The Hidden Face of Lyme Disease: Neuroinfection With Cranial Nerve Involvement

ABSTRACTBackgroundCranial nerve involvement is a well‐recognized feature in Guillain–Barré syndrome (GBS) but remains less well understood in chronic forms of autoimmune neuropathies. Earlier studies of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were conducted before updated diagnostic criteria and the recognition of autoimmune nodopathy (AN), which may limit the interpretation of their findings.MethodsWe retrospectively analyzed 582 patients with chronic autoimmune neuropathies—CIDP (n = 431), multifocal motor neuropathy (MMN) (n = 64), anti‐myelin‐associated glycoprotein (MAG) neuropathy (n = 54), and AN (n = 33)—from 4 Korean and 1 UK centers. Patients with cranial nerve involvement were identified and described. CIDP patients with cranial nerve involvement (cranial+ CIDP) were compared with those without (cranial− CIDP).ResultsCranial nerve involvement was observed in 8.8% (38/431) of CIDP and 24.2% (8/33) of AN patients but was absent in MMN (0/64) and anti‐MAG neuropathy (0/54). Facial palsy was overall the most common manifestation (CIDP: 45%, AN: 50%). Patients with AN more frequently exhibited bilateral optic neuropathy (50%) and facial diplegia (38%), while CIDP patients more often showed trigeminal neuropathy and oculomotor nerve palsy (both 32%). Compared with cranial− CIDP, cranial+ CIDP patients were more often younger, of variant subtypes (especially multifocal), presented (sub)acutely with preceding infection/vaccination, followed by relapsing–remitting rather than progressive courses, and achieved greater improvement despite greater pre‐treatment disability.ConclusionsCranial nerve involvement serves as a diagnostic clue in chronic autoimmune neuropathies, particularly in identifying AN and CIDP. Cranial+ CIDP appears to represent a distinct subset with partial overlap to GBS, suggesting unique underlying mechanisms.

Guillain-Barré syndrome (GBS) is an acute, immune-mediated polyneuropathy that presents significant diagnostic and therapeutic challenges, particularly in low-resource settings. While GBS has been extensively studied in high-income countries, data from conflict-affected and resource-limited regions such as Syria remain scarce. Understanding regional disease patterns and atypical clinical variants is essential for timely diagnosis and management. This prospective study was conducted at the National and Al-Mowasat University Hospitals in Damascus between September 2023 and February 2025. Patients who met the Brighton and NINDS criteria for GBS were enrolled in the study. Demographic, clinical, and electrophysiological data were collected and analysed, with subgroup comparisons between typical and atypical presentations. Forty-seven patients were included, of whom 54.4% were male. Classical ascending paralysis occurred in 83% of patients, with 55.3% classified as typical GBS and 44.7% as atypical GBS. Notable atypical features included acute anuria, isolated upper or lower limb weakness, Miller Fisher syndrome, and bilateral facial palsy or weakness with paraesthesia. AIDP was the most common subtype (59.6%), followed by AMSAN (17.0%) and AMAN (6.4%). Albuminocytologic dissociation was observed in 70.2% of cases. Atypical cases more frequently lacked a preceding infection, exhibited asymmetric or cranial nerve involvement, and showed a varied time to nadir. This is the first prospective cohort study of GBS in Syria and highlights the diverse clinical spectrum of the disease in a resource-limited context. Atypical forms constitute nearly half of all cases and present distinct diagnostic challenges. These findings emphasise the need for heightened clinical awareness and tailored diagnostic approaches in such settings.

IntroductionGuillain-Barré Syndrome (GBS) is an immune-mediated polyneuropathy that is rare in neonates. It may be acquired congenitally via maternal antibody transfer or postnatally after infection. We report a case of postnatally acquired neonatal GBS potentially triggered by SARS-CoV-2 with a review of the literature.PatientA 24-day-old term male presented with progressive hypotonia, a weak cry, and respiratory distress. He had a preceding febrile illness with gastrointestinal symptoms. Examination revealed areflexia, cranial nerve involvement, and generalised weakness.ResultsInvestigations showed albuminocytologic dissociation in cerebrospinal fluid, cauda equina enhancement on magnetic resonance imaging, and motor-sensory polyneuropathy on nerve conduction studies. The infant had high anti-SARS-CoV-2 IgG (>4000 IU/mL) with negative maternal titres. After 2 courses of intravenous immunoglobulin (IVIg), he recovered fully and remained developmentally normal at 2-year follow-up.ConclusionGBS should be considered in floppy neonates with a postinfectious presentation. This case highlights the possibility of SARS-CoV-2 as a postnatal trigger and the importance of early recognition and treatment with IVIg.

