Pulsed Radiofrequency for Auriculotemporal Neuralgia: A Case Report.

Auriculotemporal neuralgia is a rare facial pain disorder with no therapeutic evidence for refractory cases. We described a male patient with right auriculotemporal neuralgia, refractory to anesthetic nerve blocks and botulinum toxin type A injections, who was successfully treated with pulsed radiofrequency without adverse events. Pulsed radiofrequency may be an effective and safe treatment for refractory auriculotemporal neuralgia.

Intravoxel incoherent motion magnetic resonance imaging in the assessment of brain microstructure and perfusion in idiopathic normal-pressure hydrocephalus.

PURPOSE: To determine the relationship between intravoxel incoherent motion (IVIM) MRI parameters and clinical changes post-tap test (TT) in idiopathic normal-pressure hydrocephalus (iNPH) patients. METHODS: Forty-four probable iNPH patients underwent 3 T MRI before and after TT. IVIM parameters were calculated from eight different bilateral regions of interest in basal ganglia, centrum semiovale, and corona radiata. Patients were categorized based on TT response into positive (group 1) and negative (group 2) groups. A Welch two-sample t-test was used to compare differences in D, D*, f, and ADC between the two groups, while a paired t-test was employed to assess the changes within each group before and after TT. These parameters were then correlated with clinical results. RESULTS: In the lenticular and thalamic nuclei, D value was significantly lower in the group 1 compared to group 2 both pre- and post-TT (p = 0.002 and p = 0.007 respectively). Post-TT, the positive response group exhibited a notably reduced D* value (p = 0.012) and significantly higher f values (p = 0.028). In the corona radiata and centrum semiovale, a significant post-TT reduction in D* was observed in the positive response group (p = 0.017). Within groups, the positive response cohort showed a significant post-TT increase in ADC (p < 0.001) and a decrease in D* (p = 0.007). CONCLUSION: IVIM permits the acquisition of important non-invasive information about tissue and vascularization in iNPH patients. Enhanced perfusion in the lenticular and thalamic nuclei may suggest the role of re-established microvascular and glymphatic pathways, potentially elucidating the functional improvement in motor function after TT in iNPH patients.

Botulinum toxin type A improves pain in numb chin syndrome.

Numb chin syndrome is a rare pain disorder characterized by decreased sensation and paresthesia in the territory of the mental nerve. Neuropathic pain is sometimes described in this setting, and the most common treatments include oral analgesics, gabapentinoids, and carbamazepine; however, botulinum toxin type A has never been used in this setting. We describe a case of bilateral numb chin syndrome, secondary to Burkitt lymphoma, associated with refractory and persistent burning neuropathic pain, effectively treated twelve times with subcutaneous Botulinum toxin type A (BoNT/A) injections. The procedure was well tolerated, but the patient reported incomplete mouth closure of minimal entity. BoNT/A could be a safe and effective therapy for neuropathic pain associated with numb chin syndrome.

Subclinical finding in the perception of tactile sensation involvement after SARS-CoV2 infection: comparison with healthy controls using Semmes-Weinstein monofilament testing.

Background: Post-acute COVID-19 syndrome patients complain of sensory alterations, mainly positive symptoms such as paresthesia or neuropathic pain but also decreased tactile sensation. Using the Semmes-Weinstein monofilament test (SWMT), our study aims to confront recently infected SARS-CoV2 subjects with a control group. Methods: This is a cross-sectional, single-centric study. We performed the SWMT (North Coast Medical Inc.) on 30 patients with previous SARS-CoV2 infection (COVID group) and 46 controls (control group). These patients did not present comorbidities or sensory impairment and did not take any medications. The control group tested negative for SARS-CoV2 infection since the COVID-19 pandemic; the COVID group was examined for this study after the resolution of the infection. We tested the threshold of tactile sensation of the tips of the thumb, index, and little finger of each hand, one hand at a time; the dorsum and the hypothenar regions were also tested. Results: Both groups presented the perception of tactile sensation within the reference value. Despite this result, subclinical changes suggestive of the involvement in peripheral sensory nerve function have been identified in the tested sites in the COVID group compared to the control group. The overall mean target force (grams) was higher in the COVID group than in the control group: 27 (7) vs. 19 (10) mg, p < 0.001. Conclusion: Controls and the COVID group infection had normal tactile sensation thresholds. However, the COVID group presented a higher threshold than the control group, suggesting a possible subclinical perception of tactile sensation involvement of A-beta nerve fibers.

