Multiple Cranial Neuropathies Research Articles

Multiple Cranial Neuropathy with Anti-Ma2 Antibody

Multiple Cranial Neuropathy with Anti-Ma2 Antibody

Increased usage of doxycycline for young children with Lyme disease

BackgroundThe 2018 Infectious Disease Committee of the American Academy of Pediatrics stated that up to 3 weeks or less of doxycycline is safe in children of all ages. Our goal was to examine trends in doxycycline treatment for children with Lyme disease.MethodsWe assembled a prospective cohort of children aged 1 to 21 years with Lyme disease who presented to one of eight participating Pedi Lyme Net centers between 2015 and 2023. We defined a Lyme disease case with an erythema migrans (EM) lesion or positive two-tier Lyme disease serology categorized by stage: early-localized (single EM lesion), early-disseminated (multiple EM lesions, cranial neuropathy, meningitis, and carditis), and late (arthritis). We compared doxycycline treatment by age and disease stage and used logistic regression to examine treatment trends.ResultsOf the 1,154 children with Lyme disease, 94 (8.1%) had early-localized, 449 (38.9%) had early-disseminated, and 611 (53.0%) had late disease. Doxycycline treatment was more common for older children (83.3% ≥ 8 years vs. 47.1% < 8 years; p < 0.001) and with early-disseminated disease (77.2% early-disseminated vs. 52.1% early-localized or 62.1% late; p < 0.001). For children under 8 years, doxycycline use increased over the study period (6.9% 2015 to 67.9% 2023; odds ratio by year, 1.45; 95% confidence interval, 1.34–1.58).ConclusionYoung children with Lyme disease are frequently treated with doxycycline. Prospective studies are needed to confirm the safety and efficacy of doxycycline in children younger than 8 years, especially for those receiving courses longer than 3 weeks.

Blurry Vision and Multiple Cranial Neuropathies.

Blurry Vision and Multiple Cranial Neuropathies.

Exploring the use of ChatGPT in Reporting a Case of HIV, Syphilis, and Lyme Co-infection Presenting with Multiple Cranial Neuropathies (P5-7.003)

Exploring the use of ChatGPT in Reporting a Case of HIV, Syphilis, and Lyme Co-infection Presenting with Multiple Cranial Neuropathies (P5-7.003)

Outcomes of Treating a late case of Herpes Zoster Ophthalmicus masquerading as Orbital Cellulitis: A Case Report

Abstract
 Introduction : Herpes zoster ophthalmicus (HZO) refers to a reactivated varicella-zoster virus involving the V1 nerve division. Although rare, previous reports have mentioned HZO cases masquerading as orbital cellulitis (HZO-OC). This report depicts the use of acyclovir, corticosteroids and citicoline in our HZO-OC patient with treatment delay.
 Case Illustration : A healthy 66 year old man presented to our clinic 4 weeks after signs of orbital cellulitis followed by vesicular rash following the CN V(1) dermatome were felt which was untreated. The right palpebra was edematous and erythematous. Hazy cornea, mixed injection, grade IV reverse RAPD, diminished direct and consensual reflex and external ophthalmoplegia of the right eye were noted. The diagnosis of HZO-OC with multiple cranial neuropathies OD was established. Topical and symptomatic treatment were given resulting no improvement. Ptosis of the right palpebra became present. The patient was then given systemic acyclovir, methylprednisolone and citicoline. Ophthalmoplegia improved after 2 months and ptosis improved after 4 months.
 Discussion : The use of systemic acyclovir and corticosteroids in HZO-OC patients were given promptly in previous literature. Multiple cranial nerve in our patient was thought to occur due to treatment delay. As improvement was not noted after topical treatment, these systemic treatments along with citicoline were added. Citicoline was added due to its ability to promote neural regeneration.
 Conclusion : Systemic acyclovir, corticosteroids and citicoline may still be beneficial for HZO-OC patients with delayed treatment.