Dear Editor, We read the interesting letter to the Editor by Dr. Finsterer and appreciated the comments on our work. In this paper we aim at answering to the questions raised. For the first point, Dr Finsterer suggests to add information about Nerve Conduction Studies (NCS) and needle Electromyography (EMG); unfortunately, we are not able to give the neurophysiological investigations acquired during the first admission at the hospital. Anyway, as regards the type of GBS, we can point out that he was diagnosed with an Acute Motor Axonal Neuropathy (AMAN), as stated by previous medical documents. Furthermore, we can confirm that the patient presented cranial nerve involvement, as evidenced by the ascending paralysis which involved trunk and head control, swallowing problems, mimic musculature deficits and respiratory impairment.[...].

Varicella-Zoster Virus (VZV) is one of the most important pathogens in ophthalmology. Reactivation may involve the adnexa (blepharoconjunctivitis, pseudomembranous conjunctivitis), cornea (dendritic keratitis, nummular and necrotizing stromal keratitis, disciform endotheliitis, neurotrophic ulcers, mucous-plaque keratitis) and sclera (episcleritis, anterior scleritis). Uveal inflammation ranges from anterior uveitis-with iris atrophy, trabeculitis-induced glaucoma and complicated cataract-to posterior necrotizing syndromes: acute retinal necrosis in immunocompetent hosts and progressive outer retinal necrosis in immunosuppressed patients, often complicated by occlusive vasculitis, macular edema, retinal detachment and phthisis. Optic nerve and cranial nerve involvement (optic neuritis, neuroretinitis, III/IV/VI palsies) and orbital inflammation may occur even without cutaneous signs ("zoster sine herpete"), making PCR-based intraocular diagnostics essential. Management relies on early, high-dose antivirals (acyclovir or valacyclovir), judicious corticosteroids and timely surgical intervention when required. Universal childhood varicella vaccination and recombinant zoster vaccination in adults ≥50 years have reduced VZV incidence and ocular complications in settings with high vaccine coverage, though rare post-vaccine keratitis or uveitis underscore the need for ongoing vigilance. In this review, we synthesize current knowledge on varicella-zoster virus ocular disease, with a focus on host-pathogen interactions that drive both injury and defense.

Stroke characteristics in giant cell arteritis and Takayasu arteritis: A multicenter retrospective cohort study of 108 patients.

Outcomes of cranial nerve disorders in pharyngo-laryngeal herpes zoster: A retrospective study of 34 cases at a single institution.

Diplopia is an infrequent manifestation of GCA, but may precede other visual disturbances such as transient or permanent vision loss. The literature-pooled prevalence of diplopia in GCA patients stratified by cranial nerve (CN) involvement was assessed. The protocol was registered prospectively (PROSPERO: CRD420251071956). MEDLINE, Embase, and Cochrane CENTRAL databases were searched from inception to April 17, 2025. Studies were included if they reported on the proportion of diplopia events in patients with GCA. Literature screening, data extraction, and risk of bias (ROB) assessments were performed independently and in duplicate. A non-pairwise, random-effects meta-analysis was performed. 50 studies were included, encompassing 21,680 patients with GCA and 664 diplopia events. Overall, the pooled prevalence of diplopia in GCA patients was 7.15% (95% CI = 5.52-9.22%). The pooled prevalence of diplopia in GCA patients with CN III involvement was 0.96% (95% CI = 0.35-2.60%). The pooled prevalence of diplopia in GCA patients with CN IV involvement was 0.30% (95% CI = 0.07-1.26%). The pooled prevalence of diplopia in GCA patients with CN VI involvement was 1.41% (95% CI = 0.54-3.62%). Excluding studies with high risk of ROB, the pooled prevalence of diplopia in GCA patients was 7.14% (95% CI = 5.44-9.33%). Overall, 7.15% of patients with GCA experience diplopia, more frequently reported due to abducens nerve palsy compared to oculomotor and trochlear nerve palsies. Accordingly, new-onset binocular diplopia in adults may warrant consideration of GCA and further investigation. Future prospective studies should aim to better characterise underlying aetiologies and assess whether early recognition of diplopia translates to improved visual prognosis.

