
Sensory polyneuropathy after vitamin B6 overdose

Chronic

Toxic sensory neuronopathy
Hereditary sensory neuronopathy associated with various ataxias
Sensory neuronopathy associated with vitamin E deficiency

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