Paget’s disease presenting with multiple cranial neuropathies

Paget’s disease presenting with multiple cranial neuropathies

VZV infection causing multiple cranial neuropathies

VZV infection causing multiple cranial neuropathies

Neurosyphilis presenting with Guillain–Barre syndrome: a case report

BackgroundSyphilis is associated with a wide variety of systemic presentations, earning it the moniker “The great mimicker”. Neurosyphilis is classically associated with meningovasculitis in the acute-subacute stage and tabes dorsalis and dementia paralytica in later stages. However, one of the less well described presentations include Guillain–Barre Syndrome. This case presents a patient with an ascending polyneuropathy suspicious for Guillain–Barre Syndrome who also had other atypical findings including a truncal sensory loss, optic disc swelling, and rash ultimately found to have neurosyphilis. Electrodiagnostic testing was consistent with demyelination, supporting a diagnosis of neurosyphilis associated Guillain–Barre Syndrome.Case presentationA 37-year-old female presented to the emergency department with a weakness and difficulty swallowing. She described a three-month history of symptoms, initially starting with a persistent headache followed by one month of a pruritic rash on her chest, palms, and soles. Two weeks prior to presentation, she developed progressive weakness in her arms, numbness in her arms and chest, and difficulty swallowing. Neurological exam was notable for multiple cranial neuropathies, distal predominant weakness in all extremities, length-dependent sensory loss, and hyporeflexia. Investigation revealed a positive Venereal Disease Research Laboratory in her cerebrospinal fluid without significant pleocytosis, contrast enhancement in cranial nerves V, VII, and VIII on MRI, and a demyelinating polyneuropathy on electrodiagnostic testing. She was diagnosed with Guillain–Barre syndrome, secondary to neurosyphilis. The patient acutely declined and required intubation, and ultimately made a full recovery after treatment with plasmapheresis and penicillin.ConclusionsThis case describes a clinical entity of syphilitic Guillain–Barre Syndrome and highlights the importance of including syphilis in the differential of any patient presenting with ascending polyradiculopathy, especially given the resurgence of syphilis.

Multi-Disciplinary Approach to Skull Base Paragangliomas.

The treatment of skull base paragangliomas has moved towards the use of cranial nerve preservation strategies, using radiation therapy and subtotal resection in instances when aiming for gross total resection would be expected to cause increased morbidity compared to the natural history of the tumor itself. The goal of this study was to analyze the role of surgery in patients with skull base paragangliomas treated with CyberKnife stereotactic radiosurgery (SRS) for definitive tumor control. A retrospective review identified 22 patients (median age 65.5 years, 50% female) treated with SRS from 2010-2022. Fourteen patients (63.6%) underwent microsurgical resection. Gross total resection was performed in four patients for tympanic paraganglioma (n = 2), contralateral paraganglioma (n = 1), and intracranial tumor with multiple cranial neuropathies (n = 1). Partial/subtotal resection was performed for the treatment of pulsatile tinnitus and conductive hearing loss (n = 6), chronic otitis and otorrhea (n = 2), intracranial extension (n = 1), or episodic vertigo due to perilymphatic fistula (n = 1). Eighteen patients had clinical and imaging follow-up for a mean (SD) of 4.5 (3.4) years after SRS, with all patients having clinical and radiological tumor control and no mortalities. Surgery remains an important component in the multidisciplinary treatment of skull base paraganglioma when considering other outcomes besides local tumor control.

