Pediatric H3K27-altered glioma most frequently arises from midline cerebral parenchyma, whereas intracranial extra-axial locations are exceedingly rare. We report the case of an 8-year-old boy who presented with chronic-onset left-sided hearing impairment and peripheral facial paralysis. Radiological imaging identified an isolated cerebellopontine angle lesion distinct from surrounding structures. A vestibular schwannoma was diagnosed based on preoperative examinations. Surgical resection revealed an unusual facial nerve-infiltrating mass with distinct vascular features. Histopathological assessment yielded an unexpected diagnosis of H3K27-altered diffuse midline glioma, contrasting with initial preoperative impressions. This exceptional case illustrates diagnostic pitfalls in pediatric skull base pathologies and expands the recognized spectrum of diffuse midline gliomas.

Melkersson–Rosenthal syndrome (MRS) is a rare disorder that belongs to the group of orofacial granulomatosis. It is classically defined by a clinical triad consisting of recurrent ‎orofacial edema, peripheral facial nerve palsy, and fissured tongue, although the complete ‎form is uncommon. Diagnosis relies mainly on histopathological examination, which demonstrates non-caseating granulomatous inflammation. The etiology and pathophysiology of MRS ‎remain unclear, and current treatments are essentially symptomatic, with variable efficacy ‎and frequent recurrences. Dentists play a crucial role in early diagnosis, as oral manifestations ‎are often the first signs of the disease. This article reports a clinical case and discusses the ‎diagnostic approach and therapeutic management of MRS based on a review of the ‎literature‎.

Evidence summary: facial nerve rehabilitationtraining management for patients with peripheral facial paralysis.

A Longitudinal Cohort Study of Children with Peripheral Facial Nerve Palsy in Lyme Disease Endemic Areas.

Introduction: Ramsay Hunt Syndrome is an uncommon disease caused by reactivation of latent varicella zoster virus infection in the geniculate ganglion. The classic triad consists ofotalgia, vesicles in the auditory canal and ipsilateral facial paralysis. Without treatment, full recovery of the facial paralysis occurs in 20% of cases; this is much improved if treatment is started within 72 hours. Zoster sine herpete is a subtype of RH syndrome and consists of facial paralysis without vesicular rash in the skin and mucosa. Methodology: We present the clinical cases of two patients who presented to our clinic with peripheral facial paralysis but without auricular skin rash. Serological laboratory tests were positive for HZV. Both were placed on corticosteroid and antiviral therapy. According to House Beckerman classification the grade of paralysis, improved for the first case from grade 4 to grade 1 and for the second case, from grade 5 to grade 4. The time of initiation of corticosteroid and antiviral therapy was different. In the first case, therapy was started immediately and in the second case after 5 days. Conclusion: RHS is a rare syndrome that should not be underestimated. It is presented by a typical tirade: facial paralysis, auricular skin rash and otalgia. A special manifestation is the Zoster sine herpete form, which is diagnosed by serology and PCR for viral DNA. Immediate initiation of treatment (within 72h) is an important factor in prognosis.

The heterogeneous and homogeneous clinical manifestations of peripheral facial palsy (FP), hemifacial spasm (HFS), and Meige's syndrome (MS) complicate the differentiation of diagnoses for these facial motor diseases. To comprehensively investigate the white matter microstructural characteristics in patients with facial dyskinesias and palsy using geometric and integrity metrics in DTI. In this prospective study conducted from September 2020 to January 2022, patients with FP, HFS, and MS, as well as sex-matched healthy control subjects, underwent 3.0 T MRI. Geometric metrics (i.e., splay, bend, twist, and total distortion) based on "Director Field Analysis" and fractional anisotropy (FA) and mean diffusivity (MD) were calculated from DTI data. Cross-sectional tract-based spatial statistics were performed among FP, HFS, MS patients, and healthy controls. The correlation between disease severity and DTI metrics was evaluated. Additionally, the geometric microstructural properties combining FA and MD were used to classify FP, HFS, and MS patients using machine learning methods. Geometric metrics and FA/MD were widely altered across white matter in FP and HFS patients compared with healthy controls. However, in MS patients only DFA metrics were significantly altered. FA and DFA values strongly correlated with the severity of facial movement disorder in FP patients. Combing conventional FA/MD value with DFA metrics enabled the diagnostic differentiation of FP and HFS from MS. Our findings demonstrated that the geometric microstructural information of white matter fibers could provide novel insight into the underlying pathological changes in facial dyskinesias and palsy.

