Dorsal root ganglionectomy for the diagnosis of sensory neuropathies. Surgical technique and results

Colli, Benedicto Oscar; Carlotti, Carlos Gilberto; Assirati, João Alberto; Lopes, Luíza da Silva; Marques, Wilson; Chimelli, Leila; Neder, Luciano; Barreira, Amilton Antunes

doi:10.1016/j.surneu.2007.01.057

Cited by 20 publications

(14 citation statements)

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“…The hallmarks of GNP -disproportionate sensory involvement and multifocal distribution of deficits -are often difficult to determine either by clinical or electrophysiological criteria. Sometimes, additional investigation such as spinal cord MRI, skin biopsy with epidermal nerve fiber density evaluation or even dorsal root ganglia biopsy is needed to reach the correct diagnosis 6,[12][13][14] . Although valuable, some of these tests are expensive, invasive and timeconsuming.…”

Section: Discussionmentioning

confidence: 99%

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

Garcia

Ricardo

Horta

et al. 2013

Arq. Neuro-Psiquiatr.

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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 . 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. Often it is not possible to define a clear pattern of symmetry or predominant distal involvement (either by clinical or electrophysiological criteria), making it difficult to distinguish a GNP from a sensory PNP. ABSTRACTThe objective of this study was to evaluate if the ratio of ulnar sensory nerve action potential (SNAP) over compound muscle action potential (CMAP) amplitudes (USMAR) would help in the distinction between ganglionopathy (GNP) and polyneuropathy (PNP). Methods: We reviewed the nerve conductions studies and electromyography (EMG) of 18 GNP patients, 33 diabetic PNP patients and 56 controls. GNP was defined by simultaneous nerve conduction studies (NCS) and magnetic resonance imaging (MRI) abnormalities. PNP was defined by usual clinical and NCS criteria. We used ANOVA with post-hoc Tukey test and ROC curve analysis to compare ulnar SNAP and CMAP, as well as USMAR in the groups. Results: Ulnar CMAP amplitudes were similar between GNP x PNP x Controls (p=0.253), but ulnar SNAP amplitudes (1.6±3.2 x 11.9±9.1 x 45.7±24.7) and USMAR values (0.3±0.3 x 1.5±0.9 x 4.6±2.2) were significantly different. A USMAR threshold of 0.71 was able to differentiate GNP and PNP (94.4% sensitivity and 90.9% specificity). Conclusions: USMAR is a practical and reliable tool for the differentiation between GNP and PNP.Key words: clinical neurophysiology, ganglionopathy, polyneuropathy, sensory neuronopathy, ulnar nerve. RESUMOO objetivo deste estudo foi avaliar se a razão entre as amplitudes dos potenciais de ação sensitivo (SNAP) e motor (CMAP) do nervo ulnar (USMAR) auxiliaria na distinção entre ganglionopatia (GNP) e polineuropatia (PNP). Métodos: Revisamos os estudos de neurocondução e eletromiografia de 18 pacientes com GNP, 33 com PNP diabética e 56 controles. GNP foi definida pela presença simultânea de anormalidades na neurocondução e na ressonância magnética cervical. PNP foi definida por critérios clínicos e neurofisiológicos usuais. Usamos o teste ANOVA com Tukey post-hoc e análise da curva ROC para comparar o SNAP e CMAP ulnares, assim como o USMAR entre os grupos. Resultados: As amplitudes dos CMAPs ulnares foram similares entre GNP x P...

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Section: Discussionmentioning

confidence: 99%

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

Garcia

Ricardo

Horta

et al. 2013

Arq. Neuro-Psiquiatr.

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“…Degeneration of DRG “T-shaped” neurons and their projections, both central and peripheral, often results in a multifocal pattern of sensory deficits [4, 5]. This is in contrast to the usual length-dependent pattern found in most polyneuropathies.…”

Section: Overview Of Sensory Neuronopathiesmentioning

confidence: 99%

“…This explains why DRG neurons are vulnerable to such distinct mechanisms of damage [9]. In immune-mediated SN, most available data support the concept of direct inflammatory damage to DRG neurons mediated by CD8 T lymphocytes [3, 5, 9, 14–16]. Humoral dysfunction seems to play a minor role in most forms of SN, but anti-GD1b antibodies were associated to SN in cell and animal-based models [17, 18].…”