Guillain-Barré Syndrome with multiple cranial neuropathies

Background. Guillain-Barré Syndrome (GBS) is an autoimmune reaction against peripheral nerves that manifests clinically as acute polyradiculoneuropathy. Classic sensory-motor, pure motor, paraparesis, pharyngeal-cervical-brachial, Bickerstaff brainstem encephalitis, pure sensory, bilateral facial palsy with paresthesia, and Miller-Fisher syndrome are all known variants of GBS. This case report seeks to describe a case of GBS with involvement of the cranial nerves. Case report. A 39-year-old right-handed Balinese woman presented with LMN (Lower Motor Neuron) type paraparesis with ascending paralysis and involvement of cranial nerves III through XII. The results of the LCS (Liquor Cerebro-Spinal) demonstrate albuminocytological dissociation. According to the patient’s EMNG (electromyoneurography) findings, Guillain-Barré Syndrome was of the AMAN (acute motor axonal neuropathy) variety. The patient was diagnosed with multiple cranial neuropathies and GBS of the AMAN type. EGRIS (Erasmus GBS Respiratory Insufficiency Score) was 3, and EGOS (Erasmus GBS Obstructive Sleep Apnea Score) was also 3. Following IVIG (Intravenous Immunoglobulin) treatment, the patient exhibited clinical improvement (Hughes score decreased from 4 to 2). Discussion. The patient exhibits clinical manifestations of flaccid tetraparesis and multiple cranial nerve paresis. The clinical condition of the patient did not meet the criteria for GBS clinical variation. Symptoms of flaccid tetraparesis that do not involve the senses are classified as the motor-only variant. This patient’s differential diagnosis includes the possibility of MFS and ophthalmoplegia. This patient, however, had flaccid tetraparesis and no ataxia. Electromyoneurography (EMNG) examination produces AMAN-type GBS. The patient was diagnosed with one of the clinical variants of GBS, AMAN-type GBS with multiple cranial neuropathies. Conclusion. A case of GBS of the AMAN type accompanied by multiple cranial neuropathies has been reported.

Multiple cranial neuropathy due to varicella zoster virus reactivation without vesicular rash: a challenging diagnosis.

Ramsay Hunt syndrome is due to reactivation of varicella zoster virus (VZV) dormant in the geniculate ganglion of the facial nerve. The diagnosis is typically based on clinical triad of ipsilateral facial paralysis, otalgia, and vesicles in the auditory canal or the auricle. However, Ramsay Hunt syndrome may occur without skin eruption in up to onethird of patients. Moreover, the involvement of other cranial nerves in addition to the facial nerve has been also reported. Herein, we reported a case report of a man who developed a multiple cranial neuropathy caused by VZV reactivation without skin vesicular eruption. The present case underlines a possible diagnostic challenge that clinicians may hit when facing a common disorder such as peripheral facial palsy. Indeed, clinicians must be aware that Ramsay Hunt syndrome may develop without skin vesicular eruption as well it may be complicated by multiple cranial nerve involvement. Antiviral therapy is effective in VZV reactivation for recovery of nerve function.

Multiple Cranial Neuropathies with Orbital Myositis as an Initial Presentation of Lyme Disease (P9-10.002)

<h3>Objective:</h3> To report a case of Lyme disease presenting with subacute multiple cranial neuropathies and orbital myositis. <h3>Background:</h3> Lyme disease can present with multiple cranial neuropathies. Orbital myositis is not well described as one of the rare complications of Lyme disease. Here, we describe a patient with multiple cranial neuropathies and orbital myositis as an initial manifestation of Lyme disease. <h3>Design/Methods:</h3> A 69-year-old male with a history of hyperthyroidism status post thyroidectomy, presented with a new onset headache, double vision, numbness of the face, and dysphagia. On examination, he had left lower motor neuron facial weakness, left eye proptosis, and findings suggestive of III and VI nerve palsies. Brain MRI with contrast revealed abnormal enhancement along the left V2 and V3 nerves and enhancement of the bilateral III cranial nerve. Abnormal enhancement was also seen at the left orbital apex in addition to enhancement along the left retro-orbital optic nerve. MRI of the orbit showed asymmetric enlargement and subtle enhancement of the left extra orbital muscles with mild orbital fat infiltration, compatible with orbital myositis. Lyme IgG/IgM antibody screen was positive which was confirmed by Lyme immunoblot assay which was also positive for Lyme IgG (5 bands) and IgM (3 bands) antibodies. B2 Glycoprotein IgM was elevated. Cerebrospinal fluid (CSF) analysis revealed lymphocytic pleocytosis and elevated protein. CSF cytology and flow cytometry were negative for malignant cells. TSH, thyroxin, and anti-thyroid peroxidase antibodies were unremarkable. Sarcoidosis, IgG4-related disease, autoimmune, and infectious workups were unrevealing. The patient received IV ceftriaxone with significant improvement and with near resolution of the symptoms and clinical findings after 1 month. <h3>Results:</h3> NA <h3>Conclusions:</h3> Orbital myositis is a rare complication of Lyme disease. <b>Disclosure:</b> Dr. Biso has nothing to disclose. Dr. Philip has nothing to disclose. Dr. Chevula has nothing to disclose. Dr. El-Dokla has nothing to disclose.

