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.

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.

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.

Congenital Cranial Dysinnervation Disorder: An Unusual Phenotype With Multiple Cranial Neuropathies and Novel Neuroimaging Findings.

Congenital cranial dysinnervation disorders result from a maldevelopment of brainstem nuclei and/or cranial nerves. In some cases, specific genetic abnormalities have been identified. We expand the clinical phenotype of these disorders with the report of a 28-month-old girl who was initially evaluated for seizures and was found to have right sixth nerve palsy, small optic discs with reduced vision in her right eye. Her development was delayed. Brain MRI showed multiple abnormalities involving other cranial nerves, the optic chiasm and brainstem. Her developmental delay and seizure disorder suggest additional cortical involvement.

Vernet syndrome resulting from varicella zoster virus infection-a very rare clinical presentation of a common viral infection.

Vernet syndrome is a unilateral palsy of glossopharyngeal, vagus, and accessory nerves. Varicella zoster virus (VZV) infection has rarely been described as a possible cause. A 76-year-old man presented with 1-week-long symptoms of dysphonia, dysphagia, and weakness of the right shoulder elevation, accompanied by a mild right temporal parietal headache with radiation to the ipsilateral ear. Physical examination showed signs compatible with a right XI, X, and XI cranial nerves involvement and also several vesicular lesions in the right ear's concha. He had a personal history of poliomyelitis and chickenpox. Laringoscopy demonstrated right vocal cord palsy. Brain MRI showed thickening and enhancement of right lower cranial nerves and an enhancing nodular lesion in the ipsilateral jugular foramen, in T1 weighted images with gadolinium. Cerebrospinal fluid (CSF) analysis disclosed a mild lymphocytic pleocytosis and absence of VZV-DNA by PCR analysis. Serum VZV IgM and IgG antibodies were positive. The patient had a noticeable clinical improvement after initiation of acyclovir and prednisolone therapy. The presentation of a VZV infection with isolated IX, X, and XI cranial nerves palsy is extremely rare. In our case, the diagnosis of Vernet syndrome as a result of VZV infection was made essentially from clinical findings and supported by analytical and imaging data.

MR Neurographic Evaluation of Facial and Neck Pain: Normal and Abnormal Craniospinal Nerves below the Skull Base.

Cranial nerve disease outside the skull base is a common cause of facial and/or neck pain, which causes significant disability for patients and frustration for clinicians. Neuropathy in this region can be traumatic, idiopathic, or iatrogenic secondary to dental and surgical procedures. MR neurography is a modification of conventional MRI techniques dedicated to evaluation of peripheral nerves and is being increasingly used for imaging of peripheral neuropathies at various sites in the body. MR neurography facilitates assessment of different causes of craniofacial pain and cranial nerves and allows elegant depiction of a multitude of regional neuropathies. This article discusses the anatomy, pathologic conditions, and imaging findings of the commonly implicated but difficult to image infratentorial nerves, such as the peripheral trigeminal nerve and its branches, facial nerve, glossopharyngeal nerve, vagus nerve, hypoglossal nerve, and greater and lesser occipital nerves. ©RSNA, 2018.

Magnetic resonance imaging of developmental facial paresis: a spectrum of complex anomalies.

