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.

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 case of bilateral fourth nerve palsy associated with pseudotumor cerebri syndrome / Caso de paralisia bilateral de quarto nervo associada com síndrome do pseudotumor cerebral

ABSTRACT Pseudotumor cerebri syndrome is puzzling because it results in elevated intracranial pressure with no identifiable underlying cause. Ocular motor nerve palsies, other than a unilateral or bilateral sixth cranial nerve palsy, are rarely seen in patients with this condition. We report here on a 25-year-old female patient with bilateral fourth cranial nerve palsy caused by pseudotumor cerebri syndrome, whose ocular and systemic signs and symptoms of nerve palsy were completely resolved after medical treatment. We infer that fourth nerve palsy could be associated with pseudotumor cerebri syndrome; therefore, clinicians should consider rare ocular motor nerve palsies, even bilaterally, in patients with pseudotumor cerebri syndrome.

RESUMO A sindrome do pseudotumor cerebral é uma síndrome enigmática caracterizada por aumento da pressão intracraniana sem causa definida. Na síndrome do pseudotumor cerebral, as paralisias de nervo oculomotor além da paralisia unilateral ou bilateral do sexto nervo craniano são raramente vistas. Nós reportamos o caso de uma paciente feminina de 25 anos com paralisia bilateral de quarto nervo craniano como resultado da síndrome do pseudotumor cerebral. Após tratamento médico para síndrome do pseudotumor cerebral, os sinais e sintomas oculares e sistêmicos da paralisia nervosa foram resolvidos. Em conclusão, a paralisia de quarto nervo craniano pode estar associada com síndrome de pseudotumor cerebral, portanto médicos devem considerar paralisias raras de nervo oculomotor, mesmo bilateralmente, em pacientes com síndrome do pseudotumor cerebral.

Voz hipernasal y regurgitación nasal aguda: reporte de un caso clínico y revisión de la literatura / Hypernasal speech and acute nasal regurgitation: a case report and a review of the literature

RESUMEN La voz hipernasal y la regurgitación nasal son síntomas de disfunción velofaríngea. Ésta puede tener múltiples causas: anatómicas, neurológicas o funcionales. Se describe el caso de una paciente de sexo femenino, de 13 años, que se presenta con voz hipernasal y regurgitación nasal aguda. Al examen físico se evidencia inmovilidad del velo del paladar derecho sin otros hallazgos neurológicos. El estudio con resonancia nuclear magnética de cerebro y punción lumbar fueron normales. Se diagnosticó una incompetencia velofaríngea aguda transitoria, de probable etiología viral. La paciente evolucionó de forma favorable con mejoría clínica progresiva. La incompetencia velofaríngea a causa de una paresia o parálisis del nervio vago y/o nervio glosofaríngeo es una causa poco frecuente de disfunción velofaríngea.

ABSTRACT Hypernasal speech and nasal regurgitation are symptoms of velopharyngeal dysfunction. This may have multiple causes, including velopharyngeal incompetence due to paresis or paralysis of the vagus nerve and/or glossopharyngeal nerve. We describe the case of a 13 year-old female patient, with hypernasal speech and acute nasal regurgitation, with a physical examination showing immobility of the right palate with no other neurological findings. Magnetic resonance imaging of the brain and lumbar puncture was normal. Transient acute velopharyngeal incompetence was diagnosed, probably of viral etiology. The patient evolved favorably with progressive clinical improvement. Velopharyngeal incompetence due to paresis or paralysis of the vagus and/or glossopharyngeal nerves is a rare cause of velopharyngeal dysfunction.

Zoster Laryngitis with Multiple Cranial Nerve Palsy Progressed as Ascending Involvement

BACKGROUND: Herpes zoster of the head and neck commonly presents with Ramsay Hunt syndrome. However, vesicular eruptions may occur on the pharyngeal or laryngeal area with multiple lower cranial-nerve (CN) palsy. CASE REPORT: We report on the case of a 54-year-old man with herpes zoster of the pharynx and larynx with multiple CN palsy and persistent hiccups. He initially developed progressive dysphagia, hoarseness, and persistent hiccups (CN IX and X). After admission, Dizziness, hearing impairment, and peripheral facial palsy (CN VII and VIII) were complicated. The results of a polymerase chain reaction test of saliva and vesicular fluid from the ear and throat were strongly positive for varicella zoster virus. The progression of CN palsy was in an ascending sequence. CONCLUSION: We suggest that the sequence of CN palsy may be either ascending or descending, depending on the initial site of involvement.

