Neurophysiological Diagnosis of Acquired Sensory Ganglionopathies

Lauria, Giuseppe; Pareyson, Davide; Sghirlanzoni, Angelo

doi:10.1159/000073055

Cited by 47 publications

(47 citation statements)

References 18 publications

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“…In case 1 (paraneoplastic SG), for example, reduced amplitudes in the motor nerves of the lower limbs were found. This is in line with Lauria et al (2003), who showed abnormalities in motor nerve conduction, which were most frequent in the lower limbs in paraneoplastic cases.…”

Section: Discussionsupporting

confidence: 89%

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Paraneoplastic and Idiopathic Ganglionopathy: Importance of Differential Diagnosis

Gasque¹,

Schmidt²,

Carvalho³

2015

J Neurol Neurophysiol

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Sensory ganglionopathies (SG) may occur in association with different diseases and are characterized by the degeneration of the primary sensory neurons located in the dorsal root ganglia. The most important associated conditions are paraneoplastic SG, HIV infection, Sjogren's syndrome, and cisplatin or pyridoxine toxicity. Even when clinical criteria help differentiate SG from other neuropathies, an etiological diagnosis remains elusive. Thus, there is a need to identify biological markers than can distinguish immune-mediated SG from other etiologies. Objectives: The present study of two cases of SG compares their neurological outcomes with their treatment responses in accordance with the etiology of each one. Methods: evaluate the clinical presentation and examination results to be able to define the etiology and institute early treatment. Results: To differentiate paraneoplastic and idiopathic causes, more precise imaging techniques and paraneoplastic antibody tests are required. These diagnostic tools might be helpful in early diagnosis and treatment, thus affording the best chance of stabilizing the neurological symptoms. Conclusions: 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.

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Section: Discussionsupporting

confidence: 89%

“…Nerve conduction studies are the most useful tests in evaluating SG [3,5]. The distinguishing characteristics between SG and other sensory neuropathies are important to properly focus the etiological investigation and the adoption of appropriate treatment [2].…”

Section: Introductionmentioning

confidence: 99%

Paraneoplastic and Idiopathic Ganglionopathy: Importance of Differential Diagnosis

Gasque¹,

Schmidt²,

Carvalho³

2015

J Neurol Neurophysiol

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“…8 We believe the second patient also had SND despite the simultaneous abnormalities found in sensory and motor NCS. In this individual, sensory impairment, manifested by complete absence of SNAPs, diffuse areflexia, and disabling sensory ataxia, would be better explained by damage to DRGs than by an axonopathy.…”

Section: Discussionmentioning

confidence: 76%

Myositis with sensory neuronopathy

et al. 2007

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Inflammatory myopathies (IM) are a heterogeneous group of diseases characterized by immune-mediated damage to skeletal muscle. Sensory abnormalities are rare in patients with IM. We report two patients, one with dermatomyositis and the other with inclusion-body myositis, who presented with unexpected sensory abnormalities due to probable immune-mediated damage to dorsal root ganglia. We emphasize the importance of combined neuroimaging and neurophysiological assessment for proper diagnosis.

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“…Impairment of DRG large neurons causes gait ataxia, proprioceptive sensory loss and widespread muscle stretch areflexia 1,9,10 . In many patients, however, there is a disproportionate gait and truncal ataxia in comparison to limb incoordination, which is also remarkable with preserved visual input, resembling a cerebellar dysfunction.…”

Section: Discussionmentioning

confidence: 99%

Cerebellar atrophy is frequently associated with non-paraneoplastic sensory neuronopathy

Damasceno

França

Cendes

et al. 2011

Arq. Neuro-Psiquiatr.

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Sensory neuronopathies (SN) are peripheral nervous system disorders associated with degeneration of dorsal root ganglion neurons. Despite the evidence of a defective proprioceptive sensory input in SN, the prominent gait and truncal ataxia raises the question of a concomitant involvement of the cerebellum. Objective: To evaluate cerebellar atrophy in SN. Method: We analyzed MRI-based volumetry of anterior lobe (paleocerebellum) and total cerebellum in patients with non-paraneoplastic chronic SN and compared to age-and gender-matched controls. Results: Cerebellum and anterior lobe MRI volumetry were performed in 20 patients and nine controls. Mean anterior lobe and cerebellar volume were not statistically different. Three patients (15%), however, had an abnormal anterior lobe and cerebellar volume index (values outside 2.5 standard deviations). One of them also had a specific atrophy of the anterior lobe. All these patients had infectious or dysimmune associated SN. Conclusion: Cerebellar atrophy is infrequently associated with SN, but can be found in some patients with SN related to infectious or immune mediated conditions. It can be more prominent in the anterior lobe and may contribute to the ataxia seen in these patients. Key words: cerebellar atrophy, cerebellar volume reduction, magnetic resonance imaging, sensory neuron disease, sensory neuronopathy Atrofia cerebelar não é um achado frequente em pacientes com neuronopatia sensi tiva não paraneoplasica RESUMO Neuronopatias sensitivas (NS) são distúrbios do sistema nervoso periférico associados com a degeneração dos neurônios do gânglio da raiz dorsal. Apesar da evidência de um defeito das aferências proprioceptivas, a ataxia proeminente da marcha e do tronco levanta a questão de uma participação concomitante do cerebelo. Objetivo: Avaliar a atrofia cerebelar nas NS. Método: Foi analisada volumetria pela ressonância magnética do cerebelo total e do lobo anterior (paleocerebelo) em pacientes com NS crônica não-paraneoplásica e comparada a controles com idades e sexos correspondentes. Resultados: A volumetria do cerebelo e lobo anterior foi realizada em 20 pacientes e nove controles. As médias do volume cerebelar e do lobo anterior não foram estatisticamente diferentes. Três pacientes, entretanto, tiveram um valor anormal do índice de volume cerebelar e do lobo anterior (valores fora de 2,5 desvios-padrão). Um deles tinha inclusive uma atrofia específica do lobo anterior. Todos esses pacientes tinham NS associada a doenças infecciosas ou disimunes. Conclusão: Atrofia cerebelar é raramente associada com SN, mas pode ser encontrada em alguns pacientes com NS relacionada com doenças infecciosas ou imunológicas. Esta atrofia pode ser mais proeminente no lobo anterior e pode contribuir para a ataxia observada nestes pacientes.

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Neurophysiological Diagnosis of Acquired Sensory Ganglionopathies

Cited by 47 publications

References 18 publications

Paraneoplastic and Idiopathic Ganglionopathy: Importance of Differential Diagnosis

Paraneoplastic and Idiopathic Ganglionopathy: Importance of Differential Diagnosis

Myositis with sensory neuronopathy

Cerebellar atrophy is frequently associated with non-paraneoplastic sensory neuronopathy

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