Perplexing Initial Presentations of MOGAD in Two Children: Intracranial Hypertension and New-Onset Seizure.

We report two distinct challenging initial presentations of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Case 1 describes a 12-year-old boy who developed headaches refractory to pain medication followed by cranial neuropathies and intracranial hypertension, confirmed by lumbar puncture with an opening pressure >36 cm H2O. Case 2 describes a 3-year-old boy who developed new-onset seizures refractory to antiseizure medications, a presentation of FLAIR-hyperintense lesions in MOG-antibody associated encephalitis with seizures (FLAMES). On repeat magnetic resonance imaging, both patients were found to have cortical T2 hyperintensities, leptomeningeal contrast enhancement, and bilateral optic nerve enhancement. In the cerebrospinal fluid, both patients had CSF pleocytosis with neutrophilic predominance. The patients were treated with intravenous immunoglobulins, plasma exchange, and high-dose corticosteroids. The first patient achieved disease remission, whereas the second patient required the addition of rituximab for management of seizures. The two cases highlight the pleomorphic clinical phenotypes of MOGAD.

Chimeric Antigen Receptor-T Cell Mediated Bilateral Facial Nerve Palsy: A Case Report.

Chimeric antigen receptor (CAR-T) cell therapy is highly effective against hematological cancers but is associated with immune mediated side effects, including neurotoxicity. The most commonly described presentations of immune cell mediated neurotoxicity syndrome (ICANS) include cortical symptoms and generally localize to the central nervous system. In this report, we present a patient with acute onset of bilateral facial nerve palsy following CAR-T cell therapy, followed by a complete clinical recovery. Aside from a temporary anisocoria, he had no other neurologic symptoms and no encephalopathy or seizures. MRI Brain was non-contributory and cerebrospinal fluid revealed a modest increase in lymphocytes without systemic leukocytosis and viral studies were all negative. He was diagnosed with bilateral facial nerve palsy secondary to CAR-T cell therapy and subsequently treated with a course of steroids. Several weeks after presentation he returned to his neurological baseline. The presentation of CAR-T cell mediated facial nerve palsy is both clinically and scientifically relevant for physicians, patients, and researchers.

Presyncope and Ophthalmoparesis Due to Intracranial Erdheim-Chester Disease.

Introduction: An 82-year-old female presented to the emergency department with presyncope and was found to be bradycardic with proptosis and ophthalmoparesis. MRI revealed an extra-axial enhancing mass compressing the medulla and bilateral enhancing retro-orbital masses. Case Description: Imaging, including nuclear medicine bone scan, PET CT, and cardiac MRI raised the suspicion for a histiocytic neoplasm. These findings, along with a fibrohistiocytic infiltrate on bone biopsy and a BRAF V600E oncogenic mutation on plasma cell-free DNA confirmed a diagnosis of Erdheim-Chester disease. Discussion: These enhancing masses invoke a broad differential, including a histiocytic or granulomatous process, fungal infection, amyloidosis, IgG4 disease, and lymphoma. Systematic laboratory, radiologic, pathology, and genetic testing yielded a diagnosis of this rare histiocytic disorder with frequent neurologic involvement.

Zoster Polyneuritis Cranialis and Intracranial Vasculopathy.

A case report of varicella zoster virus (VZV) producing cranial polyneuropathy with intracranial vasculopathy afflicting the internal carotid artery, in a 45-year lady is presented. The clinical features included appearance of zosteriform rash in the left external auditory canal and concha, affliction of ophthalmic and maxillary divisions of V, VI, VII, VIII, IX and X cranial nerves. Contrast MRI brain demonstrated attenuated flow void in the left internal carotid artery. The diagnosis was confirmed with raised level of IgG VZV antibody in serum and detection of VZV IgG antibody in CSF, although PCR for VZV DNA in CSF was negative. The significance lies in atypical presentation, the diagnostic dilemma it poses, poor prognosis of cranial nerve function and the need for multidisciplinary approach to patient management and rehabilitation.

Giant Cell Arteritis Presenting With Multiple Cranial Neuropathies - Case Report.

