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Background Subacute oculomotor dysfunction encompasses a broad differential diagnosis, including autoimmune, infectious, vascular, and neoplastic etiologies. Primary skull base diffuse large B‐cell lymphoma (DLBCL) is a rare but treatable cause of cranial nerve dysfunction and may present subtly on conventional neuroimaging. Methods We report an 80‐year‐old man with subacute diplopia, dysfunction of multiple cranial nerves, left‐sided ptosis, and headache. Diagnostic workup included cranial imaging, cerebrospinal fluid (CSF) analysis with cytology and flow cytometry, neurophysiological studies, serum diagnostics, and dedicated MRI of the skull base. Transsphenoidal biopsy was performed following identification of an infiltrative lesion. Results Initial investigations including cranial CT, CSF analysis, and serum diagnostics were unremarkable. Dedicated skull base MRI revealed a diffuse infiltrating mass involving the clivus, petrous bone, occipital condyles, and perisellar/posterior pituitary region, with anatomical correlation to multiple cranial nerves. Mildly elevated prolactin levels suggested a stalk effect. Histological analysis confirmed DLBCL (NOS, GCB type). Treatment with corticosteroids followed by dose‐adjusted R‐CHOP resulted in complete clinical remission and radiologically confirmed tumor regression. Conclusions This case illustrates a rare presentation of primary bony skull base DLBCL with perisellar infiltration, highlighting the diagnostic challenges of complex oculomotor dysfunction. Dedicated skull base MRI is essential in the workup of unexplained cranial neuropathy. CSF‐based molecular markers may complement the diagnostic approach in similar presentations.

AimGuillain-Barré syndrome is a general term used to describe acute immune-mediated polyradiculoneuropathies. It’s one of the most common causes of acute, acquired muscle weakness. Polyneuritis cranialis is believed to be an interface between Guillain-Barré and Miller-Fischer syndromes.Material and Method.ResultsEight-year-old male patient with autism spectrum disorder presented with drooling, difficulty swallowing, slurred speech, inability to move facial muscles, drooping eyelids and unsteady gait. There was no history of fever, respiratory or gastrointestinal infection, travel, trauma, or toxic exposure. Except for consanguineous marriage between parents, the past medical and family history was unremarkable. Physical examination revealed normal vital signs, mid-dilated pupils bilaterally reactive to light, multiple cranial nerve involvement including cranial nerves II, IV, VI, VII, IX, X, XI, XII, mild upper and lower extremity weakness and ataxia. Atypical variant of Guillain-Barré syndrome was considered as a preliminary diagnosis, but differential diagnoses included brainstem lesions, myasthenic crisis, botulismus and toxic/metabolic diseases. Complete blood count, biochemistry, metabolic screenings were normal. Cranial MRI was normal, contrast weighted spinal MRI revealed enhancement of conus medullaris and cauda equina roots(figure 1). Test dose with pyridostigmine showed no improvement of symptoms excluding a myasthenic crisis. An electromyography(EMG) was performed subsequently, needle EMG revealed rare positive spikes and fibrillation in the left frontal muscle. Cerebrospinal fluid(CSF) was acellular and CSF protein was elevated (0.83 g/L, N: 0,15–0,45). All assessments indicated Guillain-Barré syndrome with multicranial neuropathy. Serum anti-ganglioside antibody test was positive for anti-GT1a, anti-GT1b, and anti-GQ1b. The patient was treated with 2 g/kg intravenous immunoglobulin. Although minor improvements were seen, because of severe bulbar involvement, 5 total plasma exchanges were done. Three months after the first presentation, the symptoms were resolved.Abstract PP-042 Figure 1Postcontrast T1 Saggital Spinal MRI. Contrast enhancement of conus medullaris and cauda equina roots.ConclusionsThe symptoms and severity of Guillain-Barré syndrome and it’s variants show diversity. It’s crucial to consider all forms when clinical suspicion exists, to avoid underdiagnosis and delayed treatment.