E025 A complex case of giant cell arteritis presenting with visual loss, tongue and scalp infarction and multiple cranial neuropathies

Abstract Background/Aims Giant cell arteritis (GCA) is the most common of the vasculitides. Visual loss due to vascular occlusion is a well recognised complication of GCA. It is important to remember that other ischaemic complications can occur, leading to atypical clinical features. We present a case of a patient with GCA who did not complain of headaches, but presented with at least five cranial neuropathies, including a right optic neuropathy, ischaemic ulceration of her tongue and a large area of scalp necrosis. Methods We present the results of our patient case review and literature search. Results An 82-year-old retired landlady who was an ex-smoker presented with haemoptysis and was diagnosed with non-small-cell lung cancer. She was treated with chemotherapy and radiotherapy in 2020, then had possible disease progression on CT thorax in 2021. She developed thigh pain, swallowing difficulties and decreased oral intake in 2021, and subsequently developed severe oral ulceration, dehydration and delirium and was admitted to the frailty unit. On examination she had a very large scalp lesion, oral lesions, and multiple cranial neuropathies (right optic neuropathy, right partial third nerve, right seventh nerve, recurrent laryngeal, and bilateral twelfth nerve palsies). Her anterior ischemic optic neuropathy meant she could only see hand movements with the right eye. Blood tests revealed anaemia of chronic disease and a CRP of over 200 mg/L. Ultrasound scan of the temporal arteries showed a halo sign confirming the diagnosis of GCA. CT angiography showed vertebral artery wall thickening and stenosis. She was treated with intravenous methylprednisolone followed by high dose oral prednisolone, and started on methotrexate. She improved symptomatically. The cranial neuropathies resolved and the area of scalp necrosis and ischemic tongue ulceration healed, but vision was not regained. Her inflammatory markers never normalised and she was subsequently started on tocilizumab, with the approval of her oncologist. Her ESR and CRP quickly fell to normal. It remains to be seen how biologic treatment may impact her malignancy. Conclusion This case highlights multiple atypical presenting features of GCA and the need for a high index of suspicion to treat early and avoid irreversible visual loss. A review of the literature reveals that cranial neuropathies secondary to ischemia can be the presenting feature in GCA and multiple cranial neuropathies can occur. This is the first case in the literature, that we are aware of, which involves this number of cranial neuropathies alongside extensive scalp and tongue infarction. These features have all resolved with treatment, with the exception of unilateral permanent visual loss. Disclosure J. Jade: None. V. Barradell: None. A. Coote: None.

Electrodiagnosis of Multiple Cranial Neuropathies and Lumbosacral Polyradiculopathy in Tuberculous Meningitis: A Case Report

Cranial neuropathy and radiculopathy are common complications of tuberculous meningitis. However, the co-occurrence of these complications and the corresponding electrodiagnostic findings have rarely been reported. We report a patient diagnosed with tuberculous meningitis who presented with difficulty in smelling, facial paralysis, hearing loss, and severe weakness in the bilateral lower extremities. A neurological examination confirmed impaired function of the olfactory nerves. Electrodiagnostic studies, including a nerve conduction study, electromyography, and brainstem auditory evoked potential, revealed multiple cranial neuropathies involving the left facial nerve and bilateral vestibulocochlear nerves, as well as bilateral lumbosacral polyradiculopathies. In patients with tuberculous meningitis, multiple cranial neuropathies and polyradiculopathies can occur simultaneously as complications, and electrodiagnostic studies can enable an accurate diagnosis of these complications and an assessment of their severity.