Comparative Study of the Efficacy of Anti-CGRP mAbs on Migraineurs: Analysis of the First Year of Therapy, 1-Month Suspension Period, and Reprisal.

Background: Few studies compare the clinical effectiveness of the three anti-CGRP mAbs. Moreover, no studies compare their efficacy during suspension and reprisal. Our study aimed to compare the efficacy of migraine frequency, intensity, and symptomatic medication intake during the first year of therapy, a 1-month suspension period, and a 3-month drug reprisal. Methods: A total of 160 migraineurs (chronic and high-frequency episodic) were treated with anti-CGRP mAbs (49 with fremanezumab, 55 with erenumab, and 55 with galcanezumab) for 12 months. They discontinued the therapy for 1 month and then reprised the therapy. In the three groups, we analyzed and compared the migraine days per month, migraine intensity, and symptomatic medication intake per month at baseline, 3-month, 6-month, and 12-month follow-up. We also compared these variables during the 1-month suspension and 3 months after the reprisal of the therapy. We compared the data and evaluated the response rate (>50% reduction in migraine days per month) at different follow-ups. This comparison was also performed separately for chronic and high-frequency episodic migraineurs. Results: There was no statistical difference in monthly migraine days, intensity, or symptomatic medication intake per month at the different follow-ups. Moreover, there was no difference in the response rate overall. However, in chronic migraineurs treated with galcanezumab, the response rate was higher during the 1-month suspension when compared to fremanezumab and erenumab. In high-frequency episodic migraineurs, fremanezumab had a higher response rate at 12-month follow-up when compared to galcanezumab and erenumab. Conclusions: In our study, the three anti-CGRP mAbs presented a similar response, with no significant differences, during the first year of therapy, the suspension period, and 3 months after the drug reprisal. The response rate during the 1-month suspension period in chronic migraineurs may be higher with galcanezumab.

The Therapeutic Effect of Botulinum Toxin Type A on Trigeminal Neuralgia: Are There Any Differences between Type 1 versus Type 2 Trigeminal Neuralgia?

BACKGROUND: Botulinum toxin type A is an effective treatment for trigeminal neuralgia. Moreover, its efficacy in type 2 trigeminal neuralgia and comparative studies between type 1 and type 2 trigeminal neuralgia (TN) still need to be improved. METHODS: We treated 40 TN patients with onabotulinumtoxinA; 18 had type 1 TN, and 22 had type 2 TN. We compared the baseline pain score with the Visual Analogue Scale (VAS) and paroxysm frequency (number per week) at the baseline with those obtained at 1-month and 3-month follow-ups. Nonetheless, we compared the baseline Penn Facial Pain Scale with the scores obtained at the 1-month follow-up. RESULTS: BoNT/A effectively reduced pain intensity and frequency at the 1-month and 3-month follow-ups. Moreover, the type 1 TN and type 2 TN groups had baseline pain scores of 7.8 ± 1.65 and 8.4 ± 1.1, respectively. Pain significantly improved (p < 0.001) in both groups to 3.1 ± 2.3 (type 1 TN) and 3.5 ± 2.3 (type 2 TN) at the 1-month follow-up and to 3.2 ± 2.5 (type 1 TN) and 3.6 ± 2.5 (type 2 TN) at the 3-month follow-up. There was no difference between the two groups (p 0.345). The baseline paroxysm frequencies (number per week) were 86.7 ± 69.3 and 88.9 ± 62.2 for the type 1 and type 2 TN groups, respectively; they were significantly reduced in both groups at the 1-month and 3-month follow-ups without significant differences between the two groups (p 0.902). The Pain Facial Pain Scale improved at the 1-month follow-up, and no significant differences were found between the two groups. There was a strong correlation between background pain and paroxysm pain intensity (r 0.8, p < 0.001). CONCLUSIONS: Botulinum toxin type A effectively reduced the pain, paroxysm frequency, and PFPS scores of type 1 and type 2 trigeminal neuralgia patients without statistically significant differences. Facial asymmetry was the only adverse event.