To investigate the differences in clinical characteristics, laboratory parameters, and radiological findings between patients with complete and incomplete recovery of isolated abducens nerve palsy (IANP). We retrospectively analyzed 107 patients diagnosed with IANP from 3 centers. Individuals with other cranial nerve involvement or neurological deficits were excluded. Data on demographics, clinical features, laboratory results, and imaging findings were collected and analyzed. Among 107 patients, 66 achieved complete recovery, while 41 had incomplete recovery. Analysis of clinical characteristics showed that the distribution pattern of the side of palsy (left, right, or bilateral, P = 0.018), etiology (P = 0.019) and preceding infection (P = 0.044) were significantly correlated with poor outcomes. Analysis laboratory results revealed that higher erythropoietin (EPO) levels were also significant associated with incomplete recovery (OR = 1.126, 95% CI 1.026-1.236, P = 0.013), while imaging findings and cerebrospinal fluid (CSF) parameters showed no significant association with recovery outcomes. Multivariate analysis identified four factors were associated with incomplete recovery: preceding infection (OR = 8.690, 95% CI 1.219-61.946, P = 0.031), trauma history (OR = 13.389, 95% CI 1.257-142.604, P = 0.032), time of symptom onset (OR = 1.033, 95% CI 1.002-1.064, P = 0.035), and EPO levels (OR = 1.139, 95% CI 1.035-1.253, P = 0.008). Preceding infection, trauma history, time of symptom onset, and EPO levels were identified as predictors of incomplete recovery in IANP patients, while imaging findings and CSF parameters showed no significant association with recovery outcomes. These findings provide new clinical insights by guiding prognostic assessment (e.g., preceding infection history and monitoring EPO levels) and highlighting modifiable (e.g., prompt medical intervention) and non-modifiable (e.g., trauma history) factors that personalized management strategies for IANP patients.

Radiation Therapy for Benign Diseases and Premalignant Conditions.

The infraorbital nerve, a terminal branch of the maxillary division (V2) of the trigeminal nerve, supplies sensation to the upper lip, lower eyelid, and midface. Neuropathy in this distribution typically presents as paresthesia with or without anesthesia, dysesthesia, and/or allodynia. While trauma, dental pathology, sinusitis, and certain neurological conditions are possible causes, emerging literature has identified post-viral neuropathies, including cranial mononeuropathies, as potential sequelae of COVID-19. This case highlights a rare presentation of presumed post-viral infraorbital neuropathy following an upper respiratory illness suspected to be COVID-19. A 34-year-old male with controlled hypertension developed unilateral paresthesia of the upper left lip and infraorbital region following a presumed viral illness. Multidisciplinary evaluation included imaging (CT, panoramic, and intraoral radiographs), nasal endoscopy, bloodwork, and dental assessment. An infraorbital nerve block with bupivacaine was used diagnostically to confirm localization. No dental, neoplastic, or significant sinus pathology was identified. Symptoms persisted despite corticosteroids and antibiotics. Infraorbital nerve block reproduced symptoms of paresthesia and temporarily eliminated pain, confirming the affected nerve region. Given the timing postinfection and absence of structural causes, a working diagnosis of post-viral infraorbital neuropathy was established. This case underscores the importance of including post-viral neuropathy in the differential diagnosis of facial paresthesia, particularly following respiratory infections like COVID-19. Diagnostic nerve blocks can aid both localization and symptom relief. As post-viral neurological sequelae become more recognized, clinicians must consider cranial nerve involvement even in the absence of confirmatory testing or imaging abnormalities. A multidisciplinary approach remains essential for accurate diagnosis and effective management.

