Magnetic resonance imaging of dorsal root ganglion in a pre-symptomatic subject with familial amyloid polyneuropathy transthyretin E61K.

We recently reported evidence of transthyretin (TTR) familial amyloid polyneuropathy (TTR FAP) associated with TTR E61K, which is characterized by late-onset sensory dominant polyneuropathy, autonomic disturbances, and cardiomyopathy. In those TTR FAP patients, no amyloid deposits were observed in the endoneurium of examined sural nerves. Furthermore, the amyloidogenicity of E61K TTR was similar to that of wild-type TTR in vitro. Thus, we speculated that dorsal root ganglia (DRGs) may be the initial sites for the lesions in amyloid neuropathy because there is no blood-nerve barrier. In the present study, lumbar magnetic resonance imaging was performed to evaluate the DRGs in pre-symptomatic TTR E61K and V30M subjects. Magnetic resonance imaging (3 T) was used for three-dimensional T2-weighted imaging (coronal sections; slice thickness, 2 mm), and the DRG volumes were measured. The mean volumes of the bilateral L3, L4, and S1 DRGs in the pre-symptomatic TTR E61K subject were larger than those for the pre-symptomatic TTR V30M subject and five control patients. The mean volumes of the bilateral L4 to S1 DRGs in the pre-symptomatic TTR V30M subject were similar to those in control patients. A number of lumbar DRGs were enlarged in the pre-asymptomatic TTR E61K subject, suggesting that DRGs may be the sites of the initial lesions in the peripheral nervous system of this FAP.

Dorsal Root Ganglion Morphometric Changes Under Oxaliplatin Treatment : Longitudinal Assessment by Computed Tomography.

PURPOSE: Magnetic resonance neurography (MRN) can detect dorsal root ganglia (DRG) hypertrophy in patients with oxaliplatin-induced peripheral neuropathy (OXIPN) but is difficult to apply in clinical daily practice. Aims of this study were (i) to assess whether DRG volume is reliably measurable by routine computed tomography (CT) scans, (ii) to measure longitudinal changes in DRG during and after oxaliplatin administration and (iii) to assess correlation between DRG morphometry and individual oxaliplatin dose. METHODS: For comparison of MRN and CT measurements, CT scans of 18 patients from a previous MRN study were analyzed. For longitudinal assessment of DRG size under treatment, 96 patients treated with oxaliplatin between January and December 2014 were enrolled retrospectively. DRG volumetry was performed by analyzing routine CT scans, starting with the last scan before oxaliplatin exposure (t0) and up to four consecutive timepoints after initiation of oxaliplatin therapy (t1-t4) with the following median and ranges in months: 3.1 (0.4-4.9), 6.2 (5.3-7.8), 10.4 (8.2-11.9), and 18.4 (12.8-49.8). RESULTS: DRG volume measured in CT showed a moderately strong correlation with MRN (r = 0.51, p < 0.001) and a strong correlation between two consecutive CTs (r = 0.77, p < 0.001). DRG volume increased after oxaliplatin administration with a maximum at timepoint t2. Higher cumulative oxaliplatin exposure was associated with significantly higher absolute DRG volumes (p = 0.005). Treatment discontinuation was associated with a nonsignificant trend towards lower relative DRG volume changes (p = 0.08). CONCLUSION: CT is a reliable method for continuous DRG morphometry; however, since no standardized assessment of OXIPN was performed in this retrospective study, correlations between DRG size, cumulative oxaliplatin dose and clinical symptoms in future prospective studies are needed to establish DRG size as a potential OXIPN biomarker.

Long-term Follow-up and Histological Correlation of Peripheral Nervous System Alterations in Neurofibromatosis Type 2.

