A Case with Anti-ganglioside Antibodies Showing Multiple Cranial Nerve Palsies Detected on Gadolinium-enhanced Magnetic Resonance Imaging.

The anti-GQ1b IgG antibody is often accompanied by other anti-ganglioside antibodies, which induces various neurological symptoms. We herein report a patient with anti-ganglioside antibodies, including anti-GQ1b IgG and anti-GT1a IgG antibodies, showing bilateral ophthalmoplegia, facial nerve palsies, dysarthria, dysphagia, dysesthesia in both hands, and enhancement of the bilateral oculomotor, abducens, and facial nerves on gadolinium (Gd)-enhanced T1-weighted brain magnetic resonance imaging (MRI). He was first treated with intravenous immunoglobulin, which improved ophthalmoplegia, bulbar palsies, and dysesthesia of hands, but the facial nerve palsies worsened, and Gd enhancement of the brain nerves persisted. High-dose methylprednisolone therapy subsequently improved the facial nerve palsies and Gd enhancement of the cranial nerves. This is the first case with anti-ganglioside antibodies presenting with multiple cranial nerve palsies that was followed to track the changes in the Gd enhancement of cranial nerves on MRI.

Internal Carotid Artery Aneurysm Presenting as Lower Cranial Nerve Palsies.

The signs of lower cranial nerve palsies are rare and are often caused by tumors. A 49-year-old woman was admitted to our hospital with progressive right-sided atrophy of the tongue, sternocleidomastoid and trapezius, dysarthria, and dysphagia for 3 years. Brain magnetic resonance imaging revealed a circular lesion adjacent to the lower cranial nerves. Cerebral angiography confirmed that the lesion was an unruptured aneurysm in the C1 segment of the right internal carotid artery. After endovascular treatment, the symptoms of this patient had partially improved.

ACR Appropriateness Criteria® Cranial Neuropathy: 2022 Update.

Cranial neuropathy can result from pathology affecting the nerve fibers at any point and requires imaging of the entire course of the nerve from its nucleus to the end organ in order to identify a cause. MRI with and without intravenous contrast is often the modality of choice with CT playing a complementary role. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer-reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances in which peer-reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

Imaging of Cranial Neuralgias.

Cranial neuralgia (CN) can cause significant debilitating pain within a nerve dermatome. Accurate diagnosis requires detailed clinical history and examination, understanding of pathophysiology and appropriate neuroimaging to develop an optimal treatment plan. The objective of this article is to review and discuss some of the more common CNs including trigeminal neuralgia and its associated painful neuropathies, occipital neuralgia, and less common glossopharyngeal neuralgia (GPN). The neuroanatomy, pathophysiology, diagnostic imaging, and treatment of each of these pathologies are reviewed with emphasis on the role of CT and MR imaging findings in guiding diagnosis. Although CT is often used to initially identify an underlying cause such as neoplasm, infection, or vascular malformation, MRI is optimal. Clinical history and examination findings along with MRI constructive interference steady state/fast imaging employing steady-state acquisition sequences and MRA of the brain can be used to distinguish between primary and secondary cranial neuropathies and to discern the best treatment option. Pharmacologic and noninvasive therapy is the first-line of treatment of these cranial and cervical neuralgias. If symptoms persist, stereotactic radiosurgery is an option for some patients, although microvascular decompression surgery is the most curative option for both trigeminal and GPN. Refractory occipital neuralgia can be treated with a nerve block, an ablative procedure such as neurectomy or ganglionectomy, or more recently occipital nerve stimulation.

Enhancement of cranial nerves in Lyme neuroborreliosis: incidence and correlation with clinical symptoms and prognosis.