The Effect of Three Different Ketogenic Diet Protocols on Migraine and Fatigue in Chronic and High-Frequency Episodic Migraine: A Pilot Study.

AIMS: We aimed to evaluate the efficacy of three different ketogenic diets on migraine and fatigue in chronic and high-frequency episodic migraineurs. METHODS: 76 patients with migraine were treated with the KD for at least three months. Three different KD protocols were used (2:1 KD, LGID, and VLCKD). We evaluated the fatigue severity scale (FSS), migraine frequency, migraine intensity, MIDAS, and HIT-6 at the baseline and 3-month follow-up, and we compared the results. We also correlated the mean FSS reduction with the mean migraine frequency, migraine intensity, BMI, fat mass, free-fat mass, MIDAS, and HIT-6 reduction. RESULTS: FSS improved from 4.977 ± 1.779 to 3.911 ± 1.779 at the 3-month follow-up (p < 0.001). This improvement was significant in both high-frequency and chronic migraineurs. Moreover, the three KD protocols effectively improved migraine intensity, frequency, MIDAS, and HIT-6. There was a mild correlation between mean FSS reduction (p < 0.001), mean MIDAS (p = 0.001), and HIT-6 (p = 0.002) reduction. CONCLUSIONS: The VLCKD, LGID, and 2:1 KD may improve migraine intensity, frequency, and fatigue in chronic and high-frequency episodic migraineurs.

2:1 ketogenic diet and low-glycemic-index diet for the treatment of chronic and episodic migraine: a single-center real-life retrospective study.

AIMS: The evidence supporting the efficacy of dietary preventive therapy in migraine is rising, particularly regarding the ketogenic diet. However, less evidence exists for the Low-Glycemic Index Diet and the 2:1 KD. This retrospective single-center real-life study aims to evaluate the efficacy of a 2:1 ketogenic diet and a Low-Glycemic-index Diet in chronic and high-frequency episodic migraine. METHODS: Sixty patients with high-frequency episodic and chronic migraine were treated with either a Low-Glycemic-index diet (39 patients) or a 2:1 (21 patients) ketogenic diet for three months. We collected data on the migraine frequency and intensity and the MIDAS and HIT-6 scores through the headache diary. Anthropometric measurements (BMI, fat mass, free fat mass, and weight) were also collected and analyzed similarly. Data obtained at the baseline and after three months of each diet were compared. RESULTS: Migraine intensity, frequency, MIDAS and HIT-6 scores, fat mass, weight, and BMI improved in both diet groups. CONCLUSIONS: Both diets are effective in reducing migraine symptoms and migraine-related disability.

Botulinum toxin type a for great auricular neuralgia.

great auricular neuralgia is a rare disorder with only 18 cases described in the literature. Since it's a rare disorder, there are no evidence-based therapeutic recommendations but only case reports to guide physicians. We report a case of great auricular neuralgia treated with botulinum toxin type A subcutaneous injection with significant remission of pain. Botulinum toxin type A could be an effective and safe treatment in this setting; however, more studies are needed to confirm our results.

Nummular headache: a case report of remission following ketogenic diet and botulinum toxin type A injections.

Nummular headache is an unusual facial pain disorder with no evidence-based therapy recommendations. The ketogenic diet is an alternative therapy that demonstrated to be effective in migraineurs, but it was never used in the setting of nummular headache. We describe a 58-years old female patient with nummular headache successfully treated with a 6-months ketogenic diet and botulinum toxin type A injections. Ketogenic diet could be an effective alternative/complementary therapy in nummular headache patients although more studies are needed to confirm our results.

Sleep of migraine patients is ameliorated by ketogenic diet, independently of pain control.