PURPOSE: To examine long-term alterations of the dorsal root ganglia (DRG) and the peripheral nerve in patients with neurofibromatosis type 2 (NF2) by in vivo high-resolution magnetic resonance neurography (MRN) and their correlation to histology. METHODS: In this prospective study the lumbosacral DRG, the right sciatic, tibial, and peroneal nerves were examined in 6 patients diagnosed with NF2 and associated polyneuropathy (PNP) by a standardized MRN protocol at 3 T. Volumes of DRG L3-S2 as well as peripheral nerve lesions were assessed and compared to follow-up examinations after 14-100 months. In one patient, imaging findings were further correlated to histology. RESULTS: Follow-up MRN examination showed a non-significant increase of volume for the DRG L3: +0.41% (p = 0.10), L4: +22.41% (p = 0.23), L5: +3.38% (p = 0.09), S1: +10.63% (p = 0.05) and S2: +1.17% (p = 0.57). Likewise, peripheral nerve lesions were not significantly increased regarding size (2.18 mm2 vs. 2.15 mm2, p = 0.89) and number (9.00 vs. 9.33, p = 0.36). Histological analyses identified schwannomas as the major correlate of both DRG hyperplasia and peripheral nerve lesions. For peripheral nerve microlesions additionally clusters of onion-bulb formations were identified. CONCLUSION: Peripheral nervous system alterations seem to be constant or show only a minor increase in adult NF2. Thus, symptoms of PNP may not primarily attributed to the initial schwannoma growth but to secondary long-term processes, with symptoms only occurring if a certain threshold is exceeded. Histology identified grouped areas of Schwann cell proliferations as the correlate of DRG hyperplasia, while for peripheral nerve lesions different patterns could be found.

Magnetic Resonance Neurography for Evaluation of Dorsal Root Ganglion Morphology.

BACKGROUND: In this study, the morphologic characteristics and anatomic position of the dorsal root ganglion (DRG) were measured and analyzed in healthy people using magnetic resonance neurography (MRN), which provided an anatomical reference for minimally invasive spinal surgery. METHODS: From January 2018 to December 2019, 20 healthy adult volunteers (10 male and 10 female volunteers between 20 and 65 years old) were scanned and imaged by 3.0 T magnetic resonance imaging combined with neuroimaging technology. Here, the position of the DRG was located, and the shape and size of the DRG, as well as its distance to the upper pedicle, were measured. RESULTS: All volunteers provided satisfactory MRN scans of the L1-S1 lumbar DRG. According to the spatial position of the DRG, the morphology of the DRG can be divided into the intervertebral foramen type (81.01%), intraspinal type (16.01%), extraforaminal type (0.8%), and mixed type (2.0%). CONCLUSIONS: The intervertebral foramen type and Intraspinal type were observed to be the main distribution forms of lumbar DRG. Due to the downward movement of lumbar segments, the position of the DRG was noted to gradually move to the spinal canal while its volume gradually increased. In addition, the distance from the upper pedicle was found to decrease gradually. MRN imaging can clearly show the shape, location, and adjacent relationship of the DRG, providing effective imaging guidance for the minimally invasive lumbar techniques.

Effect of Dorsal Root Ganglion Retraction in Endoscopic Lumbar Decompressive Surgery for Foraminal Pathology: A Retrospective Cohort Study of Interlaminar Contralateral Endoscopic Lumbar Foraminotomy and Discectomy versus Transforaminal Endoscopic Lumbar Foraminotomy and Discectomy.

OBJECTIVE: Postoperative dysesthesia (POD) is a common complication in surgery involving foraminal diseases, including lumbar foraminal or extraforaminal herniated nucleus pulposus (HNP). Minimal dorsal root ganglion (DRG) retraction is key to preventing POD. We compared the clinical results, safety, and efficacy between the paraspinal transforaminal approach requiring DRG retraction and the interlaminar contralateral approach without DRG retraction for foraminal and extraforaminal diseases. METHODS: A retrospective cohort study was performed of 50 patients who underwent uniportal transforaminal endoscopic lumbar foraminotomy and discectomy (TELD) and 50 patients who underwent anuniportal interlaminar contralateral endoscopic lumbar foraminotomy and discectomy (ICELF) because of lumbar foraminal HNP. The operated levels, combined degenerative diseases, postoperative complications, and POD were analyzed. The visual analog scale (VAS) pain scores, modified Oswestry Disability Index, and MacNab criteria for evaluating pain disability and response were analyzed. RESULTS: In the ICELF group (total, n = 7, 14%), there were 5 (10%) and 2 (4%) patients with POD grade 1 and 2, respectively. In the TELD group (total, n = 13, 26%), there were 7 (14%), 5 (10%), and 1 (2%) patients with POD grade 1, 2, and 3, respectively. The overall occurrence rate of grade 2 and greater POD was higher in the TELD group (n = 6, 12%) than in the ICELF group (n = 2, 4%). In the ICELF group, 3 of 9 patients (33%) with combined canal structure deforming diseases had POD, of whom none had POD of grade 2 and greater. In the TELD group, 4 of 7 patients (57%) with combined canal structure deforming diseases had POD, of whom all had POD of grade 2 and greater. Two surgical groups showed favorable clinical outcomes with the visual analog scale, Oswestry Disability Index, and MacNab criteria. CONCLUSIONS: Both TELD and ICELF were found to treat foraminal or extraforaminal HNP with good clinical outcomes. ICELF might have a lower POD rate in complicated cases such as adjacent segment disease, degenerative spondylolisthesis, and isthmic spondylolisthesis. This surgical procedure could be an alternative in complicated cases or in patients with an anatomically limited L5-S1 level. However, the procedure is technically challenging to perform.