Facial paralysis, whether traumatic, idiopathic or secondary to surgery, causes significant functional and aesthetic impairment. Among the various surgical options for facial rehabilitation, temporal muscle lengthening myoplasty is an effective technique for restoring the symmetry and dynamics of the smile. This procedure involves mobilising the temporalis muscle, which is generally spared in peripheral facial paralysis, and transposing it to the labial commissure. We report the case of a patient treated in our department for sequelae of facial paralysis who underwent allograft myoplasty of the temporalis muscle using the LABBE technique with a satisfactory result, demonstrating the effectiveness and cost-effectiveness of this technique.

Peripheral facial palsy represents a neurological condition with motor repercussions. Although physiotherapy is the mainstay of treatment, the evidence on the clinical efficacy of low-level laser therapy (LLLT) using photobiomodulation is still limited and heterogeneous. The primary objective is to assess the impact of low-level therapeutic laser (Class 3B) on the functional recovery of patients with peripheral facial paralysis treated at the Daniel Alcides Carrión Hospital in Huancayo, Peru. A pre-post quasi-experimental study was conducted in 41 patients with peripheral facial paralysis, of which 80.5% had Bell’s palsy. The treatment consisted of photobiomodulation with low-intensity laser (1064 nm, 63 mW, 3 J/cm2, 40 s/point) applied in ten sessions. Facial function was assessed using the House-Brackmann scale before and after treatment. The data were analyzed with the Wilcoxon test (p<0.05). The median House-Brackmann score was reduced from 4 to 2, representing a significant improvement (Δ=−2; p<0.001; r=0.87). LLLT-based photobiomodulation has proven to be safe and effective in the improvement of facial function in peripheral facial palsy, with no adverse effects. Patients with symptom duration of less than 4 weeks had significantly better functional recovery. The therapeutic effect of LLLT is supported by these results. Therefore, strict randomized controlled trials (RCTs) should be required to verify these results and provide unanimous dosimetry and frequency protocols to achieve the best clinical implementation.

Abstract Background Eagle syndrome (ES) is a rare entity caused by elongation of the styloid process or calcification of the stylohyoid ligament, leading to compression of surrounding anatomical structures. This can result in a variety of symptoms, including atypical cranial neuralgias and, in rare cases, cranial nerve palsy. Due to its nonspecific presentation, ES is often misdiagnosed as more common conditions such as trigeminal neuralgia or temporomandibular joint disorders. Case presentation We report two cases of ES with distinct clinical manifestations. The first case involves a 41-year-old female with chronic headache and facial pain with allodynia that progressed to left-sided peripheral facial palsy. Cervical Computed Tomography (CT) scan with 3D reconstruction revealed an elongated styloid process with narrowing of the pre-styloid space. The second case describes a 24-year-old female who presented with Ludwig’s angina and a history of intermittent unilateral headaches and facial pain that were resistant to pain medication. A cervical CT scan incidentally identified an elongated styloid process. Both imaging results identified ES as the cause of these atypical pain presentations. Both patients initially received conservative management, with one patient later undergoing surgical treatment and achieving significant pain relief. Conclusions These cases highlight the need for clinicians to consider ES in patients with atypical and treatment-resistant cranial neuralgia or facial nerve palsy. Diagnosis relies on a combination of clinical findings and imaging, with 3D CT reconstruction as the gold standard. Increased clinical awareness are crucial for timely diagnosis and management.