OBJECTIVE/BACKGROUND: Migraine patients are frequently affected by sleep complaints. The ketogenic diet (KD) is an option for the treatment of migraine. Our aim was: 1) to assess the effects of KD on sleep complaints in patients affected by migraine and 2) to verify if sleep changes were related to the effects of the diet on headache symptoms. PATIENTS/METHODS: From January 2020 to July 2022 we consecutively enrolled 70 migraine patients who were treated with KD as a preventive therapy. We collected information regarding: 1) anthropometric measures; 2) migraine intensity, frequency and disability; 3) subjective sleep complaints, i.e. insomnia, sleep quality, by the Pittsburgh Sleep Quality Index (PSQI), and excessive Daytime Sleepiness (EDS), by the Epworth Sleepiness Scale (ESS). RESULTS: After 3 months of KD therapy, anthropometric measures considerably changed, i.e. body mass index and free fat mass, and migraine significantly improved, i.e. lower intensity, frequency and disability. Regarding sleep, we observed that insomnia affected a decreased rate of patients (T0: 60% versus T1: 40%, p < 0.001). Similarly, patients with poor sleep were significantly less after KD therapy (T0: 74.3% versus T1: 34.3%, p < 0.001). Finally, EDS prevalence declined at the follow-up (T0: 40% versus T1: 12.9%, p < 0.001). Sleep features modifications were not correlated with migraine improvements and with anthropometric changes. CONCLUSIONS: For the first time we demonstrated that KD may improve sleep complaints in migraine patients. Interestingly, the positive effect of KD on sleep is independent of migraine improvements and anthropometric modifications.

Botulinum Toxin Type A for the Treatment of Auriculotemporal Neuralgia-A Case Series.

Auriculotemporal neuralgia is a rare pain disorder in which anesthetic nerve blockade is usually effective but not always resolutive. Botulinum toxin type A has proven to be effective in treating neuropathic pain, and patients with auriculotemporal neuralgia could also benefit from this treatment. We described nine patients with auriculotemporal neuralgia treated with botulinum toxin type A in the territory of auriculotemporal nerve innervation. We compared the basal NRS and Penn facial pain scale scores with those obtained 1 month after BoNT/A injections. Both Penn facial pain scale (96.67 ± 24.61 vs. 45.11 ± 36.70, p 0.004; mean reduction 52.57 ± 36.50) and NRS scores (8.11 ± 1.27 vs. 4.22 ± 2.95, p 0.009; mean reduction 3.89 ± 2.52) improved significantly at one month after treatment. The mean duration of the effect of BoNT/A on pain was 95.00 ± 53.03 days and no adverse effects were reported.

Efficacy and safety of two different botulinum toxin type A dilutions in chronic migraineurs.

Botulinum toxin type A is an effective preventive therapy for chronic migraine. Although the guidelines suggest a 50U/ml dilution of OnabotulinumtoxinA (BoNT/A), many clinicians use more concentrated solutions. However, there are no studies regarding the effect and safety of 100U/ml BoNT/A dilution with the saline solution following the PREEMPT paradigm. Our primary goal was to evaluate the efficacy, in reducing migraine frequency, and safety of two different BoNT/A dilutions (100U/ml vs 50U/ml) in the treatment of Chronic migraine. Our secondary goal was to determine the predictors of BoNT/A response. We retrospectively collected data from 113 chronic migraine patients treated with 3 rounds of BoNT/A according to the PREEMPT protocol as a preventive therapy. Patients were divided into two groups, based on BoNT/A dilution: 50U/ml (49 patients) vs. 100U/ml (64 patients) of sodium chloride 0.9%. We compared the migraine days/month, intensity, and intake of symptomatic medications at the baseline with the data obtained after the treatment; moreover, we evaluated the occurrence of adverse effects observed in the two groups. There was no difference regarding efficacy and safety between the two groups except for eyelid ptosis, which was more common in the 50U/ml BoNT/A group (p 0.018). Unilateral localization of migraine was associated with a more favorable outcome (OR 5.593, C.I. 2.358-13.268; p < 0.001) while Major Depressive Disorder predicted a less favorable response (OR 0.213, C.I. 0.087-0.523; p < 0.001). In our study, BoNT/A dilution did not influence the response to the therapy, but 100U/ml dilution could reduce the risk of eyelid ptosis. Unilateral localization of migraine pain might predict a more favorable response to the therapy, while the presence of a Major Depressive Disorder might predict a less favorable response.