Clinical Course and Prognostic Factors of Acquired Third, Fourth, and Sixth Cranial Nerve Palsy in Korean Patients

PURPOSE: This study aimed to evaluate the clinical course and prognostic factors of acquired third, fourth, and sixth cranial nerve (CN) palsy grouped according to etiology. METHODS: This study involved a retrospective review of the medical records of 153 patients who were diagnosed with acquired paralytic strabismus from January 2004 to July 2015. Outcomes, recovery rates, and time to recovery were investigated according to the affected CN: CN3, CN4, and CN6 palsies. The patients were classified into four groups based on etiology: idiopathic, traumatic, neoplastic, and vascular. RESULTS: The mean age of the patients was 59.8 ± 14.5 years and the mean follow-up period was 10.8 months. Out of the 153 patients, 63 (41.2%) had CN3 palsy, 35 (22.9%) had CN4 palsy, and 55 (35.9%) had CN6 palsy. The most common causes were vascular related (54.9%), followed by idiopathic (28.1%), trauma (8.5%), and neoplasm (5.88%). About 50% of the patients recovered within six months. Among the four etiologic groups, the idiopathic group showed the best prognosis because about 50% of the patients in this group recovered within three months. This was followed by the vascular, traumatic, and neoplastic groups. Cox proportional hazard analysis revealed a significant association between the baseline prism diopter and recovery rate. CONCLUSIONS: The prognosis and natural history of paralytic strabismus vary depending on its cause. The vascular group had the best recovery rate and shortest recovery time, whereas the neoplastic group required the longest time to recover.

Analysis of curative effect of microvascular decompression in treating cranial nerve diseases under endoscope and microscope / 中华显微外科杂志

Objective To analyse the safety and effectiveness of microvascular decompression completely under endoscopy-assisted or microscope-assisted (EVD or MYD) for trigeminal neuralgia.Methods A retrospective analysis was conducted for patients who underwent completely EVD or MVD from January,2014 to September,2016.The clinical data,operative data and results were analyzed.The patients were divided into two groups according to the techniques.The all procedure of distinguishing the responsible vessels and decompressing which was done completely under endoscope were EVD group (n=141).Those being done completely under microscope were MVD group (n=125).All operations were done by same operator and same approach.Results The intracranial nerve symptoms disappeared in 137 cases(97.2％) in EVD group and 120 cases(96.0％) in MVD group.There were no severe operative complications such as intracranial infection,facial paralysis,intracranial hematoma and death.Mild hearing loss was happened in 2 cases (1.42％) in EVD group and 5 cases (4.00％) in MVD group;facial hypoesthesia,5 cases (3.54％) in EVD group,8 cases(6.40％) in MVD group;Transient ataxia,3 cases (2.13％) in EVD group,5 cases(4.00％) in MVD group;cerebrospinal fluid leakage,3 cases(2.13％) in EVD group,3 cases(2.40％) in MVD group;Oral herpes,23 cases (22.5％) in EVD group,27 cases(24.3％) in MVD group.There was no statistically significant differences in curative effect and complications between two groups (P＞ 0.05).Conclusion There is no difference in curative effect and complications between the two methods of completely EVD and MVD.

Changes of serum ferritin and 25-(OH) vitamin D3 in cranial neuropathy diabetic patients / 中国医师杂志