Dorsal Root Ganglion Stimulation for the Treatment of Chronic Neuropathic Knee Pain.

BACKGROUND: To elucidate the efficacy of dorsal root ganglion stimulation in the treatment of chronic neuropathic pain confined to the knee. METHODS: Retrospective analysis of prospectively collected data of 14 consecutive patients undergoing dorsal root ganglion stimulation for chronic knee pain, in a single center. The primary outcome measure was pain reduction assessed by numeric pain rating scale score preoperatively and postoperatively. Secondary outcomes included quantification of percentage of pain area covered by stimulation, and reduction in usage of opioid medications. Responders were defined as patients that experienced a greater than or equal to 50% improvement in their preoperative pain score. RESULTS: Fourteen patients were implanted with dorsal root ganglion stimulator electrodes; 8 had a single L3 lead implanted, 1 patient had a single L4 lead implanted, and 3 patients had 2 leads implanted (L3 and L4). Two patients had their leads explanted: 1 for non-efficacy, and 1 for repeated electrode displacement. The most common indication for surgery was type 2 complex regional pain syndrome, secondary to either trauma or postoperative chronic pain (either knee replacement or arthroscopy). Median preoperative numeric rating scale score was 8.5, median postoperative numeric rating scale score was 2 (P = 0.002, Wilcoxon signed rank test). The median improvement in pain score was 80%. All 12 patients undergoing chronic stimulation were responders. Median coverage of pain area was 85%. All but 1 patient who was on opioid medication prior to surgery had reduced the dosage of regular opioid. CONCLUSIONS: In selected patients, dorsal root ganglion stimulation is an extremely efficacious means of treating otherwise refractory chronic knee pain.

Transforaminal Epidural Injection of Local Anesthetic and Dorsal Root Ganglion Pulsed Radiofrequency Treatment in Lumbar Radicular Pain: A Randomized, Triple-Blind, Active-Control Trial.

BACKGROUND: Lumbar radicular pain (LRP) results from inflammation and irritation of lumbar spinal nerves and the dorsal root ganglion (DRG). METHODS: Our study is a prospective, triple-blind, randomized, activecontrol trial (CTRI/2016/02/006666) comparing transforaminal epidural local anesthetic (LA) injection and pulsed radiofrequency treatment of DRG in patients with chronic LRP. Patients with LRP after failed conservative management for >3 months received selective diagnostic nerve root block with 1 mL 2% lidocaine. Fifty patients showing positive responses were divided into groups of 25 each. The LA group received transforaminal epidural injection of 1 mL 0.5% bupivacaine. The lumbar pulsed radiofrequency (LPRF) group received transforaminal epidural injection of 1 mL 0.5% bupivacaine with 3 cycles of pulsed radiofrequency of the DRG for 180 seconds RESULTS: Both groups were compared by observing pain intensity on a 0- to 100-point VAS and improvement in functional status by the Oswestry Disability Index (ODI version 2.0) at 2 weeks and 1, 2, 3, and 6 months. All baseline variables were comparable between the 2 groups. Statistically significant reduction in both outcomes was seen in the LPRF group compared to the LA group from 2 weeks to 6 months. One hundred percent of patients in the LPRF group had a ≥20- point decrease in VAS and significant percentage reduction in ODI at all time intervals up to 6 months, whereas it was seen in 80% and 28% of patients in the LA group at 3 and 6 months, respectively. No complications were seen in any patients CONCLUSION: Pulsed radiofrequency of the DRG applied for longer duration results in long-term pain relief and improvement in the functional quality of life in patients with chronic LRP.