Clinical applicability of arterial spin labeling magnetic resonance imaging in patients with possible idiopathic normal pressure hydrocephalus: A prospective preliminary study.

PURPOSE: idiopathic Normal Pressure Hydrocephalus (iNPH) patients have a global reduction of cerebral blood flow (CBF) and Arterial Spin Label (ASL) MRI allows a global evaluation of CBF without the injection of contrast agents. This work aims to assess the qualitative evaluation agreement of ASL CBF colored maps between different neuroradiologists and by correlating these data to the Tap Test. METHODS: Thirty - seven patients with the diagnosis of possible iNPH were consecutively submitted to a diagnostic MRI on a 1.5 Tesla Magnet before and after the lumbar infusion test and the Tap Test. Twenty - seven patients improved after the Tap Test and were addressed to surgery while 10 patients did not improve. All the MRI examinations included a 3D-Pulsed ASL sequence. Two different neuroradiologists independently reviewed all ASL images. They were asked to give a score (0 not improved; 1 improved) to global perfusion image quality by comparing ASL images obtained after the Tap Test to those obtained before. Comparison between inter- and intra-reader qualitative scores were performed with Cohen's kappa. RESULTS: Inter-reader agreement between the two neuroradiologists showed that qualitative scores were attributed similarly by two readers (k = 0.83). This technique has a good PPV (90.5 %; CI 95 %, 72.7-97.1 %), NPV (50 %; CI 95 %, 34.1-65.6 %), SN (70.37 %; CI 95 %, 49.8-86.2 %) SP (80 %; CI 95 %, 44.4-97.5 %) and accuracy (73 %; CI 95 %, 55.9-86.2 %) when considered in the setting of possible iNPH patients. CONCLUSION: ASL-MRI seems to be a promising non-invasive technique in the preoperative selection of patients affected by possible iNPH.

MDS-UDPRS-III in the diagnosis of idiopathic Normal Pressure Hydrocephalus and identification of candidates for Ventriculo-Peritoneal Shunting surgery. Results from a retrospective large cohort of patients.

OBJECTIVES: To evaluate the entity of extrapyramidal signs, characterize them and evaluate the dynamics of change by the mean of MDS-UPDRS-III in iNPH patients after the TT to determine if this tool may help the diagnosis of iNPH and the identification of candidates for Ventriculo-Peritoneal Shunting. MATERIALS AND METHODS: We retrospectively collected data from 120 patients with the initial diagnosis of possible iNPH; they underwent neurological examination by the means of MDS-UPDRS-III and other scales before and after Tap Test (TT). They were then classified as defined iNPH (57), probable iNPH (35), and NOT-iNPH (28) based on the clinical response after the Tap Test and VPS. RESULTS: After the Tap Test, defined and probable iNPH groups improved by 3.35 (2.57-4.12, p < 0.001) and 3.43 (2.43-4.4, p < 0.001) points on MDS-UPDRS-III respectively; NOT-iNPH did not improve significantly on MDS-UPDRS-III and on any other variable studies. Defined iNPH also shifted significantly from asymmetric prevalence of symptoms to a more symmetric form (from 70% before to 57% after). CONCLUSION: extrapyramidal signs improved significantly after the Tap Test in definite and probable iNPH patients. MDS-UPDRS-III may be a useful complementary tool in the diagnosis of iNPH and identification of candidates for Ventriculo-Peritoneal Shunting.

Resting-state functional-MRI in iNPH: can default mode and motor networks changes improve patient selection and outcome? Preliminary report.