Section: Overview Of Sensory Neuronopathiesmentioning

confidence: 99%

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Sensory Neuronopathy and Autoimmune Diseases

Martínez

Nunes

Nucci

et al. 2012

Autoimmune Diseases

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Sensory neuronopathies (SNs) are a specific subgroup of peripheral nervous system diseases characterized by primary degeneration of dorsal root ganglia and their projections. Multifocal sensory symptoms often associated to ataxia are the classical features of SN. Several different etiologies have been described for SNs, but immune-mediated damage plays a key role in most cases. SN may herald the onset of some systemic autoimmune diseases, which further emphasizes how important the recognition of SN is in clinical practice. We have thus reviewed available clinical, neurophysiological, and therapeutic data on autoimmune disease-related SN, namely, in patients with Sjögren's syndrome, autoimmune hepatitis, and celiac disease.

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“…DRG is invasive, however reports demonstrate high diagnostic yield and low post-operative complication rates when thoracic ganglions are biopsied. 2 Extensive investigations for alternative aetiologies were unyielding.…”

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confidence: 99%

Sensory Neuronopathy; A Case Report and a Review of the Role of Ganglion Nerve Biopsy in Diagnosis

Waddell

Farmer

Lunn

et al. 2013

J Neurol Neurosurg Psychiatry

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Dorsal root ganglionopathies present with sensory ataxia, areflexia and asymmetrical positive and negative sensory phenomenon of both limb often with trigeminal involvement. There may be associated Adie's pupil1 Despite their classical features and relatively discrete list of causes they can be difficult to definitively diagnose. We present such 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. A 54 year old telecommunications officer with a background of anterior uveitis presented with a 3 year history of progressive altered sensation of his right hand and a 2 month history of altered sensation of the right side of his face including oral mucosa. There was unilateral facial sensory loss to pin prick, right upper limb pseudoathetosis, hyperaesthesia of C5 to C8 and impaired proprioception to wrist. He was hyporeflexic throughout. He was on no regular medication, was a lifelong smoker, drank minimal alcohol and denied any family history. His immunology demonstrated a positive cANCA at 7 (0–5) and positive ENA (anti–La ). He had normal inflammatory markers, vitamins B6 and B12, homocysteine, methylmalonic acid, folate, serial antineuronal antibodies, HIV, angiotensin converting enzyme, phytanic acid, protein electrophoresis, chest X–ray, contrast MRI imaging of brain, cervical spine and brachial plexus. He had 3 large volume CSF analyses with normal cell count, protein, glucose ratio, and negative oligoclonal banding and cytology. His neurophysiology was positive for a possible right sided ganglionopathy. There was an associated parathyroid adenoma which was removed. Repeat CT of chest abdomen and pelvis demonstrated a pulmonary nodule with no change noted on serial images and follow up FDG–PET at 2 years was normal. He was initially treated with IV methylprednisolone and oral taper. He also received immunoglobulin. There was further clinical deterioration so was initiated. Despite these immunosuppressive therapies he continued to clinically deteriorate with distal mild weakness, areflexia and worsening proprioceptive deficits to his left wrist and MTP joints bilaterally. Inflammatory markers were persistently normal throughout. A radial nerve biopsy demonstrated axonal loss with no evidence of active inflammation. It was therefore unclear whether his ongoing deterioration was related to neurodegeneration or ongoing inflammation. A thoracic DRG biopsy was therefore undertaken. This demonstrated florid macrophage–mediated neuropathy with frequent myelin digestion. Due to evidence of an active inflammatory process he was treated with pulsed IV cyclophosphamide to a total of 10g with stabilisation of his limb signs. He is now maintained on Azathioprine. Our case demonstrates the important information that can be gained from DRG biopsy in those with clinical deterioration despite immunosuppressive treatment. DRG is invasive, however reports demonstrate high diagnostic yield and low pos...

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Dorsal root ganglionectomy for the diagnosis of sensory neuropathies. Surgical technique and results

Cited by 20 publications

References 16 publications

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

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

Sensory Neuronopathy and Autoimmune Diseases

Sensory Neuronopathy; A Case Report and a Review of the Role of Ganglion Nerve Biopsy in Diagnosis

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