PURPOSE: Symptoms of cranial neuritis are a common presentation of Lyme neuroborreliosis (LNB). Imaging studies are scarce and report contradictory low prevalence of enhancement compared to clinical studies of cranial neuropathy. We hypothesized that MRI enhancement of cranial nerves in LNB is underreported, and aimed to assess the prevalence and clinical impact of cranial nerve enhancement in early LNB. METHODS: In this prospective, longitudinal cohort study, 69 patients with acute LNB were examined with MRI of the brain. Enhancement of cranial nerves III-XII was rated. MRI enhancement was correlated to clinical findings of neuropathy in the acute phase and after 6 months. RESULTS: Thirty-nine of 69 patients (57%) had pathological cranial nerve enhancement. Facial and oculomotor nerves were most frequently affected. There was a strong correlation between enhancement in the distal internal auditory canal and parotid segments of the facial nerve and degree of facial palsy (gamma = 0.95, p < .01, and gamma = 0.93, p < .01), despite that 19/37 nerves with mild-moderate enhancement in the distal internal auditory canal segment showed no clinically evident palsy. Oculomotor and abducens nerve enhancement did not correlate with eye movement palsy (gamma = 1.00 and 0.97, p = .31 for both). Sixteen of 17 patients with oculomotor and/or abducens nerve enhancement had no evident eye movement palsy. CONCLUSIONS: MRI cranial nerve enhancement is common in LNB patients, but it can be clinically occult. Facial and oculomotor nerves are most often affected. Enhancement of the facial nerve distal internal auditory canal and parotid segments correlate with degree of facial palsy.

Improved Lesion Conspicuity with Contrast-Enhanced 3D T1 TSE Black-Blood Imaging in Cranial Neuritis: A Comparative Study of Contrast-Enhanced 3D T1 TSE, 3D T1 Fast-Spoiled Gradient Echo, and 3D T2 FLAIR.

BACKGROUND AND PURPOSE: Contrast-enhanced 3D-turbo spin-echo (TSE) black-blood sequence has gained attention, as it suppresses signals from vessels and provides an increased contrast-noise ratio. The purpose was to investigate which among the contrast-enhanced 3D T1 TSE, 3D T1 fast-spoiled gradient echo (FSPGR), and 3D T2 FLAIR sequences can better detect cranial nerve contrast enhancement. MATERIALS AND METHODS: Patients with cranial neuritis based on clinical findings (n = 20) and control participants (n = 20) were retrospectively included in this study. All patients underwent 3T MR imaging with contrast-enhanced 3D T1 TSE, 3D T1 FSPGR, and 3D T2 FLAIR. Experienced and inexperienced reviewers independently evaluated the 3 sequences to compare their diagnostic performance and time required to reach the diagnosis. Additionally, tube phantoms containing varying concentrations of gadobutrol solution were scanned using the 3 sequences. RESULTS: For the inexperienced reader, the 3D T1 TSE sequence showed significantly higher sensitivity (80% versus 50%, P = .049; 80% versus 55%; P = .040), specificity (100% versus 65%, P = .004; 100% versus 60%; P = .001), and accuracy (90% versus 57.5%, P = .001; 90% versus 57.5%, P = .001) than the 3D T1 FSPGR and 3D T2 FLAIR sequences in patients with cranial neuritis. For the experienced reader, the 3D T1-based sequences showed significantly higher sensitivity than the 3D T2 FLAIR sequence (85% versus 30%, P < .001; 3D T1 TSE versus 3D T2 FLAIR, 85% versus 30%, P < .001; 3D T1 FSPGR versus 3D T2 FLAIR). For both readers, the 3D T1 TSE sequence showed the highest area under the curve (inexperienced reader; 0.91, experienced reader; 0.87), and time to diagnosis was significantly shorter with 3D T1 TSE than with 3D T1 FSPGR. CONCLUSIONS: The 3D T1 TSE sequence may be clinically useful in evaluating abnormal cranial nerve enhancement, especially for inexperienced readers.

Magnetic resonance imaging features of COVID-19-related cranial nerve lesions.