BACKGROUND: Idiopathic normal pressure hydrocephalus (iNPH) is a progressive and partially reversible form of dementia, characterized by impaired interactions between multiple brain regions. Because of the presence of comorbidities and a lack of accurate diagnostic and prognostic biomarkers, only a minority of patients receives disease-specific treatment. Recently, resting-state functional-magnetic resonance imaging (rs-fMRI) has demonstrated functional connectivity alterations in inter-hemispheric, frontal, occipital, default-mode (DMN) and motor network (MN) circuits. Herein, we report our experience in a cohort of iNPH patients that underwent cerebrospinal fluid (CSF) dynamics evaluation and rs-fMRI. The study aimed to identify functional circuits related to iNPH and explore the relationship between DMN and MN recordings and clinical modifications before and after infusion and tap test, trying to understand iNPH pathophysiology and to predict the best responders to ventriculoperitoneal shunt (VPS) implant. METHODS: We prospectively collected data regarding clinical assessment, neuroradiological findings, lumbar infusion and tap test of thirty-two iNPH patients who underwent VPS implant. Rs-fMRI was performed using MELODIC-ICA both before and after the tap test. Rs-fMRI data of thirty healthy subjects were also recorded. RESULTS: At the baseline, reduced z-DMN and z-MN scores were recorded in the iNPH cohort compared with controls. Higher z-scores were recorded in more impaired patients. Both z-scores significantly improved after the tap test except in subjects with a low resistance to outflow value and without a significant clinical improvement after the test. A statistically significant difference in mean MN connectivity scores for tap test responders and non-responders was demonstrated both before (p = 0.0236) and after the test (p = 0.00137). A statistically significant main effect of the tap test on DMN connectivity after CSF subtraction was recorded (p = 0.038). CONCLUSIONS: Our results suggest the presence of a partially reversible plasticity functional mechanism in DMN and MN. Low values compensate for the initial stages of the disease, while higher values of z-DMN were recorded in older patients with a longer duration of symptoms, suggesting an exhausted plasticity compensation. The standardization of this technique could play a role as a non-invasive biomarker in iNPH disease, suggesting the right time for surgery. Trial Registration Prot. IRB 090/2021.

Dystonic tremor and blepharospasm in a patient with deletion of 18q.

18q- Syndrome is a rare chromosomic syndrome where neurological involvement is scarcely described. Movement disorders are rare and only one case with dystonia was described. In our paper, we describe the second report of a patient with 18q- Syndrome, blepharospasm, and dystonic tremor of his right hand and hyperthyroidism instead of hypothyroidism.

Subcutaneous BoNT/A Injection for Intractable Pain and Disability in Complex Regional Pain Syndrome: A Case Report.

We treated a 51-year-old woman with refractory Complex Regional Pain Syndrome type I (CRPS-I) involving her left hand and forearm with subcutaneous injections of BoNT/A. The injections were performed every 3 months, with a total of six treatments. Each treatment was able to effectively improve pain and motor impairment; however, the duration of the effect was limited to only a few months. BoNT/A could improve patients' quality of life with CRPS; however, extensive clinical studies are needed to determine its role in clinical practice.

Posterior Reversible Encephalopathy Syndrome in Guillain-Barré Syndrome: Just a Problem of Immunoglobulins? Controversy From Two Atypical Case Reports.

Background: Posterior reversible encephalopathy syndrome (PRES), reversible cerebral vasoconstriction syndrome (RCVS), or the coexistence of these two entities shares similar risk factors and clinical features. For these conditions, a common origin has been supposed. Even if the majority of patients show a favorable course and a good prognosis, a small percentage of cases develop neurological complications. Up to date, only about 30 cases of PRES associated with Guillain-Barré syndrome (GBS) have been reported in the literature. Cases: Here, we present two cases of a particularly aggressive PRES/RCVS overlap syndrome, associated with acute motor axonal neuropathy (AMAN) and acute inflammatory demyelinating polyneuropathy (AIDP) variants of GBS, respectively, presenting with similar initial clinical aspects and developing both an atypical and unfavorable outcome. On MRI examination, the first patient showed typical aspects of PRES, while, in the second case, radiological features were atypical and characterized by diffusion restriction on the apparent diffusion coefficient (ADC) map. The first patient demonstrated rapid worsening of clinical conditions until death; the second one manifested and maintained neurological deficits with a permanent disability. Conclusions: We suggest that PRES may conceal RCVS aspects, especially in most severe cases or when associated with a dysimmune syndrome in which autoimmune system and endothelial dysfunction probably play a prominent role in the pathogenesis. Although the role of IVIg treatment in the pathogenesis of PRES has been proposed, we suggest that GBS itself should be considered an independent risk factor in developing PRES.