Objective To observe the changes of serum ferritin and 25-(OH) vitamin D3 in patients with diabetic cranial neuropathy.Methods There were 50 patients without diabetic Cranial neuropathy,46 patients with diabetic cranial neuropathy,and 40 cases of normal control group.The changes of serum ferritin and 25-hydroxy vitamin D3 were observed in each group.The correlation between two indexes and the correlation with diabetic cranial neuropathy were analzyzed.Results The serum ferritin levels in diabetic group and diabetic neuropathy group were significantly higher than those in normal control group (P ＜ 0.01),and its level in patients with diabetic cranial neuropathy [(687.54 ± 65.38)ng/ml] was significantly higher than that of patients without diabetic cranial neuropathy [(497.28 ± 46.39) ng/ml,P ＜0.01].The serum 25-(OH) vitamin D3 levels in the diabetic group and diabetic neuropathy group were lower than those in the control group (P ＜ 0.01),and its level in patients with diabetic cranial neuropathy [(26.45 ± 8.93)nmol/l] was significantly less than that of patients without diabetic cranial neuropathy [(37.19-± 9.74)nmol/L,P ＜ 0.01].Serum ferritin levels were positively correlated with 25-(OH) vitamin D3 (r =-0.59,P ＜ 0.01).Multivariate unconditional Logistic regression analysis showed that diabetic neuropathy was negatively correlated with 25-(OH) vitamin D3 (P ＜ 0.05).Conclusions The increases of serum ferritin and 25-(OH) vitamin D3 are closely related to the occurrence and development of diabetic cranial neuropathy,which provides the theoretical basis for clinical intervention therapy.

Bilateral Cranial IX and X Nerve Palsies After Mild Traumatic Brain Injury

We report a 57-year-old man with bilateral cranial nerve IX and X palsies who presented with severe dysphagia. After a mild head injury, the patient complained of difficult swallowing. Physical examination revealed normal tongue motion and no uvular deviation. Cervical X-ray findings were negative, but a brain computed tomography revealed a skull fracture involving bilateral jugular foramen. Laryngoscopy indicated bilateral vocal cord palsy. In a videofluoroscopic swallowing study, food residue remained in the vallecula and pyriform sinus, and there was reduced motion of the pharynx and larynx. Electromyography confirmed bilateral superior and recurrent laryngeal neuropathy.

Relapsing polychondritis presenting with inflammatory pseudotumor

No abstract available.

Churg-Strauss Syndrome as an Unusual Cause of Dysphagia: Case Report

Systemic vasculitis is a rare disease, and the diagnosis is very difficult when patient shows atypical symptoms. We experienced an unusual case of dysphagia caused by Churg-Strauss syndrome with lower cranial nerve involvement. A 74-year-old man, with a past history of sinusitis, asthma, and hearing deficiency, was admitted to our department for evaluation of dysphagia. He also complained of recurrent bleeding of nasal cavities and esophagus. Brain magnetic resonance imaging did not show definite abnormality, and electrophysiologic findings were suggestive of mononeuritis multiplex. Dysphagia had not improved after conventional therapy. Biopsy of the nasal cavity showed extravascular eosinophilic infiltration. All these findings suggested a rare form of Churg-Strauss syndrome involving multiple lower cranial nerves. Dysphagia improved after steroid therapy.

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.

MRI findings of nasopharyngeal carcinoma in patients presenting with ocular signs / 中华放射学杂志

Objective To study the MRI features of nasopharyngeal carcinoma (NPC) in patients who presented with ocular abnormalities. Methods The clinical signs and MRI features of Ⅲ, Ⅳ and/or Ⅵ cranial nerve involvement in 22 cases of histologically proved nasopharyngeal carcinoma were retrospectively analyzed. Results All 22 cases showed perineural tumor spread along the course of Ⅲ, Ⅳ and Ⅵ cranial nerve. Diplopia was the most common sign, occurred in 14/22 patients. The incidence of incomplete palsy of Ⅲ, Ⅳ and Ⅵ cranial nerve were 16/44, 7/44 and 4/44, respectively. There were four types of abnormal MRI findings: cavernous sinus thickening (10/44) and mass ( 16/44), superior orbital fissure enlargement (4/44) and abnormal signals (9/44), thickened oculomotor muscles with obscured adjacent fatty space and abnormal enhancement (10/44). The dynamic contrast enhancement patterns of the masses in cavernous sinus and orbital were the same as that of nasopharynx lesions; all appeared as rapid initial enhancement followed by a plateau phase. Conclusion Oculomotor nerve involvement can be the first clinical signs of NPC, and MRI can show the perineural extension along the Ⅲ, Ⅳ and Ⅵ cranial nerve in NPC.