PURPOSE: Despite its clinical implications, the MRI features of developmental facial paresis (DFP) were described in a few case reports. This study aims to describe MRI features of DFP in relation to the embryological development with a proposed radiological new grading system. METHODS: The clinical records and MRI of the brain and internal auditory canal of 11 children with DFP were retrospectively reviewed. The following sequences were analyzed: axial, oblique sagittal SPACE of the internal auditory canal and brainstem; axial T2, T1WI and coronal T2WI of the brain. The severity of the maldevelopment of the seventh nerve was graded from 0 to 4: 0 = no abnormalities, 1 = unilateral facial nerve hypoplasia, 2 = unilateral facial nerve aplasia, 3 = aplasia or hypoplasia involving facial nerves on both sides, and 4 = facial nerve aplasia or hypoplasia associated with other cranial nerve palsy. RESULTS: Isolated facial nerve palsy was diagnosed in seven patients. It was of grade 1 in five and grade 3 in two. Hypoplasia of the nerve with interrupted course was encountered in two cases. Other associated cranial nerve abnormalities (grade 4) were seen in four patients; two of them were diagnosed previously as Moebius syndrome. In addition to inner ear anomalies, middle and external ear and parotid gland anomalies were described. CONCLUSION: To our knowledge, this is the largest series of patients with DFP that represents a continuum of isolated and combined malformations. Understanding of embryological basis can give insights into the anomalous development of the facial nerve.

Sarcoidosis in the Head and Neck: An Illustrative Review of Clinical Presentations and Imaging Findings.

OBJECTIVE: Sarcoidosis is referred to as a great imitator because of its propensity to radiologically mimic a variety of pathologic entities. Symptomatic neurosarcoidosis is present in approximately 5% of patients with sarcoidosis, and it is found histopathologically in approximately 25% of asymptomatic patients. CONCLUSION: An understanding of the multifaceted imaging manifestations of head and neck sarcoidosis will aid early recognition of the diagnosis, with a goal for earlier initiation of therapy and prevention of irreversible sequelae of the disease.

Bilateral Internal Carotid Artery Aneurysms at the Subpetrosal Portion with Unilateral Lower Cranial Nerve Palsies: Review and Consideration of Surgical Strategy.

BACKGROUND: Symptomatic bilateral extracranial internal carotid artery (ICA) aneurysms at the subpetrosal portion are extremely rare, and their treatment strategy remains unknown. CLINICAL PRESENTATION: A 42-year-old man presented to our hospital with a 2-month history of sudden onset of hoarseness, dysarthria, and dysphagia. Magnetic resonance imaging, magnetic resonance angiography, and computed tomography angiography revealed extracranial bilateral ICA aneurysms at the subpetrosal portion. The left-sided aneurysm compressed the left-sided lower cranial nerves (IX, X, XI, and XII), whereas the right-sided aneurysm was asymptomatic. We prioritized the treatment of the right-sided aneurysm to prevent bilateral lower cranial nerve deficits. This strategy was used because aneurysm treatment is not guaranteed to cure the left-sided cranial nerve palsies that lasted for 2 months. The right-sided ICA aneurysm was treated with ICA ligation and high-flow extracranial-intracranial bypass using the radial artery as bypass graft. Stent-assisted coil embolization was performed to the left-sided ICA aneurysm after 17 days. The patient showed no right-sided symptoms, and his left-sided symptoms remarkably improved 1 year after surgery. CONCLUSION: Our unique surgical strategy of prioritizing the aneurysm on the "asymptomatic" side may be one of the best treatment approaches in an extremely rare bilateral aneurysm case.

[Villaret syndrome and lung adenocarcinoma: an unusual association]. / Síndrome de Villaret y adenocarcinoma de pulmón: Una asociación infrecuente.

Villaret syndrome is defined by the affection of the glossopharyngeal (IX), vagal (X), accessory (XI) and hypoglossal (XII) cranial nerves associated with ipsilateral Horner syndrome. It is caused by the compression of these nerves and the neighboring sympathetic plexus fibers at the base of the skull, particularly in the retroparotid space. Even though the invasion of the central nervous system in patients with advanced lung cancer is a frequent and well known occurrence, this particular symptomatic association is extremely rare. We are reporting the case of a newly diagnosed lung adenocarcinoma patient who is simultaneously developing this syndrome.

Collet-Sicard syndrome: a rare but important presentation of internal jugular vein thrombosis.