Takotsubo cardiomyopathy (TCM), also known as stress cardiomyopathy, affects 0.02% of hospitalized patients and is primarily triggered by emotional stressors, although unusual medical situations have also been documented. We report the case of a 36-year-old female patient who presented with symptoms of infection (oropharyngeal pain and diarrhea) 10 days before admission. Upon arrival at the emergency department, she exhibited progressive weakness, lower cranial nerve involvement, and areflexia, necessitating invasive mechanical ventilation support. Diagnostic evaluation revealed sinus tachycardia and atrial fibrillation, leading to a diagnosis of Guillain-Barré syndrome (GBS) with associated cardiovascular dysautonomia. Echocardiography demonstrated apical hypokinesia of the left ventricle, consistent with TCM. Furthermore, her thyroid profile indicated hyperthyroidism, fulfilling the criteria for a thyroid storm. Treatment comprised intravenous human immunoglobulin, methimazole, and propranolol. We present the first documented case of TCM attributed to dysautonomia resulting from GBS and thyroid storm.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated neuropathy characterized by demyelination and axonal injury of peripheral nerves. While its hallmark presentation involves progressive sensorimotor deficits, an underrecognized subset of patients develop ocular and neuro-ophthalmic manifestations. These include optic neuritis, optic atrophy, vision loss, ophthalmoplegia, extraocular muscle enlargement with proptosis, intracranial hypertension, and corneal small fiber pathology. Electrophysiologic and imaging studies, including visual evoked potentials, optical coherence tomography, and corneal confocal microscopy, have further revealed subclinical involvement of the optic nerve and retina, underscoring an expanded disease spectrum beyond classic peripheral neuropathy. The pathophysiology is multifactorial, involving demyelination, inflammatory cell infiltration, and immune-mediated axonal injury, with evidence suggesting potential central nervous system and cranial nerve involvement. These atypical manifestations often mimic disorders such as multiple sclerosis, neuromyelitis optica spectrum disorders, or thyroid eye disease, complicating early diagnosis. Importantly, most cases respond to standard immunomodulatory therapies, including intravenous immunoglobulin, corticosteroids, and plasma exchange. Advances in ocular imaging and biomarker discovery provide promising avenues for earlier detection and monitoring of neuro-ophthalmic involvement. Recognition of ocular features in CIDP is essential for timely diagnosis and treatment. Interdisciplinary collaboration between neurologists and ophthalmologists can prevent vision-threatening complications, refine diagnostic accuracy, and optimize long-term outcomes. Future studies are warranted to elucidate the mechanisms underlying ocular involvement and to evaluate emerging targeted therapies that may improve prognosis in these atypical but clinically significant phenotypes.

Idiopathic intracranial hypertension (IIH) typically presents with chronic headache, pulsatile tinnitus, transient visual obscurations, and papilledema. The fulminant form, representing 2-3% of cases, is characterized by rapid visual decline within four weeks of symptom onset. Sixth cranial nerve palsy is the most frequently reported cranial neuropathy in IIH, whereas involvement of other cranial nerves is exceedingly rare. We describe a 16-year-old girl with fulminant IIH and oculomotor nerve palsy associated with risperidone-induced weight gain. She presented with one week of nausea, vomiting, and occipital headache, followed by blurry vision and right ptosis. Examination revealed partial right oculomotor palsy with ptosis, a dilated pupil without a relative afferent pupillary defect, and severe bilateral papilledema. Neuroimaging excluded mass lesion, aneurysm, and inflammation, but showed findings consistent with IIH. Lumbar puncture revealed an opening pressure of 86 cm H2O with normal CSF composition. Due to progressive symptoms, she underwent ventriculoperitoneal shunting, with near-complete resolution of oculomotor palsy and mild residual ptosis at two-week follow-up. Oculomotor nerve palsy is a rare manifestation of IIH; including our case, we identified eight patients (seven female) presenting in the setting of IIH. All presented with new-onset IIH, and three were fulminant cases with markedly elevated CSF pressures (>50 cm H2O) and ultimately received shunting. Fulminant IIH should be considered in the differential diagnosis of acute oculomotor palsy, particularly when risk factors such as recent weight gain or medication use are present, as early recognition and CSF diversion are important to prevent irreversible vision or cranial nerve deficits.