T12 Dorsal Root Ganglion Stimulation to Treat Chronic Low Back Pain: A Case Series.

INTRODUCTION: Dorsal root ganglion stimulation (DRG-S) is a neuromodulation technique for treating neuropathic pain syndromes. Research has demonstrated DRG-S to be more effective than conventional SCS in treating RSD/CRPS, particularly of the lower extremities. Results from recent case series and prospective studies suggest that DRG-S may be effective in treatment of pain syndromes considered to have non-neuropathic components and characteristics (e.g. nociceptive). There have been multiple, small studies demonstrating efficacy of DRG-S for axial low back pain. There has, however, been no consensus regarding the best location for DRG lead placement in the treatment of low back pain. METHODS: Patients presenting with refractory low back pain in a private pain management practice were considered for DRG-S. Patients were provided a trial stimulator prior to potential implantation. Per standard practice, pain intensity, disability, general health status, and quality of life were followed using the visual analog scale (VAS), Oswestry Disability Index, EQ-5D index, and the SF-36 survey, respectively. Data were collected prior to implantation and at variable follow-ups after DRG-S initiation. RESULTS: Seventeen consecutive patients presented with predominantly axial low back pain with/without a secondary component of lower extremity pain. All were trialed and subsequently implanted for DRG-S. Leads were placed at T12 to target the low back. Stimulation levels were set very low, below that of which patients experienced paresthesias. Last follow-up times averaged 8.3 months. More than half of the patients experienced pain relief ≥80%, with an average low back pain relief of 78% at last follow-up. Additionally, substantial improvements in physical and mental functioning, disability, and quality of life were reported. CONCLUSIONS: T12 DRG-S can be an effective treatment for chronic axial low back pain. Stimulation results in reduced pain and disability, while improving quality of life. These outcomes can be achieved without paresthesias.

Modified technique for thermal radiofrequency ablation of Thoracic dorsal root ganglia under combined fluoroscopy and CT guidance: a randomized clinical trial.

BACKGROUND: This study is comparing thermal radiofrequency ablation (TRFA) of the thoracic dorsal root ganglia (TDRG) guided by Xper CT and fluoroscopy with the standard fluoroscopy. METHODS: This randomized clinical trial included 78 patients suffering from chronic refractory pain due to chest malignancies randomly allocated into one of two groups according to guidance of TRFA of TDRG. In CT guided group (n = 40) TRFA was done under integrated Xper CT-scan and fluoroscopy guidance, while it was done under fluoroscopy guidance only in standard group (n = 38). The primary outcome was pain intensity measured by visual analog scale (VAS) score, functional improvement and consumption of analgesics. The secondary outcome measures were patient global impression of changes (PGIC) and adverse effects. RESULTS: VAS scores decreased in the two groups compared to baseline values (p < 0.001) and were lower in CT guided group up to 12 weeks. Pregabalin and oxycodone consumption was higher in the standard group at 1, 4 and 12 weeks (p < 0.001). Functional improvement showed near significant difference between the two groups (P = 0.06 at week 1, 0.07 at week 4 respectively) while the difference was statistically significant at week 12 (P = 0.04). PGIC showed near significant difference only at week 1 (P = 0.07) while the per-patient adverse events were lower in CT guided group (p = 0.027). CONCLUSIONS: Integrated modality guidance with Xper CT-scan and fluoroscopy together with suprapedicular inferior transforaminal approach may improve efficacy and safety of TRFA of TDRG for the treatment of intractable chest pain in cancer patients. TRIAL REGISTRATION: The study was retrospectively registered at clinicaltrials.gov on 04/22/2018 (Registration No.: NCT03533413).

Microsurgery versus Medical Treatment for Neuropathic Pain Caused by Foraminal Extraforaminal Lumbar Disc Herniation: An Observational Study.