Background: Vision loss accounts for most ophthalmic presentations of giant cell arteritis (GCA), but an important minority of patients present with diplopia and other cranial neuropathies. Case study: Here we present the case of an 84-year-old woman with a prior history of multiple cancers who was admitted to our hospital after developing double vision. She was found to have mydriasis, ptosis, and ophthalmoplegia in the right eye (OD) consistent with a combined R CNIII/CNVI neuropathy, as well as highly elevated inflammatory markers. Given her cancer history, the patient was initially worked up for various neoplastic, paraneoplastic, inflammatory, and infectious causes of multiple cranial neuropathies; however, as these results were negative, GCA became a more likely contender as a possible rare cause of multiple cranial neuropathies. The patient underwent temporal artery biopsy which showed pathology consistent with giant cell arteritis, and she was treated with steroids with eventual improvement in ophthalmoplegia and ptosis. Conclusions: This case illustrates the importance of recognizing GCA as a rare possible cause of multiple cranial neuropathies, including the indispensable role of temporal artery biopsy.

Abducens nerve palsy associated with pseudomeningocele after cervical spine surgery: a case report.

Background: Cerebrospinal fluid leakage can cause abducens nerve palsy (ANP) secondary to downward brain traction, caused by intracranial hypotension. We present the first case after cervical fixation and fusion with spinal cord decompression. Case Description: We present a 65-year-old male, who undergone C5-C6 decompression by laminectomy and C3-T2 fixation and fusion, without intraoperative complications. Two months later, the patient referred a 2-week history of diplopia, with no other accompanying symptom. Clinical examination revealed a lack of lateral gaze of the left eye. Cervical MRI disclosed findings compatible with pseudomeningocele. Given the time of evolution, the subacute clinical findings and the absence of image or clinical data of infection or intracranial hypotension, we decided to perform conservative treatment. We submitted the patient to periodic clinical examinations and we confirmed progressive clinical improvement of diplopia, in association with neurologic and ophthalmologic specialists. At this time, six months after surgery, the patient is asymptomatic. The swelling has significantly decreased in size. Control MRI revealed no growth of the pseudomeningocele. Conclusions: ANP secondary to intracranial hypotension after cervical spine surgery requires immediate imaging tests and clinical evaluation from neurology and ophthalmology specialists. Management can be conservative, as long as diplopia is the only clinical and radiological finding and wound does not show signs of infection.

Development and 5-year Evaluation of Diagnosis-Specific Protocols for Visual Neuro-Rehabilitation in a Multicenter Inpatient Rehabilitation Network.

Objective: To provide a retrospective evaluation of a new eye and vision rehabilitation care pathway in a U.S. multi-site inpatient rehabilitation network involving the occupational therapy (OT) staff and a consulting doctor of optometry (OD) specializing in vision rehabilitation. Design: Retrospective study. Setting: Two Inpatient Rehabilitation Facilities (IRFs) and 1 Long Term Acute Care Hospital (LTACH). Participants: There were 2083 records reviewed (44% women, avg. age 59 years). The most common diagnoses were hemispatial neglect (19.2%), homonymous field defects (18.5%), and oculomotor cranial nerve palsies (16.7%) (N=2083). Interventions: Clinical care was reviewed where diagnosis-specific protocols were developed and training was provided to OTs in order to reinforce OD-prescribed interventions during daily treatment sessions, including (1) third, fourth, and sixth ocular cranial nerve palsies (OCNPs) with prisms fitted for full time, postural adaptation training, and oculomotor re-education using pursuits, saccades, head-rotations, and binocular vision exercises including alternate cover and vergence; (2) homonymous hemianopia with training awareness of field loss, eccentric viewing, and fitting of Peli lens for optical field expansion; and (3) prism adaptation therapy (PAT) for left hemispatial neglect. Main Outcome Measures: Frequency of diagnoses. Hypothesis: Diagnoses with developed protocols were most common. Secondarily, feasibility and efficacy by anonymous OT survey. Results: 2083 vision consults were performed over 5 years. The most common diagnoses were hemispatial neglect (n=399, 19.2%), homonymous field defects (n=386, 18.5%), and OCNPs (n=347, 16.7%). None of the OTs reported the protocols were infeasible and 63% (IQR 38%-69%) reported their patients benefited from the interventions. The survey suggested prism for OCNPs helped in 42%, and Peli lens and PAT both helped in 38%. Conclusions: Data support the feasibility of this inpatient eye and vision rehabilitation care pathway which may be used as a foundation for creating or refining similar programs elsewhere. Uniform administration of IRF-based visual neuro-rehabilitation care could provide a substrate for future clinical trials to evaluate efficacy.

