Spinal cord diffusion tensor imaging in patients with sensory neuronopathy

Casseb, Raphael Fernandes; Paiva, Jean Levi Ribeiro de; Branco, Lucas M. T.; Martinez, Alberto Rolim Muro; Reis, Fabiano; Lima-Júnior, José Carlos de; Castellano, Gabriela; França, Marcondes Cavalcante

doi:10.1007/s00234-016-1738-2

Cited by 10 publications

(7 citation statements)

References 11 publications

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“…This neuroimaging signature reflects damage resulting from dorsal root ganglia (DRG) degeneration. Indeed, these radiological changes are very similar to those reported in other disorders characterized by extensive DRG damage, either inherited (eg, Friedreich's ataxia) or acquired (immune‐mediated sensory neuronopathies) 10,25 …”

Section: Discussionsupporting

confidence: 79%

RFC1‐Related Disorder: In Vivo Evaluation of Spinal Cord Damage

et al. 2022

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A BS TRACT: Background: RFC1-related disorder is a novel heredodegenerative condition with a broad phenotypic spectrum. Its neuropathological bases are not yet fully understood, particularly regarding the pattern, extent, and clinical relevance of spinal cord (SC) damage. Objectives: The objectives were to determine the SC structural signature in RFC1-related disorder in vivo and to identify potential clinical correlates for these imaging abnormalities. Methods: We enrolled 17 subjects with biallelic RFC1 (AAGGG)n expansions and 11 age-and sex-matched healthy controls that underwent multimodal magnetic resonance imaging SC acquisitions in a 3T Philips Achieva scanner. Both global morphometry and tractspecific analyses were then performed across all cervical levels. Between-group comparisons were assessed using nonparametric tests.Results: In the patient group, mean age and disease duration were 62.9 AE 9.3 and 9.3 AE 4.0, respectively. Compared to controls, patients had remarkable SC cross-sectional area reduction along all cervical levels but anteroposterior flattening only in the lower cervical levels. There was also prominent SC gray matter atrophy. Diffusivity abnormalities were identified in the dorsal columns but not in the lateral corticospinal tracts. Disease severity did not correlate with these imaging parameters. Conclusion: SC damage is a hallmark of RFC1-related disorder and characterized by gray as well as white matter involvement. In particular, dorsal columns are severely and diffusely affected. The clinical correlates of these imaging abnormalities still deserve additional investigations.

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Section: Discussionsupporting

confidence: 79%

RFC1‐Related Disorder: In Vivo Evaluation of Spinal Cord Damage

et al. 2022

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“…Whereas Camdessanche reported that 5% of sensory neuronopathy patients had abnormal spinal-cord MRI studies, [ 37 ] most other studies reporting on more than 10 patients have reported that 50% to 85% of sensory neuronopathy patients have abnormal spinal-cord MRI studies. [ 15 – 18 , 38 – 40 ] Therefore, our findings reaffirm how the multidisciplinary group of physicians who may encounter sensory neuronopathies can recognize and utilize spinal-cord MRI as a valuable adjunctive marker. Furthermore, our study describes how spinal-cord MRI can be used as a longitudinal indicator of treatment efficacy.…”

Section: Discussionsupporting

confidence: 69%

Magnetic resonance imaging of the spinal cord in the evaluation of 3 patients with sensory neuronopathies

