Sensory Ganglioionitis

[issie]

I thought this may be of interest to others since there is seeming to be so many of us finding connections with autoimmune issues and inflammation. Notice how this can show up after pregnancies and with other traumas, but is found equally in women and men. It was recently found that my dad has this. Makes me wonder if there is not a genetic component to it. My dad's neuropathy is so bad and he was like his dad and I'm more like my dad. My neuropathy seems lots better since I'm addressing my autoimmune system.

Issie

http://www.medlink.com/medlinkcontent.asp (I couldn't get this link to work - so here is some of the copied version of it. If you can get the full article it's very interesting.)

Sensory ganglionitis

Contributors

Kyoko Saida MD, author. 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 and Merck.

Publication dates

Originally released May 22, 2001; last updated June 13, 2012; expires June 13, 2015

Key points

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

Historical note and nomenclature

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: (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). Chronic immune sensory polyradiculopathy of unknown origin has been reported (Sinnreich et al 2004) in which demyelination of spinal roots is noticed.

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

Acute sensory autonomic neuronopathy

Autoimmune autonomic ganglionopathy

Subacute

Carcinomatous neuronopathy

Sensory neuronopathy associated with Sjögren syndrome

Idiopathic sensory neuronopathy

Chronic

Paraproteinemic sensory neuronopathy

Idiopathic sensory neuronopathy

Autoimmune autonomic ganglionopathy

Toxic, metabolic, hereditary

Acute

Toxic sensory neuronopathy

Subacute

Toxic sensory neuronopathy

Sensory neuronopathy associated with vitamin E deficiency

Chronic

Toxic sensory neuronopathy

Hereditary sensory neuronopathy associated with various ataxias

Sensory neuronopathy associated with vitamin E deficiency

Clinical manifestations

In general, the key symptoms of sensory ganglionitis are a decrease or loss of kinesthetic and proprioceptive sensation. When large diameter ganglionic neurons are decreased or lost, the clinical signs seen include sensory ataxia exhibited by gait unsteadiness (“stamp and stick” type of walk), positive Romberg sign, reduced or absent deep tendon reflexes, poor coordination, and pseudo-athetoid movements in the hands with fingers assuming hyperextended positions (Smith et al 1993). When small-diameter neurons are affected as a non-length-dependent small fiber gangliopathy, symmetric or asymmetric sensory symptoms such as paresthesias, numbness, shooting pains, or burning dysesthesias of the feet and hands occur, often in a segmental fashion (hyperalgesic type) (Gorson et al 2008). When ganglionic neurons with short segmental roots or nerves are affected, symptoms proceed from distal to proximal parts of the body and occasionally involve the face and trunk. Although an ataxic picture is most commonly seen, both manifestations of the syndrome can be variably mixed depending on the pathophysiology.

The symptoms of sensory ganglionitis are exclusively sensory by definition. However, in many instances of both carcinoma and paraproteinemia, motor weakness and muscle atrophy may appear during the course of disease. Less frequently, autonomic symptoms such as Adie pupils, loss of sinus arrhythmia, orthostatic hypotension, sudden hypertension, or segmental loss of sweating may appear. Demyelinative changes are not observed either in electrophysiological or pathological studies. Although the disease may remit when associated with Sjögren syndrome, it is almost always progressive; recovery is poor in cases related to paraproteinemia and toxic exposures.

Sensory ganglionitis associated with carcinoma typically develops in middle-aged or older men and less frequently in women (Graus et al 2001). It has a subacute onset with hyperalgesic features and progresses in a few months. This type constitutes about 10% of paraneoplastic neuropathies, and most cases also have a sensory ataxia as well. It may rarely involve the trunk and face, possibly appearing in the face as the "numb-chin syndrome." In some cases, nystagmus, diplopia, memory loss, and dementia may accompany the disease as a consequence of paraneoplastic encephalomyelitis. The onset of neuropathy may often precede symptoms and signs of the primary cancer. Small-cell lung cancer is associated with most cases (Horwich et al 1977; McLeod 1993) of paraneoplastic sensory ganglionitis.

Sensory ganglionitis associated with Sjögren syndrome (Mori et al 2005) mostly occurs in middle-aged women. It varies in onset from acute to indolent, progresses rapidly, and is usually a mixed type featuring numbness and sensory ataxia. The trigeminal nerve may be affected, manifesting as perioral numbness (Griffin et al 1990). In severe cases, the entire body may be numb.

Sensory ganglionitis associated with paraproteinemia is usually seen in older males. It has an indolent onset in months, progresses slowly, and is usually ataxic in type. Cases with IgM paraproteinemia can be hyperalgesic. Patients often become wheelchair-bound after several years. There are also similar cases with polyclonal gammopathy. A coarse action tremor of hands may be present (Sobue et al 1988).

Acute sensory ganglionitis or neuronopathy syndrome usually develops with rapidly progressing sensory ataxia and widespread sensory loss either as a postinfectious complication or following certain events such as child delivery. Both women and men aged between 30 to 70 years are typically affected. Symptoms stabilize in most cases but progress in some; however, improvement of symptoms is usually poor (Windebank et al 1990).

Chronic immune sensory polyradiculopathy is a newly recognized condition in which patients develop an idiopathic chronic progressive sensory ataxia of the limbs. There is usually normal sensory nerve conduction and normal action potentials in the distal axons, but the sensory-evoked potentials and nerve root biopsy reveal abnormalities around the nerve roots (Sinnreich et al 2004).

Inflammatory neuropathies of the enteric nervous system have been described. These are autonomic and, therefore, motor syndromes. This enteric ganglionitis is infectious or paraneoplastic in origin and produces acutely or slowly progressive segmental symptoms such as achalasia, gastroparesis, and pseudo-obstruction (De Giorgio et al 2004). There is also an autoimmune autonomic ganglionopathy in which IgG antibodies against the ganglionic acetylcholine receptors are found (Vernino et al 2008).

[HopeSprings]

The pathology report from my first skin biopsy says "similar pattern of denervation can be seen in ganglionopathy." I'm not sure what that means - I think it refers the nerve root or something. Too foggy too think about this now, but saw the word "ganglionitis" and remembered seeing the word in my medical records.

[Guest Alex]

Issie,

thanks for posting this. It definitely looks interesting.

Alex

[issie]

It made me think about the antibodies that others are having with AAG and AChr. Wonder if there is a connection between/with these. I'm hoping that my dad's doctor will do some more testing and maybe figure some more out about this.

Issie

[Natops]

Thanks for posting Issie. This is the first piece of literature that I have seen that associates my paraneoplastic syndrome with the sensory neuropathy that is plaguing me.