Orbital apex syndrome due to herpes zoster infection in a patient with chronic kidney disease: A case report.

We report the case of an 89-year-old man with typical herpes zoster ophthalmicus on the left side of his face with delayed clinical presentation of orbital apex syndrome. After treatment with intravenous antiviral agents and corticosteroids, the patient fully recovered. Clinical suspicion should be concerned in immunocompromised cases.

An Unusual Case of Bilateral Subperiosteal Orbital Hemorrhage.

Subperiosteal orbital hemorrhage usually occurs in the setting of facial or orbital trauma. Non-traumatic subperiosteal orbital hemorrhage (NTSOH) has rarely been reported in literature. The proposed mechanism of NTSOH is the transmission of sudden increase in cranial venous pressure to the orbital veins, which are valveless. We present a case of a 37 year old right-handed woman with a past medical history significant for type 1 diabetes, end-stage renal disease, peripheral artery disease and hypertension who developed NTSOH following an elective revision of a clotted right upper extremity arteriovenous fistula. During this procedure, she had acute eye pain, bilateral complete vision loss and emesis. CT of the orbits revealed large heterogeneously hyperdense lesions in the bilateral orbital apex extending anteriorly along the roof of the orbit, concerning for hemorrhage. Cultures obtained through nasal endoscopy were negative for a bacterial or fungal infection involving the sinuses. Ophthalmology was consulted and she underwent bilateral canthotomy and lateral cantholysis. Postoperatively, she was started on systemic and topical ocular antihypertensives, as well as prophylactic antibiotics. Visual acuity remained poor with finger counting on the right eye and lack of consistent response to light on the left eye. This case highlights periprocedural increase in systemic venous pressure secondary to a fistula repair procedure as a potential cause of NTSOH.

Charles Bonnet Syndrome in a Patient With Acute Glaucoma Following Carotid Endarterectomy.

Acute neovascular glaucoma is an extremely rare complication following carotid artery revascularization that can lead to permanent vision loss. We describe an unusual case of acute glaucoma following carotid endarterectomy presenting with mechanical pupillary dilation and vivid visual hallucinations consistent with the Charles Bonnet Syndrome. This case highlights the importance of screening patients complaining of vision loss or eye pain for neovascular eye changes prior to carotid revascularization. These patients' eye health should be closely monitored peri-operatively.

Bilateral Giant Familial Carotid Body Tumors With Concomitant Skull-Base Paraganglioma and Facial Nerve Palsy.

Carotid body tumors, rare neck paragangliomas arising from the common carotid artery bifurcation, can be classified as sporadic, hyperplastic, or familial. The familial type is often bilateral and associated with germline mutation of the mitochondrial enzyme succinate dehydrogenase. We report the rare case of a 42-year-old man who presented with bilateral giant familial carotid body tumors associated with a concomitant skull-base paraganglioma, left-sided facial nerve palsy, and an incomplete circle of Willis. We describe the excision of the tumors in 2 stages (the left mass and associated paraganglioma first and the right mass second), 6 months apart, with use of general anesthesia, and we discuss other operative considerations.

Magnetic resonance imaging of the cranial nerves in infectious, neoplastic, and demyelinating diseases, as well as other inflammatory diseases: a pictorial essay.

The cranial nerves, which represent extensions of the functional structures of the brain, traverse the head and neck. They are connected to various cranial structures and are associated with several diseases. An in-depth understanding of their complex anatomy and normal imaging appearance allows the examiner to identify and characterize abnormalities with greater precision. One important tool for evaluating the cranial nerves is contrast-enhanced magnetic resonance imaging, especially that employing three-dimensional steady-state free precession sequences, which provide high soft-tissue and spatial resolution, despite the slen-derness of the nerves. In most cases of cranial nerve abnormalities, the imaging findings are nonspecific. Therefore, to narrow the differential diagnosis, it is necessary to take a full patient history, perform a focused physical examination, and order laboratory tests. In this pictorial essay, we review, illustrate, and discuss, from a pathophysiological perspective, infectious, neoplastic, and demyelinating disorders, as well as other inflammatory disorders, affecting the cranial nerves, the aim being to provide a practical, tangible reference for radiologists to use in daily practice.