Background: We report an uncommon presentation of Lyme disease and highlight the importance of a detailed history in a patient with new-onset sixth nerve palsy. Methods: Case report and literature review. Results: A 46-year-old man receiving infliximab presented to the ophthalmology emergency department with horizontal binocular diplopia. History revealed a diffuse headache that had begun three weeks earlier. Ophthalmologic examination demonstrated a left sixth cranial nerve palsy. The workup showed positive Borrelia serum IgG, which was interpreted as a likely false-positive result given the limited specificity of serologic testing. At follow-up, the patient reported left-sided peripheral facial palsy, and worsening headache and diplopia. Further history revealed prior erythema migrans treated with doxycycline four months earlier. Considering these new findings, a lumbar puncture was performed and demonstrated intrathecal production of Borrelia antibodies. Neuroborreliosis, a neurologic involvement secondary to systemic infection by the spirochete Borrelia burgdorferi, was diagnosed. The patient was treated with oral doxycycline for 28 days with complete resolution of symptoms. Conclusions: Lyme disease may present with progressive neuro-ophthalmologic symptoms, underscoring the crucial role of ophthalmologists in its diagnosis. Moreover, immunosuppression may delay diagnosis and allow neurological progression, highlighting the need for careful history taking and close follow-up.

To observe the clinical efficacy of pulling technique by needle stuck for subacute peripheral facial paralysis based on the infrared thermography. A total of 60 patients with subacute peripheral facial paralysis were randomized into an observation group and a control group, 30 cases in each one. In the control group, conventional acupuncture was applied at Qianzheng (Extra), Sibai (ST2), Yangbai (GB14), Yuyao (EX-HN4), Jiache (ST6), Dicang (ST4), and etc. on the affected side. On the basis of the treatment in the control group, in the observation group, acupuncture at Yangbai (GB14)-toward-Yuyao (EX-HN4), Sibai (ST2)-toward-Dicang (ST4), and Dicang (ST4)-toward-Jiache (ST6) was applied and combined with pulling technique by needle stuck. In both groups, the needles were retained for 30 min a time, once every other day, until the 28th day of the disease course. Before and after treatment, the temperature difference (ΔT) between the healthy side and the affected side of Yangbai (GB14), Sibai (ST2), and Dicang (ST4) was measured by infrared thermography; the House-Brackmann (H-B) grade and the Sunnybrook facial nerve assessment scale score were compared in the two groups. The recovery time was compared by survival analysis, and the clinical efficacy was evaluated in the two groups. After treatment, ΔT of Yangbai (GB14), Sibai (ST2), and Dicang (ST4) was decreased compared with that before treatment in the two groups (P<0.05), and that in the observation group was lower than the control group (P<0.05). After treatment, the H-B grade and the Sunnybrook facial nerve assessment scale scores were improved compared with those before treatment in the two groups (P<0.05), and those in the observation group were better than the control group (P<0.05). The median recovery time in the observation group was shorter than that in the control group (P<0.05). The total effective rate was 96.7% (29/30) in the observation group, which was superior to 90.0% (27/30) in the control group (P<0.05). Pulling technique by needle stuck can enhance the clinical efficacy when added to conventional acupuncture for subacute peripheral facial paralysis, improve the clinical symptoms and shorten the course of the disease.

Background: Facial palsy constitutes a profoundly disabling condition, often leading to marked functional deficits and a decline in facial appearance, which substantially reduces the patient’s quality of life. A combined therapy of botulinum toxin (BoNTA), hyaluronic acid (HA) and calcium hydroxylapatite (CaHA) appears promising in the pharmacological approach of these patients. Methods: We reported our single center experience of patients with facial palsy, either of central or peripheral etiology who were treated with the combination of BoNTA, HA and CaHA, during a 6-month period (January 2025–June 2025). Results: Eight consecutive adult patients [mean age: 49.50 ± 7.95 years, 6 (75%) female] with facial palsy, either of central (4 patients) or peripheral (4 patients) etiology, received the combination of BoNTA, HA and CaHA. No serious adverse reactions were documented. Localized bruising and swelling at injection sites resolved without requiring any additional intervention. Facial Disability Index (FDI) was assessed both prior to and following treatment. The functional subscale increased from 65.63 ± 16.13 to 80.63 ± 10.50 (improvement rate = 24.4%, p-value = 0.002), while the psychosocial subscale increased from 63.00 ± 17.34 to 74.50 ± 10.89 (improvement rate = 18.3%, p-value = 0.004). Consequently, the total FDI score improved from 128.63 ± 28.92 to 155.13 ± 17.96 (overall improvement = 20.6%, p-value = 0.001). Conclusions: The present case series underscores the potential therapeutic role of CaHA as an adjunct to BoNTA and HA injections in patients with central or peripheral facial palsy.

