The dorsal root ganglion under attack: the acquired sensory ganglionopathies

  title={The dorsal root ganglion under attack: the acquired sensory ganglionopathies},
  author={Sarah I. Sheikh and Anthony A. Amato},
  journal={Practical Neurology},
  pages={326 - 334}
Acquired sensory ganglionopathies—or sensory neuronopathies—are a rare type of peripheral neuropathy characterised by damage to the sensory nerve cell bodies in the dorsal root ganglia. Subacute or chronic in onset, sensory ganglionopathies typically present with a non-length dependent pattern of large fibre sensory loss. The causes of this distinct clinical picture include paraneoplastic syndromes, immune mediated diseases, infections, as well as drug, toxin and excess vitamin exposure. Here… 

Sensory neuronopathies

The sensory neuronopathies (or ganglionopathies) are a small subcategory of neuropathies characterized by primary degeneration of the dorsal root ganglia and trigeminal ganglion sensory neurons,

Acute Sensory and Autonomic Neuronopathy: A Devastating Disorder Affecting Sensory and Autonomic Ganglia.

A systematic review of the epidemiology, pathophysiology, diagnosis, and management of ASANN, with three representative cases that recently presented at the clinic, illustrating the variable phenotype of the disorder.


A case in whom biopsy of the thoracic dorsal root ganglion resulted in identifying of continued inflammation despite normal haematological makers thereby altering subsequent management and demonstrates the important information that can be gained from DRG biopsy in those with clinical deterioration despite immunosuppressive treatment.

Neuroimaging in Sensory Neuronopathy

The aim of this review is to gather available information on neuroimaging findings of SN, discuss their clinical correlates and the potential impact of novel MRI‐based techniques.

Sensory Polyneuropathies.

Despite advances in diagnosing small fiber neuropathies and sensory neuronopathies, many of these Neuropathies remain refractory to treatment and should be considerediopathic.

Magnetic resonance imaging of the spinal cord in the evaluation of 3 patients with sensory neuronopathies: Diagnostic assessment, indications of treatment response, and impact of autoimmunity

In this case-series of three sensory neuronopathy patients, serial MRI studies revealed partial resolution of dorsal column lesions, associated with decreased sensory ataxia and improved nerve-conduction studies, which suggest that such dorsal Column lesions may reflect inflammatory as well as a gliotic burden of injury.

Sensory ganglionopathy associated with drug-induced hypersensitivity syndrome caused by mexiletine

A 70-year-old woman, previously administered mexiletine hydrochloride for 4 weeks, who presented with systemic oedematous erythema and subacute progressive gait disturbance is described, suggesting the possibility that reactivation of HHV-6 may be involved in the pathomechanism of sensory ganglionopathy.

Sensory neuronopathies: A case series and literature review

Sensory neuronopathies caused severe disability, especially in patients with generalised areflexia and pseudoathetosis, and with motor involvement and raised CSF protein by univariate analysis.

Paraneoplastic and Idiopathic Ganglionopathy: Importance of DifferentialDiagnosis

The identification of paraneoplastic antibodies before the onset of cancer can reduce the percentage of cases misdiagnosed as idiopathic, leading to early treatment and perhaps a more favorable prognosis.

Spinal cord lesion mimicking a dysimmune myelitis revealing CANVAS syndrome.

This is the first case of CANVAS syndrome presenting initially with an isolated spinal cord lesion mimicking dysimmune myelitis, and is to add to the current literature about this evolving neurological syndrome to aid clinicians in their diagnostic approach in clinical practice.



Sensory neuron diseases

Clinical features and pathophysiological basis of sensory neuronopathies (ganglionopathies)

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 and in SS sensory ganglionitis, response to treatment has been disappointing, but immunomodulating treatments are emerging.

Neurophysiological Diagnosis of Acquired Sensory Ganglionopathies

Investigation of chronic progressive ganglionopathy of different etiology found a widespread decrease in sensory nerve action potential amplitudes, which involved both upper and lower limb nerves, even in patients with asymmetrical or patchy clinical presentation.

Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndrome

The possibility of Sjögren's syndrome should be considered in patients, especially women, who develop acute, subacute, or chronic sensory and autonomic neuropathies, with ataxia and kinesthetic loss.

The pattern and diagnostic criteria of sensory neuronopathy: a case–control study

In the study population, sensory neuronopathy showed a common clinical and electrophysiological pattern that was independent of the underlying cause, including unusual forms with only patchy sensory loss, mild electrical motor nerve abnormalities and predominant small fibre or isolated lower limb involvement.

Causes and Diagnosis of Sensory Neuropathies: A Review

  • H. MitsumotoA. Wilbourn
  • Medicine, Psychology
    Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society
  • 1994
The electrophysiological features, laboratory findings, and nerve biopsy results in patients and discuss in detail the potential underlying diseases included in the differential diagnosis of sensory neuropathies.

Clinical and magnetic resonance imaging findings in chronic sensory ganglionopathies

Twenty‐two of 29 patients with chronic sensory ataxic neuropathy showed T2‐weighted magnetic resonance imaging high signal intensity in the posterior columns of the cervical spine. T2 changes

MRI Shows Dorsal Lesions and Spinal Cord Atrophy in Chronic Sensory Neuronopathies

  • M. FrançaA. D'abreu F. Cendes
  • Medicine, Psychology
    Journal of neuroimaging : official journal of the American Society of Neuroimaging
  • 2008
The frequency and extent of spinal cord MRI abnormalities in a group of patients with sensory neuronopathies is estimated and these are correlated with clinical and neurophysiological features.

The acute sensory neuronopathy syndrome: A distinct clinical entity

During follow‐up of five years, no evidence of neoplastic disease or immunological disorder has appeared, and three adults suddenly experienced numbness and pain over the face and entire body.