AIM: To investigate the incidence of neuropathic pain (NP) in patients with foraminal/extraforaminal lumbar disc herniation (FEFLDH), the prognosis of NP and the effect of microsurgery on patients treatment. MATERIAL AND METHODS: Two patient groups with FEFLDH were investigated: the surgery group underwent surgical treatment, and the medical-treated group received medical treatment. Patients were diagnosed with NP when the Self-Administered Leeds Assessment of Neuropathic Symptoms and Signs (S-LANSS) pain scale was ≥ 12 points. The NP scores were evaluated during patient admission and at 1, 6 and 12 months postoperation or during medical treatment. RESULTS: The surgery group included 37 patients (18 women, 19 men); FEFLDHs were localised at the L3-4 (n=9), L4-5 (n=23) and L5-S1 (n=5) levels. NP was detected in 16 patients before surgery (43%). The medical-treated group included 46 patients (19 women, 27 men); FEFLDHs were localised at the L2-3 (n=7), L3-4 (n=12) and L4-5 (n=27) levels. NP was detected in 20 patients before medical treatment (43%). The most common neuropathic symptom for patients was a burning sensation. NP was found more common in patients who were of at advanced age ( > 65 years) (p=0.019), had a longer clinical duration (p=0.007) or had a foraminal disc herniation (p=0.038). CONCLUSION: Chronic compression of the dorsal root ganglion by FEFLDH is a significant cause of NP. If surgical treatment is delayed for FEFLDH, the risk of persistent NP may increase.

CT-Guided Block and Radiofrequency Ablation of the C2 Dorsal Root Ganglion for Cervicogenic Headache.

The purpose of this report is to describe the technical aspects of CT-guided C2 dorsal root ganglion diagnostic block and radiofrequency ablation for refractory cervicogenic headache. CT guidance allows precise and safe positioning of a needle adjacent to the C2 dorsal root ganglion. At-risk neural and vascular structures can be avoided with CT, and it can provide a thorough understanding of upper cervical neuroanatomy.

Peripheral nervous system alterations in infant and adult neurofibromatosis type 2.

OBJECTIVE: To examine the involvement of dorsal root ganglia and peripheral nerves in children with neurofibromatosis type 2 compared to healthy controls and symptomatic adults by in vivo high-resolution magnetic resonance neurography. METHODS: In this prospective multicenter study, the lumbosacral dorsal root ganglia and sciatic, tibial, and peroneal nerves were examined in 9 polyneuropathy-negative children diagnosed with neurofibromatosis type 2 by a standardized magnetic resonance neurography protocol at 3T. Volumes of dorsal root ganglia L3 to S2 and peripheral nerve lesions were assessed and compared to those of 29 healthy children. Moreover, dorsal root ganglia volumes and peripheral nerve lesions were compared to those of 14 adults with neurofibromatosis type 2. RESULTS: Compared to healthy controls, dorsal root ganglia hypertrophy was a consistent finding in children with neurofibromatosis type 2 (L3 +255%, L4 +289%, L5 +250%, S1 +257%, and S2 +218%, p < 0.001) with an excellent diagnostic accuracy. Moreover, peripheral nerve lesions occurred with a high frequency in those children compared to healthy controls (18.89 ± 11.11 vs 0.90 ± 1.08, p < 0.001). Children and adults with neurofibromatosis type 2 showed nonsignificant differences in relative dorsal root ganglia hypertrophy rates (p = 0.85) and peripheral nerve lesions (p = 0.28). CONCLUSIONS: Alterations of peripheral nerve segments occur early in the course of neurofibromatosis type 2 and are evident even in children not clinically affected by peripheral polyneuropathy. While those early alterations show similar characteristics compared to adults with neurofibromatosis type 2, the findings of this study suggest that secondary processes might be responsible for the development and severity of associated polyneuropathy.

Effects of early intravesical administration of resiniferatoxin to spinal cord-injured rats in neurogenic detrusor overactivity.