A man in his 50s experienced novel, continuous, and progressive headache and neck pain prior to the onset of left-sided peripheral facial nerve palsy. Sequential palsies of left lower Cranial Nerves IX and XII followed. Imaging showed spontaneous cervical artery dissection (sCeAD) of the ipsilateral internal carotid artery. Lower cranial nerve palsies in sCeAD are a frequent result of a local mass effect exerted by the formation of a mural hematoma. The only close topographical relationship between the facial nerve and the internal carotid artery is within the petrous part of the temporal bone but still separated in two different bony canals (facial canal and carotid canal). Thus, a mural hematoma of an internal carotid artery dissection could not cause compression of the facial nerve. In the rare case of facial nerve palsy due to sCeAD, hypoperfusion of the vasa nervorum is the most likely cause. As sCeAD is one of the main reasons for stroke in the youth, it is critical to know and identify potential red flags in patients with peripheral facial nerve palsy, which should lead to additional vascular imaging.

Efficacy of acupuncture combined with various traditional Chinese medicine therapies for peripheral facial paralysis: A systematic review and network meta-analysis.

Chronic non-flaccid peripheral facial palsy is frequently associated with synkinesis, residual motor deficits, and reduced quality of life. Evidence-based, standardized rehabilitation protocols remain limited. This study examined the effect of an intensive two-week electromyography (EMG) and video-based biofeedback program on changes in physical and psychosocial patient-reported outcomes in patients with chronic facial palsy. This retrospective cohort study included patients with chronic non-flaccid facial palsy, either with synkinesis or residual hypotonia, who completed a two-week EMG and video-biofeedback program between 2020 and 2023. The intervention targeted synkinetic co-activation and improved voluntary motor control in hypotonia. Data consisted of routine documentation and patient-reported outcome measures (PROMs) collected at baseline 6 months before therapy (T1), therapy initiation (T2), therapy conclusion (T3), and six-month follow-up (T4). PROMs included the Facial Disability Index (FDI), Facial Clinimetric Evaluation Scale (FaCE), Short Form-36 Health Survey (SF-36), and Beck Depression Inventory (BDI). Analyses used repeated-measures ANOVA and segmented regression. A total of 175 patients were included. Significant improvements were observed across all PROMs. From T2 to T3, FDI total score increased by 5.24 points (95% CI 3.90 to 6.60) and FaCE total score by 9.86 points (95% CI 7.80 to 11.92). SF-36 showed improvements in social functioning (+4.67 points, 95% CI 2.19 to 7.15) and emotional well-being (+4.32 points, 95% CI 2.60 to 6.00). BDI decreased by 3.29 points (95% CI -4.63 to -1.96). Segmented regression indicated small but significant pre-therapy improvements from T1 to T2. At follow-up, outcomes remained above baseline, with FDI total score rising from 65.64 ± 14.88 at T1 to 75.31 ± 13.83 at T4 and FaCE total score from 53.89 ± 16.54 to 63.72 ± 17.10 (all p < 0.001). Older age was associated with lower FDI values; male gender was associated with higher FDI and FaCE and lower BDI scores. Participation in an intensive EMG- and video-based biofeedback program was associated with clinically relevant and sustained improvements in facial function, quality of life, and psychosocial well-being in patients with chronic non-flaccid facial palsy. Age- and gender-related differences highlight the importance of individualized rehabilitation approaches.