Birnbaum¹,

Lalji

Piccione

et al. 2017

Medicine

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Rationale:Sensory neuronopathy can be a devastating peripheral nervous system disorder. Profound loss in joint position is associated with sensory ataxia, and reflects degeneration of large-sized dorsal root ganglia. Prompt recognition of sensory neuronopathies may constitute a therapeutic window to intervene before there are irreversible deficits. However, nerve-conduction studies may be unrevealing early in the disease course. In such cases, the appearance of dorsal column lesions on spinal-cord MRI can help in the diagnosis. However, most studies have not defined whether such dorsal column lesions may occur within earlier as well as chronic stages of sensory neuronopathies, and whether serial MRI studies can be used to help assess treatment efficacy. In this case-series of three sensory neuronopathy patients, we report clinical characteristics, immunological markers, nerve-conduction and skin-biopsy studies, and neuroimaging features.Patient concerns:All three patients presented with characteristic features of sensory neuronopathy with abnormal spinal-cord MRI studies. Radiographic findings included non-enhancing lesions in the dorsal columns that were longitudinally extensive (spanning ≥ 3 vertebral segments).Diagnoses:All patients had anti-Ro/SS-A and/or anti-La/SS-B antibodies, with patients one and two having Sjögren's syndrome. MRI findings were similar when performed in the earlier stages of a sensory neuronopathy (patient one, after four months) and chronic stages (patients two and three, after five and three years, respectively).Interventions:Patient one was treated with rituximab combined with intravenous immunoglobulin therapy.Outcomes:Patient one was initially wheelchair-bound and had improved ambulation after treatment. In this patient, serial MRI studies revealed partial resolution of dorsal column lesions, associated with decreased sensory ataxia and improved nerve-conduction studies.Lessons:In addition to vitamin B12 and copper deficiency, it is important to include sensory neuronopathies in the differential diagnosis of dorsal column lesions. MRI spinal-cord lesions have similar appearances in the earlier as well as chronic phases of a sensory neuronopathy, and therefore suggest that such dorsal column lesions may reflect inflammatory as well as a gliotic burden of injury. MRI may also be a useful longitudinal indicator of treatment response.

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“…Neuroimaging of the cortical and subcortical regions in patients with painful neuropathies have identified alterations in activity and functional connectivity that correlate with the subjects' neuropathic pain characteristics and treatment, including in patients with low back pain [32], PHN [117], diabetic polyneuropathy [118], neuroma pain [119], phantom limb [120], and CRPS [121,122]. Additionally, structures in the mesencephalic reticular formation (including possibly the PAG and nucleus cuneiformis) that, in preclinical studies, have been shown to be essential to mechanical allodynia after peripheral nerve injury, demonstrate increased neuronal activity on functional neuroimaging in a human capsaicin-evoked secondary hyperalgesia model [123].…”

Section: Evidence For Central Mechanisms: Clinicalmentioning

confidence: 99%

Neuropathic Pain: Central vs. Peripheral Mechanisms

Meacham

Shepherd

Mohapatra

et al. 2017

Curr Pain Headache Rep

333

203

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Recent preclinical studies in pNP support and provide key details concerning the role of multiple mechanisms leading to fiber hyperexcitability and sustained electrical discharge to the CNS. In studies regarding central mechanisms, new preclinical evidence includes the mapping of novel inhibitory circuitry and identification of the molecular basis of microglia-neuron crosstalk. Recent clinical evidence demonstrates the essential role of peripheral mechanisms, mostly via studies that block the initially damaged peripheral circuitry. Clinical central mechanism studies use imaging to identify potentially self-sustaining infra-slow CNS oscillatory activity that may be unique to pNP patients. While new preclinical evidence supports and expands upon the key role of central mechanisms in neuropathic pain, clinical evidence for an autonomous central mechanism remains relatively limited. Recent findings from both preclinical and clinical studies recapitulate the critical contribution of peripheral input to maintenance of neuropathic pain. Further clinical investigations on the possibility of standalone central contributions to pNP may be assisted by a reconsideration of the agreed terms or criteria for diagnosing the presence of central sensitization in humans.

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Spinal cord diffusion tensor imaging in patients with sensory neuronopathy

Abstract: DTI-based analysis enables in vivo detection of posterior column damage in sensory neuronopathy patients and is a useful diagnostic test for this condition. It also helps the differential diagnosis between sensory neuronopathy and distal polyneuropathies.

Cited by 10 publications

References 11 publications

RFC1‐Related Disorder: In Vivo Evaluation of Spinal Cord Damage

RFC1‐Related Disorder: In Vivo Evaluation of Spinal Cord Damage

Magnetic resonance imaging of the spinal cord in the evaluation of 3 patients with sensory neuronopathies

Neuropathic Pain: Central vs. Peripheral Mechanisms

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