We describe a rare neurological presentation of internal jugular vein thrombosis induced by central venous catheter placement in a patient with cancer. A 71-year-old man gave a 3-week history of dysphagia and dysarthria with left-sided neck pain and headache. He was receiving chemotherapy for appendiceal adenocarcinoma. On examination, he had left 9th-12th cranial neuropathies, manifesting as voice hoarseness, decreased palatal movement, absent gag reflex, weakness of scapular elevation and left-sided tongue wasting. CT scan of neck showed the left subclavian central venous catheter tip was in the left internal jugular vein. Skull base MRI showed thrombus within the left jugular foramen extending intracranially. We diagnosed Collet-Sicard syndrome secondary to thrombosis in the sigmoid-jugular venous complex. His headache and neck pain resolved 2 days after removing the catheter and starting anticoagulation. Collet-Sicard syndrome is an unusual syndrome of lower cranial nerve palsies, often signifying disease at the skull base, including malignancy, trauma or vascular causes.

Cranial Nerve Disorders in Children: MR Imaging Findings.

Cranial nerve disorders are uncommon disease conditions encountered in pediatric patients, and can be categorized as congenital, inflammatory, traumatic, or tumorous conditions that involve the cranial nerve itself or propagation of the disorder from adjacent organs. However, determination of the normal course, as well as abnormalities, of cranial nerves in pediatric patients is challenging because of the small caliber of the cranial nerve, as well as the small intracranial and skull base structures. With the help of recently developed magnetic resonance (MR) imaging techniques that provide higher spatial resolution and fast imaging techniques including three-dimensional MR images with or without the use of gadolinium contrast agent, radiologists can more easily diagnose disease conditions that involve the small cranial nerves, such as the oculomotor, abducens, facial, and hypoglossal nerves, as well as normal radiologic anatomy, even in very young children. If cranial nerve involvement is suspected, careful evaluation of the cranial nerves should include specific MR imaging protocols. Localization is an important consideration in cranial nerve imaging, and should cover entire pathways and target organs as much as possible. Therefore, radiologists should be familiar not only with the various diseases that cause cranial nerve dysfunction, and the entire course of each cranial nerve including the intra-axial nuclei and fibers, but also the technical considerations for optimal imaging of pediatric cranial nerves. In this article, we briefly review normal cranial nerve anatomy and imaging findings of various pediatric cranial nerve dysfunctions, as well as the technical considerations of pediatric cranial nerve imaging. Online supplemental material is available for this article. (©)RSNA, 2016.

Clinical-Radiologic Correlation of Extraocular Eye Movement Disorders: Seeing beneath the Surface.

Extraocular eye movement disorders are relatively common and may be a significant source of discomfort and morbidity for patients. The presence of restricted eye movement can be detected clinically with quick, easily performed, noninvasive maneuvers that assess medial, lateral, upward, and downward gaze. However, detecting the presence of ocular dysmotility may not be sufficient to pinpoint the exact cause of eye restriction. Imaging plays an important role in excluding, in some cases, and detecting, in others, a specific cause responsible for the clinical presentation. However, the radiologist should be aware that the imaging findings in many of these conditions when taken in isolation from the clinical history and symptoms are often nonspecific. Normal eye movements are directly controlled by the ocular motor cranial nerves (CN III, IV, and VI) in coordination with indirect input or sensory stimuli derived from other cranial nerves. Specific causes of ocular dysmotility can be localized to the cranial nerve nuclei in the brainstem, the cranial nerve pathways in the peripheral nervous system, and the extraocular muscles in the orbit, with disease at any of these sites manifesting clinically as an eye movement disorder. A thorough understanding of central nervous system anatomy, cranial nerve pathways, and orbital anatomy, as well as familiarity with patterns of eye movement restriction, are necessary for accurate detection of radiologic abnormalities that support a diagnostic source of the suspected extraocular movement disorder. ©RSNA, 2016.

A Rare Case of Basioccipital Encephalomeningocele with Tethered Dermoid Presenting as Cranial Nerve Palsy: A Case Report.