To observe the clinical effect of electroacupuncture (EA) at different intensities on peripheral facial paralysis (PFP) and its influence on brainstem excitability. Sixty-six patients with PFP were randomly assigned to a motion-threshold EA group (22 cases, 2 dropped out), a sensory-threshold EA group (22 cases, 1 dropped out) and a sham-EA group (22 cases, 2 dropped out). Sibai(ST2), Dicang(ST4), Hegu(LI4) and Yuji(LU10) on the affected side were selected in 3 groups and stimulated with EA. In the motion-threshold EA group, the stimulation intensity was determined by the visible muscle jumping in the local area. In the sensory-threshold EA group, the minimal sensory stimulation was adjusted. In the sham-EA group, the skin was not invaded and no electric stimulation was delivered. In each group, the intervention was given once every 3 days, 30 min each time and the duration of treatment was composed of 20 interventions. At the baseline (T0), after the 10th intervention (T1), after the 20th intervention (T2) and in 1 month followup after treatment completion (T3), the House-Brackmann scale for grading facial nerve function (H-B score) and the facial disability index (FDI, comprising physical function [FDIP] and social function [FDIS]) were assessed separately. Besides, the changes of facial nerve injury rate and blink reflex (BR) were observed before and after treatment. In the self-group comparison, H-B score at T2 and T3 was improved significantly compared with that at T0 (P<0.001, P<0.05) in the motion-threshold EA and sensory-threshold EA groups separately;FDIP score was increased significantly at T2 and T3 in the motion-threshold EA group (P<0.001), and FDIP score was increased significantly at T2 in the sensory-threshold EA group (P<0.01);the FDIS score was reduced significantly from T1 to T3 in the motion-threshold EA group (P<0.05, P<0.001), and it was reduced significantly at T2 and T3 in the sensory-threshold EA group (P<0.001, P<0.05). In comparison with the sham-EA group at the same time point, H-B score and FDIS score at T2 and T3 were decreased in the motion-threshold EA group and the sensory-threshold EA group (P<0.01, P<0.05, P<0.001). After treatment completion, in the motion-threshold EA group and the sensory-threshold EA group, the injury rate of the temporal and buccal branches of the facial nerve was declined (P<0.01, P<0.001) compared with baseline, and it was lower than that of the sham-EA group (P<0.001, P<0.01). Besides, the R2 latency was shorter in comparison with the baseline in the motion-threshold EA group and the sensory-threshold EA group (P<0.01), and the latency in the motion-threshold EA group was superior to the sensory-threshold EA group (P<0.05). EA at both motion threshold and sensory threshold obtains a similar therapeutic effect on PFP, and it effectively relieves symptoms of PFP and presents a certain long-term effect. Motor-threshold EA demonstrates modulatory effect on the brainstem's neural reflex pathway.

BACKGROUND Multifocal acquired demyelinating sensory-motor neuropathy (MADSAM) is recognized as a variant of chronic inflammatory demyelinating polyneuropathy. The primary characteristics of MADSAM include multifocal sensory loss and muscle weakness, which are frequently asymmetrical and predominantly affect the upper limbs. Involvement of the lower limbs is less commonly observed in MADSAM. CASE REPORT A 27-year-old female patient presented with recurrent numbness and weakness in her left lower limb was admitted to our hospital. Her medical history included episodes of left peripheral facial paralysis and lower-limb numbness and weakness, which had previously improved after short-term oral steroid therapy. In addition to motor and sensory peripheral nerve impairment in the left lower limb, the neurological examination revealed atrophy of the tongue muscle and a leftward deviation of the tongue. Cerebrospinal fluid examination and magnetic resonance imaging indicated no abnormalities. Electromyography suggested demyelination of motor and sensory nerves in the left lower limb. Sural nerve biopsy demonstrated demyelination changes and axonal degeneration. A diagnosis of multifocal sensory and motor neuropathy was considered, and the patient was administered corticosteroids and tacrolimus. As the condition progressed, electromyography showed gradual involvement of both lower limbs, leading to the consideration of MADSAM. Despite treatment with corticosteroids and tacrolimus, the patient experienced relapse. Rituximab was initiated, resulting in symptoms improvement and reduced recurrence without adverse events. CONCLUSIONS Corticosteroids, plasma exchange, and immunoglobulins have been demonstrated to be effective treatments for CIDP. In our MADSAM case, rituximab proved effective when the patient did not respond to corticosteroids and tacrolimus. We propose that rituximab may serve as an alternative option for patients with MADSAM.