OBJECTIVES: To investigate if intravesical administration during spinal shock of resiniferatoxin (RTX), an ultrapotent desensitizing agonist of transient receptor potential vanilloid-1 (TRPV1), would silence TRPV1-expressing bladder afferents at an early stage of disease progression and modulate neurogenic detrusor overactivity (NDO) emergence. MATERIALS AND METHODS: Rats submitted to largely incomplete spinal cord transection at T8/9 spinal segment were treated with intravesical RTX (50 nM) or its vehicle during spinal shock. Four weeks after spinal lesion, bladder-reflex activity was evaluated by cystometry under urethane anesthesia, after which the bladder, spinal cord, and dorsal root ganglia were collected and processed. RESULTS: We found improvements on bladder function several weeks after early intravesical RTX administration, including a marked decrease of intravesical pressures and amplitude of bladder contractions. Such strong long-lasting urodynamic effects resulted from the very potent desensitizing activity of RTX on peripheral terminals of sensory afferents, an effect restricted to the bladder. CONCLUSION: Our results support that an early intervention with RTX could potentially attenuate NDO development and ensuing urinary incontinence, with a dramatic impact on the quality of life of spinal cord injury patients.

Dorsal root ganglia volume is increased in patients with the Fabry-related GLA variant p.D313Y.

PURPOSE: To examine dorsal root ganglia and proximal nerve segments in patients carrying the Fabry-related GLA-gene variant p.D313Y in comparison to patients with classical Fabry mutations and healthy controls by morphometric and functional magnetic resonance neurography. METHODS: This prospective multicenter study examines the lumbosacral dorsal root ganglia and sciatic nerve in 11 female p.D313Y patients by a standardized magnetic resonance neurography protocol at 3 T. Volumes of dorsal root ganglia L3 to S2, permeability of dorsal root ganglia L5 and S1, and spinal nerve L5 as well as cross-sectional area of the sciatic nerve were assessed and compared to 10 females carrying a classical Fabry mutation and 16 healthy female controls. RESULTS: Compared to healthy controls, dorsal root ganglia volumes of p.D313Y females were enlarged by 53% (L3), 48% (L4), 43% (L5), 57% (S1) (p < 0.001), and 55% (S2) (p < 0.05), but less pronounced compared to females carrying a classical Fabry mutation. Compared to healthy controls, p.D313Y patients showed no changes of dorsal root ganglia vascular permeability, while patients with a classical Fabry mutation showed a distinct decrease (p < 0.05). Sciatic nerve cross-sectional area was mildly increased by 6% in p.D313Y as well as in classical Fabry patients (p < 0.05). CONCLUSIONS: Patients carrying the GLA-gene variant p.D313Y show distinctly enlarged dorsal root ganglia, while vascular permeability remains within normal limits. Overall, these alterations partially share characteristics commonly seen in patients with a mutation causing classical FD. This suggests that p.D313Y causes a potentially treatable condition resembling an early stage of Fabry disease.

Open Microsurgical Dorsal Root Ganglion Lead Placement.

INTRODUCTION: Dorsal root ganglion stimulation (DRG) is a new but well-established neuromodulation technique allowing new indications and superiority to pre-existing stimulation techniques such as spinal cord stimulation in selected pain etiologies. Previous surgical procedures in the implantation area pose a challenge for the percutaneous technique and are therefore considered contraindications for DRG stimulation surgery. We describe the successful open DRG electrode placement in two patients with previous surgeries suffering from severe radiculopathy due to foraminal stenosis. METHODS: Percutaneous implantation attempts failed and an open laminotomy/foraminotomy followed by open lead placement was performed. Leads and loops were placed under the microscope, lead location was verified by x-ray during surgery. Leads and loops were kept in position with fibrin glue and fibrin sealant patches. No special tool was required for open lead placement. RESULTS: In both patients, surgery resulted in lead and loop placement resembling the results seen in percutaneous technique. Programming and stimulation results are similar to observations made following percutaneous techniques in one patient significantly lower stimulation amplitudes were necessary. In 18 and 12 months follow-up, respectively, lead location and paresthesia coverage were stable. CONCLUSION: The option of open electrode placement should be taken into account following unsuccessful percutaneous lead placement. A combination of fibrin sealant patch and fibrin glue may be a good option for stabilization of the lead and specially of the strain relief loops in open placement. Knowledge of basic spinal surgery techniques and experience in percutaneous DRG stimulation is necessary to perform this procedure.