The complete features of the neurological complications of coronavirus disease 2019 (COVID-19) still need to be elucidated, including associated cranial nerve involvement. In the present study we describe cranial nerve lesions seen in magnetic resonance imaging (MRI) of six cases of confirmed COVID-19, involving the olfactory bulb, optic nerve, abducens nerve, and facial nerve. Cranial nerve involvement was associated with COVID-19, but whether by direct viral invasion or autoimmunity needs to be clarified. The development of neurological symptoms after initial respiratory symptoms and the absence of the virus in the cerebrospinal fluid (CSF) suggest the possibility of autoimmunity.

Eight-and-a-half syndrome: video evidence and updated literature review.

The eight-and-a-half syndrome (EHS)-defined by the combination of a seventh cranial nerve palsy and an ipsilateral one-and-a-half syndrome-is a rare brainstem syndrome, which localises to the caudal tegmental region of the pons. We present a case of the EHS secondary to an inflammatory lesion on a previously healthy 26-year-old woman, with a literature review emphasising the relevance of aetiological assessment.

MRI Results of Patients with Acute Isolyted Cranial Nerve Palsies.

AIM: To investigate the magnetic resonance imaging (MRI) results of patients complaining from diplopia with ocular nerve palsy. MATERIAL AND METHODS: A routine ophthalmic examination was performed, a neurological consultation was requested, and cranial MRI was performed for all patients. The image results were sorted into four groups: ischemic lesions, demyelinating disease lesions, tumors, and no lesions. White matter gliosis and cerebral infarcts were included in the ischemic lesion group. The medical histories of the patients were acquired from medical records. The chi-squared test was used to analyze the relationship between age and cranial MRI images and to analyze the relationship between the image and paresis type. The statistical significance threshold was set at p < 0.05, unless otherwise stated. RESULTS: Ischemic MRI images were the most common image type seen in our study. Third nerve paresis was significantly correlated with ischemic cerebral lesions observed by MRI (p=0.009). Furthermore, lesions were significantly correlated with patients aged above 50 years (p=0.004). There were no significant correlations between fourth or sixth nerve paresis and cranial ischemic images (p=0.680 and p=0.678, respectively). There were two instances of cerebral artery aneurysm, three instances of cerebral infarct, and one instance of intracranial mass, all in patients aged over 50 years. CONCLUSION: Although our patients had minimal or nonexistent neurological symptoms, some had serious cranial pathologies. These pathologies were commonly seen in patients aged over 50 years. We recommend performing MRI on all patients with binocular diplopia.

Cranial Nerve Imaging and Pathology.

This review provides a symptom-driven approach to neuroimaging of disease processes affecting the cranial nerves. In addition to describing characteristic imaging appearances of a disease, the authors emphasize exceptions to the rules and neuroimaging pearls. The focus is on adult neurology although some important pediatric conditions are included. On reviewing this material, the reader should be able to (1) differentiate intra- and extra-axial causes of cranial nerve dysfunction and (2) appropriately use neuroimaging to investigate abnormalities of cranial nerve function.

A long styloid process and Collet-Sicard syndrome / Proceso estiloides alargado y síndrome de Collet-Sicard

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Skull Base-related Lesions at Routine Head CT from the Emergency Department: Pearls, Pitfalls, and Lessons Learned.

Routine non-contrast material-enhanced head CT is one of the most frequently ordered studies in the emergency department. Skull base-related pathologic entities, often depicted on the first or last images of a routine head CT study, can be easily overlooked in the emergency setting if not incorporated in the interpreting radiologist's search pattern, as the findings can be incompletely imaged. Delayed diagnosis, misdiagnosis, or lack of recognition of skull base pathologic entities can negatively impact patient care. This article reviews and illustrates the essential skull base anatomy and common blind spots that are important to radiologists who interpret nonenhanced head CT images in the acute setting. The imaging characteristics of important "do not miss" lesions are emphasized and categorized by their cause and location within the skull base, and the potential differential diagnoses are discussed. An interpretation checklist to improve diagnostic accuracy is provided. ©RSNA, 2019.