A Case Study on Tubercular Cavernous Sinus Syndrome

Tubercular cavernous sinus syndrome is a rare condition of extra-pulmonary tuberculosis EPTB in which cavernous sinus involvement is observed. Cavernous sinus is a venous structure that holds and protects pituitary gland on one side and temporal lobe on other side. CSS is mainly characterized by multiple cranial neuropathies which is effected by various etiologies. This is a case report of tubercular cavernous sinus syndrome admitted to hospital with chief complaints of fever headache drooping of eyelids altered sensorium with disorientation and fever that reduced over next 3 to 4 days but headache was still persisting due to the damage to the cranial nerves. Based on these symptoms they underwent clinical examination which revealed hypotension elevated CRP levels and MRI suggestive of acute dural venous sinus thrombosis involving superior sagittal sinus and bilateral bulky cavernous sinus with transverse T2 hypo intensity in right transverse sagittal superior sagittal and internal jugular vein IJV. Granulomatous etiology indicated tubercular cavernous sinus syndrome. For this treatment was initiated with anti-tubercular drug therapy with vitamin supplements and anticoagulants. EPTB remains a significant health problem in developing countries. Prevalence of EPTB is increasing over the last several years globally.

Neurological manifestations in patients and disease carriers in an Italian family with osteosclerosis.

Hereditary cranial hyperostosis is a rare disease never described in Italy, so the neurological manifestations in patients and carriers of the disease have been little studied. We describe the neurological and neuroimaging features of patients and carriers of the gene from a large Italian family with sclerosteosis. In this family, genetic testing detected the homozygous p.Gln24X (c.70C > T) mutation of the SOST gene in the proband and a heterozygous mutation in 9 siblings. In homozygous adults, severe craniofacial hyperostosis was manifested by cranial neuropathy in childhood, chronic headache secondary to intracranial hypertension, and an obstructive sleep apnea syndrome in adults. In one of the adult patients, there was a compressible subcutaneous swelling in the occipital region caused by transosseous intracranial-extracranial occipital venous drainage, a compensation mechanism of obstructed venous drainage secondary to cranial hyperostosis. Mild cranial hyperostosis causing frequent headache and snoring was evident in the nine heterozygous subjects. Multiple cranial neuropathies and headache in children, while severe chronic headache and sleep disturbances in adults, are the neurological manifestations of the first Italian family with osteosclerosis. It is reasonable to extend neurological and neuroimaging evaluation to gene carriers as well.

Labyrinthitis With Negative MRI As a Precursor to Rapidly Developing Primary CNS Lymphoma of the Cerebellopontine Angle.

Few case reports have described primary central nervous system lymphoma (PCNSL) presenting as a cerebellopontine angle (CPA) lesion in HIV-positive patients. We describe a rare presentation of rapidly progressing PCNSL of the CPA/internal auditory canal (IAC) as labyrinthitis with initial negative MRI in an HIV-positive patient. A 58-year-old male with well-controlled HIV presented with sudden left sensorineural hearing loss, tinnitus, and imbalance. Vestibular testing suggested an uncompensated left peripheral vestibular weakness. MRI demonstrated facial and cochleovestibular nerve enhancement within the CPA and IAC. The presumptive diagnosis of labyrinthitis was made. Two months later, he presented to his infectious disease provider with left facial weakness and disequilibrium and was treated for presumed Bell's palsy. One month later, he presented with left corneal reflex dysfunction, decreased visual acuity, diplopia, and worsening ataxia. Repeat MRI demonstrated a new 3.6 cm lesion of the left CPA/IAC with vasogenic edema. Despite location, the mass lacked the brainstem compression characteristic of other extra-axial CPA masses such as vestibular schwannoma. Flow cytometry and cytology from cerebrospinal fluid was consistent with primary central nervous system large B-cell lymphoma. We present a unique case of rapidly progressing PCNSL of the CPA/IAC in an HIV-positive patient, presenting initially as labyrinthitis with negative MRI followed by development of multiple cranial neuropathies and 3-month repeat MRI demonstrating a large CPA mass. In HIV-positive patients with a similar initial presentation, PCNSL should considered early in the diagnostic evaluation with close clinical monitoring and a low threshold for repeat imaging.