A Case of Lewis-Sumner Syndrome Showing Dramatic Improvement after Plasma Exchange

We report a patient with Lewis-Sumner syndrome (LSS) who showed an improvement only with plasma exchange (PE). The patient, 32-yr old man, had progressive multifocal motor-sensory deficits with persistent, multiple conduction blocks and marked slowing of NCVs. Nerve pathology supported a diagnosis of demyelinating neuropathy by revealing marked loss of myelinated fibers with inter- and intrafascicular variation. Although the patient was refractory to treatment with corticosteroid and intravenous immunoglobulin, PE produced a dramatic improvement. Our experience strongly proposes that PE should be tried for refractory LSS.

Paralisia facial periférica: incidência das várias etiologias num ambulatório de atendimento terciário / Facial nerve palsy: incidence of different ethiologies in a tertiary ambulatory

Introdução: O diagnóstico etiológico diferencial da paralisia facial periférica continua a ser desafiante e os diversos estudos na literatura têm apresentado resultados conflitantes quanto a sua epidemiologia. Objetivo: Traçar a incidência das várias etiologias e o perfil dos pacientes atendidos com paralisia facial periférica no ambulatório do Serviço de Otorrinolaringologia da Faculdade de Ciências Médicas e Biológicas da Pontifícia Universidade Católica de São Paulo - Campus Sorocaba. Método: Foram analisados de maneira retrospectiva os prontuários dos pacientes com paralisia facial periférica no período de 2007 e 2008. Resultados: Dos 54 pacientes analisados, 55,5% eram do sexo masculino, apresentaram idade média de 40,6 anos e o lado direito da face acometido em 66,6%. Como sintomas associados, observamos a parestesia da hemiface acometida em 51,85% e o aumento do lacrimejamento em 66,6% dos pacientes. A paralisia de Bell foi à etiologia mais frequente (53,7%), seguido por: traumática (24%), Síndrome de Ramsay-Hunt (9,2%), colesteatoma (5,5%), otite externa maligna (3,7%) e otite média aguda (3,7%). Três casos de paralisia de Bell na gestação foram encontrados nesta serie. Conclusão: Os dados encontrados são semelhantes à maior parte da literatura, mostrando que a paralisia de Bell continua sendo a mais frequente seguido das causas traumáticas e demais. Há um equilíbrio em relação aos sexos com leve prevalência do sexo masculino e da paralisia do lado direito da face.

Introduction: The ethiologic diferencial diagnostic for facial nerve paralisis is still a challenge and the literature has shown conflictive results concerning its epidemiology. Objective: To outline the incidence of the different ethiologies and the profile of peripheral facial nerve paralysis patients in the otolaryngology ambulatory of the Faculdade de Ciencias Medicas e Biologicas da PUC-SP - campus Sorocaba. Method: The records of 54 patients with facial nerve paralysis seen during the years of 2007 and 2008 were analysed retrospectively. Results: From the 54 patients analysed, 55,5% were male, median age of 40,6 years and had the right side of the face acomitted in 66,6%. Parestesia of the accomited side in 51,85% and increased tears in 66,6% of the patients were observed as associated symptoms. Bellïs palsy was the most frequent ethiology (53,7%), follwed by: traumatic (24%), Ramsay Hunt syndrome (9,2%), Cholesteatoma (5,5%), malignant otitis media (3,7% and acute otits media (3,7%). Three cases of Bellïs palsy during pregancy was also seen in this series. Conclusion: The data found are similiar of the most of the literature, showing that Bellïs palsy is still the most frequent, followed by traumatic causes and others. There is an equilibrium concerning to the gender, with a slight prevalence for males and for the right side of the face.

Emphasizing the correlation between brain and eye to improve neuro-ophthalmology / 中华眼底病杂志

Eye and nervous system are anatomically and physiologically very close.About 40% of the nerve fibers in brain are correlated with visual function.Seven of twelve cranial nerves are straightly correlated with visual afferent or eye movement.Neurological diseases can affect eye in many different ways.Furthermore,systemic disorders can cause secondary neurological lesions or even primary neurological disorders,which sequentially show ocular symptoms and signs.Better understanding of the close relationship between eye and brain,together with intimate cooperation between ophthalmologists,neurologists,neurosurgeons and other specialties,are the key point to improve our neuro-ophthalmology service in China.