Background and purposeFacial nerve palsy is the most common cranial nerve disorder, and over 60% are idiopathic (Bell’s palsy, BP). An inflammatory process is discussed as a causative factor. The aim of this study was to search for changes in various cytokine concentrations in serum and cerebrospinal fluid (CSF) in patients with facial nerve palsy.MethodsIn this prospective study, 47 patients with peripheral facial nerve palsy were included. Of these, 40 patients were diagnosed as BP and seven patients as non-idiopathic peripheral facial palsy (nipFP). Seventeen cytokines, including IL-1A, IL-5, IL-9, granulocyte colony-stimulating factor (G-CSF), CXCL-13, granulocyte–macrophage-colony-stimulating factor (GM-CSF), tumor necrosis factor-SF13 (TNFSF13), IL-8, IL-1ß, CXCL-10, fractalkine (Fract), monocyte chemotactic protein (MCP-1), IFN-y, IL-4, IL-17A, tumor necrosis factor (TNF), and Granzyme, were measured in the serum and CSF. For comparison, sera from 10 healthy individuals were used.ResultsIn serum, the levels of cytokines G-CSF, CXCL13, TNFSF13, and Granzyme were statistically significantly higher in patients with BP and nipFP compared to healthy individuals (p < 0.05). Cytokine IL-1ß was significantly higher in nipFP patients compared to healthy individuals and patients with BP (p < 0.05). Cytokine IL-8 was significantly lower in both patient groups than in healthy individuals (p < 0.05). In CSF, G-CSF, CXCL13, TNFSF13, IL-8, IL-1ß, and IL-17A were statistically significantly higher in patients with nipFP compared to patients with BP (p < 0.05). In addition, patients with BP also showed a clustering of cytokine elevation. For differentiating nipFP from BP, serum G-CSF and IL1ß indicated a certain discrimination (AUC 0.643; 0.614). Patients with severe facial palsy revealed higher CSF TNFSF13 (p = 0.02), and clinical outcome after 3 months was less favorable at higher CSF Fractalkine (p = 0.025). Elevated CSF cell count was associated with elevated CXCL13, IL-8, IL-1ß, IP-10, IFNa and granzyme in the CSF (r = 0.32–0.57; p < 0.05).ConclusionCompared to healthy individuals, our study revealed an altered cytokine profile in patients with BP that resembles patients with nipFP. In CSF, a subset of cytokines was identified in patients with BP, but higher levels were found in patients with nipFP, suggesting a graduated inflammatory process.Study registrationThe study "Serum and CSF cytokine profile in patients with facial palsy (Bell´s palsy)" has been officially registered at the German Clinical Trials Register (DRKS00037815).

ObjectivesThis study aimed to determine the preferred timing and measurement sites for electroneuronography (ENoG) to predict early recovery from acute peripheral facial paralysis.MethodsWe retrospectively evaluated 42 patients with acute peripheral facial paralysis who received standard treatment with oral corticosteroids. The severity of facial paralysis was assessed at the initial visit and after 1 month using the House–Brackmann grading system. Patients were classified into recovery and non-recovery groups according to changes in the grade. ENoG was performed at the initial visit and after 2 weeks. ENoG amplitudes of four facial muscles (frontalis, nasalis, orbicularis oculi, and orbicularis oris) at the initial visit and after 2 weeks, as well as age, sex, affected side, and diagnosis, were compared between the two groups.ResultsNo differences were observed in degeneration ratios across all subsites in the initial ENoG, which can be explained by the fact that Wallerian degeneration is not yet complete at this early stage. However, the second ENoG, performed after degeneration had progressed, showed significant differences across all subsites. Binary logistic regression analysis revealed that the degeneration ratio of the orbicularis oris muscle was the best predictor of early recovery (odds ratio, 0.961; p = 0.014). Receiver operating characteristic curve analysis also revealed that the degeneration ratios of all subsites measured in the second ENoG were useful in predicting early recovery, with the highest possibility at the orbicularis oris muscle (area under the curve = 0.789). When the degeneration ratio exceeded 60% in all subsites in the second ENoG, a favorable prognosis was not expected.ConclusionThis study provides the preferred testing time and measurement sites for ENoG to predict early recovery from facial paralysis. Given the personal and social impact of facial paralysis, predicting early recovery is crucial for reassuring patients, providing better treatment, and encouraging early reintegration into society.

Guidelines for Lyme borreliosis: clinical manifestations.