BackgroundHereditary gelsolin amyloidosis (AGel amyloidosis) is a rare autosomal dominant systemic amyloidosis caused by mutations in the Gelsolin (GSN) gene encoding gelsolin. The condition is characterized by a triad of cranial nerve involvement, corneal lattice amyloidosis, and skin laxity, with a small proportion of cases involving the kidneys and heart. We report the first case of renal AGel amyloidosis associated with a c.487G > A mutation in the GSN gene, presenting as isolated proteinuria.Case presentationA 55-year-old woman presented with proteinuria. She had a history of hypertension and diabetes but no family history of kidney disease. Physical examination revealed no abnormalities in the heart, eyes, nerves, or skin. Urinalysis showed moderate proteinuria (1975.5 mg/24h), and serum creatinine was normal (0.71 mg/dL). Renal biopsy revealed Congo red-positive glomeruli on light microscopy, with apple-green birefringence under polarized light. Electron microscopy showed randomly arranged fibrillar deposits in the glomeruli. Mass spectrometry analysis confirmed that the deposits were consistent with gelsolin protein. Genetic testing revealed a heterozygous missense mutation in the GSN gene (NM_198252.3; c.487G > A; p.Asp163Asn). The patient was diagnosed with AGel amyloidosis. Additionally, we compiled the phenotypic and genotypic characteristics of previously reported AGel amyloidosis cases.ConclusionWe report a novel mutation in AGel amyloidosis with renal involvement. This case highlights the importance of renal biopsy, mass spectrometry analysis, and genetic testing in establishing a definitive diagnosis. It expands the known spectrum of GSN gene mutations and further supports the heterogeneity of the AGel amyloidosis phenotype.Supplementary InformationThe online version contains supplementary material available at 10.1186/s12882-025-04599-x.

Nasopharyngeal adenoid cystic carcinoma (NACC) is an exceptionally rare malignancy, representing <1% of nasopharyngeal tumors. Over the last decade, fewer than 200 cases have been reported worldwide, usually presenting at an advanced stage with perineural invasion and skull base involvement. The role of surgery is often limited, and particle therapy, including intensity-modulated proton therapy (IMPT) and carbon ion radiotherapy (CIRT), has emerged as a promising alternative. We report the case of a 35-year-old male with NACC presenting with nasal obstruction, epistaxis, and cranial nerve involvement. MRI revealed a 45×35×40 mm mass extending to the clivus, cavernous sinus, and parapharyngeal space. Histopathology confirmed adenoid cystic carcinoma (ACC) (cribriform and solid type), with immunohistochemistry positive for CD117 and CK7, focally positive for p63 and smooth muscle actin (SMA), and negative for p40 and Epstein-Barr virus (EBV) (in situ hybridization). The patient underwent IMPT to a total dose of 45 Gy (relative biological effectiveness [RBE]) in 15 fractions, as recommended by a multidisciplinary tumor board. Treatment was well tolerated, with grade 2 mucositis, grade 2 otitis media, and mild xerostomia. However, pulmonary metastases developed within 10 months, and systemic therapy with lenvatinib plus doxorubicin was initiated with limited efficacy. This case illustrates the challenges of managing NACC, emphasizing the role of particle therapy for local control (LC), the importance of multidisciplinary evaluation, and the need for vigilant follow-up given the high risk of distant metastases. Collaborative registries and clinical trials are warranted to optimize treatment strategies for this rare disease.

Abstract Dengue, a major mosquito-borne infection, can rarely cause neurological complications. We report a case of a man in his early thirties presenting with fever, myalgia, and progressive limb weakness. Examination showed fever (38°C), tachycardia (110/min), absent deep tendon reflexes, muscle strength of 1/5 in all limbs, and severe muscle tenderness, with no sensory or cranial nerve involvement. Investigations revealed hypokalemia (2.2 mEq/L), thrombocytopenia (12,000/ml), elevated CPK (1174 U/L), and dengue IgM positivity. A diagnosis of dengue-associated myositis with hypokalemic paralysis was made. The patient responded well to potassium supplementation, antipyretics, and supportive care, with complete recovery within 1 week.

Tunnel sign on magnetic resonance imaging in neuromelioidosis: A systematic literature review.

Cranial neuropathies related to immune checkpoint inhibitors.