The basioccipital skull base is an extremely rare site for meningoencephalocele. The herniation of the brainstem or part of it through a basioccipital defect is even rarer. We report a case of rhombencephalomeningocele associated with a tethered dermoid. An extensive literature search revealed no such case report of basioccipital cranial dysraphism so far presenting with cranial nerve palsy.

Blau syndrome: cross-sectional data from a multicentre study of clinical, radiological and functional outcomes.

OBJECTIVE: To report baseline articular, functional and ocular findings of the first international prospective cohort study of Blau syndrome (BS). METHODS: Three-year, multicentre, observational study on articular, functional (HAQ, Childhood HAQ and VAS global and pain), ophthalmological, therapeutic and radiological data in BS patients. RESULTS: Baseline data on the first 31 recruited patients (12 females and 19 males) from 18 centres in 11 countries are presented. Of the 31 patients, 11 carried the p.R334W NOD2 mutation, 9 the p.R334Q and 11 various other NOD2 missense mutations; 20 patients were sporadic and 11 from five BS pedigrees. Median disease duration was 12.8 years (1.1-57). Arthritis, documented in all but one patient, was oligoarticular in 7, polyarticular in 23. The median active joint count was 21. Functional capacity was normal in 41%, mildly impaired in 31% and moderate-severe in 28% of patients. The most frequently involved joints at presentation were wrists, ankles, knees and PIPs. On radiographs, a symmetrical non-erosive arthropathy was shown. Previously unknown dysplastic bony changes were found in two-thirds of patients. Ocular disease was documented in 25 of 31 patients, with vitreous inflammation in 64% and moderate-severe visual loss in 33%. Expanded manifestations (visceral, vascular) beyond the classic clinical triad were seen in 52%. CONCLUSION: BS is associated with severe ocular and articular morbidity. Visceral involvement is common and may be life-threatening. Bone dysplastic changes may show diagnostic value and suggest a previously unknown role of NOD2 in bone morphogenesis. BS is resistant to current drugs, suggesting the need for novel targeted therapies.

RADIOLOGY OF THE MONTH: Vocal Cord Dysfunction with Multiple Cranial Neuropathies.

A 67-year-old female presented to our institution with a progressive history of hoarseness for the past six months. The patient also referred shoulder weakness and cough during the same period of time. She denied weight loss and tobacco use. Past medical history was negative for squamous cell carcinoma of the head and neck region. Physical examination demonstrated asymmetry of the sternocleidomastoid and trapezius muscles. Flexible laryngoscopy was performed and revealed complete unilateral vocal cord paralysis on the right. Magnetic Resonance (MR) of the brain with intravenous contrast (Figure 1) and computer tomography (CT) of soft tissue of the neck with contrast (Figure 2) were performed for further evaluation.

ANCA-negative Wegener's granulomatosis with multiple lower cranial nerve palsies.

Wegener's granulomatosis (WG) is a systemic vasculitis affecting small and medium-sized vessels with granulomatous formation. Though it is known for respiratory tract and kidney involvement, neurologic manifestation has been also reported. Herein we report a patient who suffered pansinusitis with multiple lower cranial nerve palsies but reached remission by immunosuppressant after the diagnosis of WG. A 54-yr-old female visited with headache, hearing difficulty, and progressive bulbar symptoms. She experienced endoscopic sinus surgeries due to refractory sinusitis. Neurologic examination revealed multiple lower cranial nerve palsies. Vasculitic markers showed no abnormality. Nasal biopsy revealed granulomatous inflammation and vasculitis involving small vessels. Given cyclophosphamide and prednisolone, her symptoms were prominently improved. WG should be considered in the patient with multiple cranial nerve palsies, especially those with paranasal sinus disease. Because WG can be lethal if delayed in treatment, prompt immunosuppressant is warranted after the diagnostic tissue biopsy.