Multiple Cranial Neuropathies Following Zoster Vaccination

Multiple Cranial Neuropathies Following Zoster Vaccination

PMON42 A unique case of Norepinephrine secreting Head and Neck Paraganglioma

Abstract Introduction Head and neck Paragangliomas (HNPGLs) are rare, slow growing neuroendocrine tumors arising from parasympathetic and sympathetic paraganglia in the head and neck region. Traditionally, HNPGLs were presumed to be rarely secretory compared to thoracoabdominal sympathetic PGLs(&amp;lt;5%) and most of them are thought to be identified due to compressive symptoms or incidentally on imaging performed for screening in genetically predisposed individuals. However, most recent evidence has suggested that &amp;gt;10% of HNPGLs can be secretory although data has also shown that typically catecholamines. Case Presentation A 55-year-old man presented to the emergency room with worsening headaches, right sided neck pain and dizziness. Physical examination revealed multiple cranial neuropathies including right facial weakness with ipsilateral lagophthalmos, tongue atrophy, dystonic and atrophic sternocleidomastoid muscle, and vagal deficit. Blood pressure was markedly elevated at 227/108 mmHg. Computed tomography scan of head and neck revealed a large bony destructive lesion involving the right skull base measuring 9.0×7.0 cm, with involvement cervical vertebral bodies, as well as ventriculomegaly suggestive of obstructive hydrocephalus with mass effect on the brain stem and cerebellum. History revealed knowledge of this skull base mass for 13 years. Initially the mass was 3.2 cm in the largest dimension with elevated 24-hour urine normetanephrine 1113 mcg/day, Ref: 50-650 mcg/day and dopamine 667 mcg/day Ref: 60-440 mcg/day. He didn't seek further medical care at that time due to his family's opposition to traditional medicine. Magnetic resonance imaging upon this admission revealed 9.0×6.0×5.5 cm expansile, hypervascular, destructive mass at the right skull base with pronounced mass effect and brainstem compression with significant narrowing at the foramen magnum. 24-hour urine normetanephrines were significantly elevated at 4229 mcg/day, ref: 114-865 mcg/day, as well as elevated plasma Normetanephrine at 11.58 nmol/L, ref: 0.00-0.89 nmol/L, and urine Dopamine 10,071 mcg/day, ref: 71-485 mcg/day. Antihypertensive treatment was initiated with alpha blockade followed by additional beta blockade. After multidisciplinary discussion, the decision was made to pursue external radiation therapy given the considerable risk associated with the surgery due to location and vascularity. Genetic testing is pending at the time of abstract submission. Conclusion Recent evidence has suggested that &amp;gt;10% HNPGLs can be secretory as opposed to previous assumption that they are rarely secretory. Norepinephrine is the most common catecholamine secreted by HNPGL and dopamine is very rare. We present a unique case of HNPGL when surgery was deferred for more than a decade due to opposition to traditional medicine resulting in a large norepinephrine and dopamine secreting tumor (10 times the upper limit of normal) and multiple compressive cranial neuropathies. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Optic neuritis and multiple cranial neuropathies in patient with chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic recurrent acquired immune-mediated disease of the peripheral nerves that presents with progressive sensory and motor deficits in all four limbs. Cranial nerve involvement is not as common as in Guillain-Barre syndrome, and central nervous system involvement including optic neuritis has rarely been reported in patients with CIDP. We recently experienced a case with classic CIDP involving bilateral facial and trigeminal nerves, right lower cranial nerves, and the right optic nerve.