Os nervos cranianos, que representam extensões das estruturas funcionais do cérebro, atravessam a cabeça e o pescoço. Eles estão conectados a várias estruturas cranianas e estão associados a várias doenças. Uma compreensão profunda de sua complexa anatomia e aparência normal por imagem permite ao examinador identificar e caracterizar as anormalidades com maior precisão. Uma ferramenta importante para avaliar os nervos cranianos é a ressonância magnética com contraste, especialmente as sequências tridimensionais steady-state free precession, que proporcionam alta resolução espacial e de partes moles, apesar da fina espessura dos nervos. Na maioria dos casos, os achados radiológicos não são específicos. Para estreitar o diagnóstico diferencial é necessário fazer uma anamnese completa do paciente, realizar um exame físico dirigido e solicitar testes laboratoriais. Neste ensaio iconográfico revisamos, ilustramos e discutimos, sob uma perspectiva fisiopatológica, os distúrbios infecciosos, neoplásicos, inflamatórios e desmielinizantes, visando a ser uma referência prática e tangível para a prática diária dos radiologistas.

Magnetic resonance imaging of the cranial nerves in infectious, neoplastic, and demyelinating diseases, as well as other inflammatory diseases: a pictorial essay / Ressonância magnética dos nervos cranianos em doenças infecciosas, neoplásicas, desmielinizantes e outras doenças inflamatórias: ensaio iconografico

Abstract The cranial nerves, which represent extensions of the functional structures of the brain, traverse the head and neck. They are connected to various cranial structures and are associated with several diseases. An in-depth understanding of their complex anatomy and normal imaging appearance allows the examiner to identify and characterize abnormalities with greater precision. One important tool for evaluating the cranial nerves is contrast-enhanced magnetic resonance imaging, especially that employing three-dimensional steady-state free precession sequences, which provide high soft-tissue and spatial resolution, despite the slen-derness of the nerves. In most cases of cranial nerve abnormalities, the imaging findings are nonspecific. Therefore, to narrow the differential diagnosis, it is necessary to take a full patient history, perform a focused physical examination, and order laboratory tests. In this pictorial essay, we review, illustrate, and discuss, from a pathophysiological perspective, infectious, neoplastic, and demyelinating disorders, as well as other inflammatory disorders, affecting the cranial nerves, the aim being to provide a practical, tangible reference for radiologists to use in daily practice.

RESUMO Os nervos cranianos, que representam extensões das estruturas funcionais do cérebro, atravessam a cabeça e o pescoço. Eles estão conectados a várias estruturas cranianas e estão associados a várias doenças. Uma compreensão profunda de sua complexa anatomia e aparência normal por imagem permite ao examinador identificar e caracterizar as anormalidades com maior precisão. Uma ferramenta importante para avaliar os nervos cranianos é a ressonância magnética com contraste, especialmente as sequências tridimensionais steady-state free precession, que proporcionam alta resolução espacial e de partes moles, apesar da fina espessura dos nervos. Na maioria dos casos, os achados radiológicos não são específicos. Para estreitar o diagnóstico diferencial é necessário fazer uma anamnese completa do paciente, realizar um exame físico dirigido e solicitar testes laboratoriais. Neste ensaio iconográfico revisamos, ilustramos e discutimos, sob uma perspectiva fisiopatológica, os distúrbios infecciosos, neoplásicos, inflamatórios e desmielinizantes, visando a ser uma referência prática e tangível para a prática diária dos radiologistas.

Case Report: Opsoclonus-Myoclonus Syndrome Associated With Contactin-Associated Protein-Like 2 and Acetylcholine Receptor Autoantibodies in the Setting of Non-Small Cell Lung Carcinoma.