The Coronavirus disease 19 (COVID-19), caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is a threat to human health. Although the COVID-19 pandemic is finished, some peoples are still suffering from this disease. Herein, we report the first case of SARS-CoV-2-associated Guillain-Barré syndrome (GBS) presenting as polyneuritis cranialis (PNC) and acute panautonomic neuropathy (APN) variants, accompanied by mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) and hyponatremia. A 32-year-old female patient with symptoms indicating multiple cranial nerve involvement, as well as sympathetic and parasympathetic nervous system dysfunction, was diagnosed as SARS-CoV-2-associated GBS presenting as PNC and APN variants, accompanied by MERS and hyponatremia. Following treatment with immunoglobulin, methylprednisolone, and symptomatic care, the patient's inflammatory cytokines and serum sodium became normal. However, some residual symptoms such as postural hypotension, fatigue, and mild dysarthria persisted at the 9-month follow-up. This case highlights the unique presentation of SARS-CoV-2 infection. The involvement of both the central nervous system (CNS) and the peripheral nervous system (PNS) in this case underscores the complex neurological manifestations of COVID-19. Although the exact underlying pathogenesis of this case is unclear, inflammatory cytokines, particularly IL-6, may be implicated. Further research is needed to better understand the mechanisms underlying these complications and to optimize treatment strategies for affected patients.

Background Polyneuritis cranialis (PNC), a rare variant of Guillain-Barré syndrome (GBS), is usually characterized by ocular and pharyngeal weakness without obvious numbness or weakness of the limbs or ataxia. Horner’s syndrome is extremely rare in patients with PNC. Here, we describe a case of GBS presenting with acute PNC and unilateral Horner syndrome. Case presentation A 53-year-old male presented with headache, abducent paresis, peripheral-type facial palsy, bulbar type dysarthria, decreased gag reflex and tongue palsy. Neurological examination showed Cranial Nerve V, VI, VII, IX, X and XII were affected, and Horner’s syndrome was observed. Cerebrospinal fluid analysis showed albuminocytologic dissociation. Sensorimotor conduction velocity and needle electromyography of limbs were normal. Magnetic resonance imaging of brain was normal. Finally, the patient was diagnosed as PNC combined with Horner’s syndrome. The patient received plasma exchange and intravenous immunoglobulin, which relieved the symptoms rapidly. Conclusion GBS presenting only as Horner syndrome and PNC is a challenge for etiological diagnosis. Clinicians need to know enough to distinguish GBS and its variants from other potential similar diseases.

Background: Central or atypical skull base osteomyelitis (CSBO) often presents with severe unrelenting headache and progressive mono or polyneuritis cranialis. MRI and CT are used as initial imaging techniques but have a poor specificity and sensitivity. Objective: To analyze our cohort of CSBO. Materials and Methods: Over a 5-year period [2015-2020], we retrospectively analyzed the records of all patients with CSBO who had undergone a 3T MRI Brain, MR angiography, regional FDG PET-CT, and skeletal scintigraphy with 99mTc MDP/SPECT-CT. Surgical biopsy specimens were sent for bacterial and fungal cultures. Results: In total, 17 patients with CSBO were identified. Typically, 88% of patients presented with severe unilateral headache. All patients had at least a cranial mono or polyneuritis. The majority of patients were diabetic [64%]. MRI was normal in 42% of patients, whereas PET-CT and with 99mTc MDP scan and SPECT-CT were abnormal in all patients. Conclusion: Our series of CSBO showed a 40% mortality rate with significant morbidity and relentless progression. Patients required repeated PET CT and bone scans to detect regression of disease activity. The average duration of IV therapy ranged from 3 weeks to 9 months and oral therapy for around 2-3 months. Cure was defined after taking into account the original diagnosis, symptom resolution, and concordant reduction of tissue uptake on PET CT and 99mTc bone scan. The combination of MRI, FDG PET CT, and 99mTc bone scan with concurrent SPECT CT was able to detect disease and disease progression in all patients.