Enfermedad diseminada por arañazo de gato: el amplio espectro de la presentación / Disseminated cat scratch disease: The wide variety of clinical presentations

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Newly occurring cranial nerve palsy after endovascular treatment of cavernous sinus dural arteriovenous fistulas.

OBJECTIVE: Cranial nerve palsy (CNP) is rarely reported after endovascular intervention for cavernous sinus dural arteriovenous fistulas (CS DAVFs). Our aim was to evaluate the treatment outcomes of CS DAVFs, and the clinical course and risk factors of newly occurring CNPs, with a review of the literature. METHODS: 121 patients with 134 lesions treated in our institution were selected. They were retrospectively analyzed in terms of baseline characteristics, radiologic results, clinical outcome, and newly occurring CNPs after treatment. Angiographic and clinical follow-up data were available for 104 lesions (77.6%) and 130 lesions (97.0%), respectively. RESULTS: The angiographic results showed partial:complete obliterations in 13:91 (87.5%) at the final follow-up. Clinical outcomes were deteriorated or no change:improved or completely recovered (3:126 (96.9%)) at the final follow-up. New CNPs occurred in 24 patients (19.8%), including the sixth CNP alone or mixed in 23 patients (95.8%). 23 cases were completely recovered or improved (21 and 2 cases, respectively), and 19 (90.4%) of 21 were completely recovered within 6 months after treatment. In the multivariate analysis, pretreatment cranial nerve symptoms (OR=0.33; 95% CI 0.14 to 0.76; P=0.010) and complete obliteration immediately after treatment (OR=0.32; 95% CI 0.12 to 0.85; P=0.021) were significant preventive factors for new CNPs. Embolization in the posterior compartments of the CS (OR=5.15; 95% CI 1.71 to 15.47; P=0.004) was a significant risk factor. CONCLUSIONS: Endovascular intervention was satisfactory in patients with CS DAVFs. The prognosis of newly occurring CNPs was favorable.

Longitudinal Morphometric Analysis of Sub-Basal Nerve Plexus in Contralateral Eyes of Patients with Unilateral Neurotrophic Keratitis.

Objectives: To investigate longitudinally corneal sub-basal nerve plexus (SNP) by means of in vivo confocal microscopy (IVCM) in the contralateral eye (CE) of patients with unilateral neurotrophic keratitis (NK) secondary to central nervous system (CNS) diseases who underwent different treatments. Methods: Ten patients with NK and 10 matched controls were included. In 7 NK patients, conservative treatment maintained unchanged the clinical picture over the 1-year follow-up (Group 1), while NK progressed in 3 patients who underwent direct corneal neurotization (Group 2). IVCM scans of SNP of NK patients were acquired in CE at baseline (V0) ad after 1-year follow-up (V1). All images were analyzed with the automated software "ACCMetrics" and compared with controls. The following IVCM corneal nerve parameters were calculated at V0 and V1 with ACCMetrics: fiber density (CNFD), branch density (CNBD), fiber length (CNFL), total branch density (CTBD), fiber area (CNFA), fiber width (CNFW), and fractal dimension (CNFrD). Results: At V0, significantly lower mean values of CNFD and CNBD, and higher values of CNFW were detected in CE of NK patients compared to controls (respectively, 16.9 ± 8.7 vs 25.0 ± 8.3 n/mm2, P= .029; 19.3 ± 13.8 vs 33.8 ± 18.9 n/mm2, P= .023; 0.022 ± 0.002 vs 0.020 ± 0.001 mm/mm2, P< .001). From V0 to V1, all IVCM metrics of CE remained unchanged in Group 1, while they improved in Group 2. Conclusions: Contralateral eye of patients with unilateral NK secondary to CNS disease showed lower CNFD and CNBD and higher CNFW compared to controls. Unlike conservative treatment, direct corneal neurotization was able to improve SNP metrics also in CE.