Opsoclonus myoclonus syndrome (OMS) is a rare immune-mediated paraneoplastic or para/-post-infectious syndrome characterized by "dancing" eye movements, myoclonus, and ataxia. Neuropsychiatric symptoms have also been reported. Without treatment, OMS may progress to further neurological impairment and even death. Autoimmune attack of CNS structures in OMS is most commonly mediated by anti-Ri (also known as ANNA2) IgG antibodies, with additional findings implicating antibodies targeting various neurotransmitter receptors. Prompt immunotherapy and neoplasm treatment may result in improvement. We report a novel association of Contactin-Associated Protein-Like 2 (Caspr2) antibodies occurring in association with paraneoplastic OMS. While breast cancer and small cell lung cancer (SCLC) are more commonly associated with OMS among adults, we characterize a novel association between Caspr2 antibody in a patient with mixed non-small cell and small cell lung carcinoma.

Diplopia developed by cervical traction after cervical spine surgery.

Diplopia is a rare complication of spine surgery. The abducens nerve is one of the cranial nerves most commonly related to diplopia caused by traction injury. We report a case of a 71-year-old woman who presented with diplopia developing from abducens nerve palsy after C1-C2 fixation and fusion due to atlantoaxial subluxation with cord compression. As soon as we discovered the symptoms, we suspected excessive traction by the instrument and subsequently performed reoperation. Subsequently, the patient's symptoms improved. In other reported cases we reviewed, most were transient. However, we thought that our rapid response also helped the patient's fast recovery in this case. The mechanisms by which postoperative diplopia develops vary and, thus, remain unclear. We should pay attention to the fact that the condition is sometimes an indicator of an underlying, life-threatening condition. Therefore, all patients with postoperative diplopia should undergo thorough ophthalmological and neurological evaluations as well as careful observation by a multidisciplinary team.

Complex strabismus: a case report of hypoplasia of the third cranial nerve with an unusual clinical presentation / Estrabismo complexo: um caso relacionado à hipoplasia do terceiro nervo craniano com apresentação clínica incomum

ABSTRACT Congenital cranial dysinnervation disorders are a group of complex strabismus syndromes that present as congenital and non-progressive ophthalmoplegia. The genetic defects are associated with aberrant axonal targeting onto the motoneurons, development of motoneurons, and axonal targeting onto the extraocular muscles. We describe here the surgical management of a 16-year-old boy who presented with complex strabismus secondary to hypoplasia of the third cranial nerve and aberrant innervation of the upper ipsilateral eyelid.

RESUMO Os distúrbios de inervação craniana congênita en­globam um grupo de síndromes associadas a estrabismos complexos, que se apresentam como oftalmoplegia congênita e não progressiva e são frequentemente herdadas. Os defeitos dos genes estão associados a erros no desenvolvimento ou direcionamento axonal dos motoneurônios, e erros no direcionamento axonal para os músculos extraoculares. Este caso descreve o caso de um menino que apresenta estrabismo complexo secundário à hipoplasia do terceiro nervo craniano e inervação aberrante da pálpebra superior ipsilateral, bem como o resultado após a correção cirúrgica.

A retrospective review and multi-specialty, evidence-based guideline for the management of necrotising otitis externa.

BACKGROUND: Necrotising otitis externa is a progressive infection of the external auditory canal which extends to affect the temporal bone and adjacent structures. Progression of the disease process can result in serious sequelae, including cranial nerve palsies and death. There is currently no formal published treatment guideline. OBJECTIVE: This study aimed to integrate current evidence and data from our own retrospective case series in order to develop a guideline to optimise necrotising otitis externa patient management. METHODS: A retrospective review of necrotising otitis externa cases within NHS Lothian, Scotland, between 2013 and 2018, was performed, along with a PubMed review. RESULTS: Prevalent presenting signs, symptoms and patient demographic data were established. Furthermore, features of cases associated with adverse outcomes were defined. A key feature of the guideline is defining at-risk patients with initial intensive treatment. Investigations and outcomes are assessed and treatment adjusted appropriately. CONCLUSION: This multi-departmental approach has facilitated the development of a succinct, systematic guideline for the management of necrotising otitis externa. Initial patient outcomes appear promising.