BackgroundPolyneuritis cranialis (PNC) with the disease characteristics of Guillain-Barré syndrome (GBS) in addition to both ocular and bulbar weakness in the absence of limb paralysis or ataxia is defined as an unusual variant of GBS. As evidence of central nervous system (CNS) involvement, visual impairment is an unusual finding complicating with GBS spectrum disorders and has never been reported in patients with PNC.MethodsWe describe a very rare case who clinically presented with progressive multiple cranial nerve palsy and visual impairment. Furthermore, a literature search of concurrent GBS and optic neuritis (ON) as well as PNC attributed to GBS was conducted.ResultsA diagnosis of PNC was considered due to the typical clinical characteristics as well as the presence of cerebrospinal fluid cytoalbumin dissociation and serum antibodies against gangliosides. The clinical manifestations and the bilateral optic nerve involvement in brain magnetic resonance imaging further suggested possible optic neuritis (ON). The patient received treatment with intravenous immunoglobulin followed by short-term use of corticosteroids and finally achieved a full recovery. Thirty-two previously reported cases (17 women, mean age 40) of concurrent GBS and ON and 20 cases of PNC (5 women, mean age 40) were analyzed. We further provided a comprehensive discussion on the potential etiologies, clinical features, therapeutic strategies, and prognosis.ConclusionsThis rare case with the co-occurrence of PNC and visual impairment and the related literature review may help clinicians advance the understanding of GBS spectrum disorders and make appropriate diagnoses and treatment decisions for the rare variants and CNS complications of GBS.

Background Polyneuritis cranialis is a rare condition than can be caused by various diseases. We report the first case of multiple cranial nerve paralysis resulting in neurotrophic keratopathy with corneal ulceration as a sequel of polyneuritis cranialis due to actinomycosis. Case presentation An 84-year-old German woman developed corneal ulceration with a high risk of perforation because of neurotrophic keratopathy due to polyneuritis cranialis. Actinomycosis of a wisdom tooth causing polyneuritis cranialis was detected on computed tomography and magnetic resonance imaging and was treated with systemic antibiotics. Penetrating keratoplasty with simultaneous amniotic membrane transplantation was performed successfully after conservative treatment failure. Conclusions To the best of our knowledge, we report on the first case of multiple cranial nerve failure due to actinomycosis and requiring ophthalmological intervention.

Background Multiple cranial neuropathies carry a wide range of differential diagnoses, and when combined with cerebrospinal fluid monocytosis they often suggest an infective etiology. Reactivation of varicella zoster virus has been associated with a wide range of neurological complications. Among the cranial nerves, the upper cranial nerves (trigeminal and facial nerves) are more commonly affected; there have been some reports of lower cranial polyneuropathies resulting from varicella zoster virus reactivation. However, polyneuropathy involving both the cranial and cervical nerves is rarely reported. Case presentation This report highlights the case of a 64-year-old Chinese man presenting with an acute, severe dysphagia and dysphonia secondary to herpes zoster-associated progressive polyneuropathy involving the lower cranial and upper cervical nerves, without any mucocutaneous manifestations. Conclusions To our knowledge, this is the first case of varicella zoster virus-associated cranial and cervical polyneuropathy in the literature. The report also highlights the poor sensitivity of varicella zoster virus DNA detection by polymerase chain reaction in the cerebrospinal fluid, and proposes that serology be routinely performed in such polymerase chain reaction-negative cases without a clear diagnosis.

A 16-year-old Thai girl developed right facial palsy, a lower motor neuron lesion, and numbness 3 h after receiving the first dose of the BNT162b2 mRNA vaccine. Neurological examination showed the involvement of the right cranial nerves (CN) V, VII, IX, and X. Electrophysiological tests revealed the absence of an F wave response, suggesting a proximal demyelinating process. Magnetic resonance imaging of the brain demonstrated abnormal enhancement of the right CN VII. The cerebrospinal fluid profile on day 7 after the onset of symptoms was normal. The patient was diagnosed with polyneuritis cranialis, a rare variant of Guillain-Barre syndrome. She was successfully treated with intravenous immunoglobulin therapy.