Dorsal root ganglia volume differentiates schwannomatosis and neurofibromatosis 2.

Schwannomatosis and neurofibromatosis type 2 are hereditary tumor syndromes, and peripheral neuropathy has been reported in both. We prospectively applied in vivo morphometric measurement of dorsal root ganglia volume in 16 schwannomatosis patients, 14 neurofibromatosis type 2 patients, and 26 healthy controls by magnetic resonance neurography. Compared to healthy controls, dorsal root ganglia hypertrophy was a consistent finding in neurofibromatosis type 2 (L3, + 267%; L4, + 235%; L5, + 241%; S1, + 300%; S2, + 242%; Bonferroni-adjusted p < 0.001) but not in schwannomatosis. Dorsal root ganglia may be a vulnerable site in origination of areflexia and sensory loss and a useful diagnostic marker in neurofibromatosis type 2. Ann Neurol 2018;83:854-857.

Stretching of roots contributes to the pathophysiology of radiculopathies.

PURPOSE: To perform a synthesis of articles addressing the role of stretching on roots in the pathophysiology of radiculopathy. METHODS: Review of relevant articles on this topic available in the PubMed database. RESULTS: An intraoperative microscopy study of patients with sciatica showed that in all patients the hernia was adherent to the dura mater of nerve roots. During the SLR (Lasègue's) test, the limitation of nerve root movement occurs by periradicular adhesive tissue, and temporary ischemic changes in the nerve root induced by the root stretching cause transient conduction disturbances. Spinal roots are more frail than peripheral nerves, and other mechanical stresses than root compression can also induce radiculopathy, especially if they also impair intraradicular blood flow, or the function of the arachnoid villi intimately related to radicular veins. For instance arachnoiditis, the lack of peridural fat around the thecal sac, and epidural fibrosis following surgery, can all promote sciatica, especially in patients whose sciatic trunks also stick to piriformis or internus obturator muscles. Indeed, stretching of roots is greatly increased by adherence at two levels. CONCLUSIONS: As excessive traction of nerve roots is not shown by imaging, many physicians have unlearned to think in terms of microscopic and physiologic changes, although nerve root compression in the lumbar MRI is lacking in more than 10% of patients with sciatica. It should be reminded that, while compression of a spinal nerve root implies stretching of this root, the reverse is not true: stretching of some roots can occur without any visible compression.

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: A case report.

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.

Human dorsal root ganglion in vivo morphometry and perfusion in Fabry painful neuropathy.

OBJECTIVE: To evaluate functional and morphometric magnetic resonance neurography of the dorsal root ganglion and peripheral nerve segments in patients with Fabry painful neuropathy. METHODS: In this prospective study, the lumbosacral dorsal root ganglia and proximal peripheral nerve segments of the lower extremity were examined in 11 male patients with Fabry disease by a standardized 3T magnetic resonance neurography protocol. Volumes of L3 to S2 dorsal root ganglia, perfusion parameters of L5-S1 dorsal root ganglia and the spinal nerve L5, and the cross-sectional area of the proximal sciatic nerve were compared to healthy controls. RESULTS: Dorsal root ganglia of patients with Fabry disease were symmetrically enlarged by 78% (L3), 94% (L4), 122% (L5), 115% (S1), and 119% (S2) (p < 0.001). In addition, permeability of the blood-tissue interface was decreased by 53% (p < 0.001). This finding was most pronounced in the peripheral zone of the dorsal root ganglion containing the cell bodies of the primary sensory neurons (p < 0.001). Spinal nerve permeability showed no difference between patients with Fabry disease and controls (p = 0.7). The sciatic nerve of patients with Fabry disease at the thigh level showed an increase in cross-sectional area by 48% (p < 0.001). CONCLUSIONS: Patients with Fabry disease have severely enlarged dorsal root ganglia with dysfunctional perfusion. This may be due to glycolipid accumulation in the dorsal root ganglia mediating direct neurotoxic effects and decreased neuronal blood supply. These alterations were less pronounced in peripheral nerve segments. Thus, the dorsal root ganglion might play a key pathophysiologic role in the development of neuropathy and pain in Fabry disease.