Eight-and-a-half syndrome as the first presentation of multiple sclerosis in an Asian male: a case report.

BACKGROUND: Multiple sclerosis is a diffuse chronic demyelinating disease of the central nervous system. It is relatively uncommon in the Asian population and even more so in males. Despite the usual involvement of the brainstem, eight-and-a-half syndrome remains a rare first presentation in multiple sclerosis. Only a few cases have been reported previously, but none involving the Asian population. Eight-and-a-half syndrome, a neuro-ophthalmological condition, is characterized by one-and-a-half syndrome with ipsilateral lower facial nerve palsy, which localizes lesions to the pontine tegmentum. This case report demonstrates the first case of eight-and-a-half syndrome as the first presentation of multiple sclerosis in an Asian male. CASE PRESENTATION: A healthy 23-year-old Asian man presented with sudden onset of diplopia followed by left-sided facial asymmetry for 3 days. Assessment of extraocular movement revealed left conjugate horizontal gaze palsy. On right gaze, there was limited left eye adduction and horizontal nystagmus of the right eye. These findings were consistent with a left-sided one-and-a-half syndrome. Prism cover test revealed left esotropia of 30 prism diopters. Cranial nerve examination showed left lower motor neuron facial nerve palsy, while other neurological examination was normal. Magnetic resonance imaging brain showed multifocal T2 fluid attenuated inversion recovery hyperintense lesions, involving bilateral periventricular, juxtacortical, and infratentorial regions. A focal gadolinium contrast-enhanced lesion with open ring sign on T1 sequence was seen at the left frontal juxtacortical region. Multiple sclerosis was diagnosed on the basis of the clinical and radiological evidence, which fulfilled the 2017 McDonald criteria. Positive oligoclonal bands in cerebrospinal fluid analysis further confirmed our diagnosis. He had a complete resolution of symptoms 1 month after a course of pulsed corticosteroid therapy, and was subsequently placed on maintenance therapy with interferon beta-1a. CONCLUSION: This case illustrates eight-and-a-half syndrome as the first presentation of a diffuse central nervous system pathology. A wide range of differential diagnoses needs to be considered in such a presentation as based on the patient's demographics and risk factors.

Síndrome del ocho y medio como manifestación de enfermedad desmielinizante aguda por adenovirus / Eight-and-a-half syndrome as manifestation of acute disseminated adenovirus encephalomyelitis

La encefalomielitis diseminada aguda es una enfermedad inflamatoria-desmielinizante inmunomediada que suele manifestarse tras una infección o vacunación en niños en edad escolar. Típicamente presenta una fase prodrómica con un cuadro pseudogripal seguida de una fase con clínica muy variada, pudiendo aparecer alteraciones neurooftalmológicas como oftalmoplejía o neuritis óptica.La etiología es variada, incluyendo enfermedades tumorales, vasculares, infecciosas, inflamatorias y desmielinizantes. El diagnóstico se basa en la historia clínica y en las características de la resonancia magnética cerebral, prueba de imagen de elección. El estudio del líquido cefalorraquídeo puede servir de ayuda en la orientación del cuadro clínico.El pronóstico es favorable, con excelente respuesta a los corticoides e inmunoglobulinas y con mínimas secuelas a largo plazo en la mayoría de los casos.Presentamos el caso de un varón de 8años con enfermedad desmielinizante aguda por adenovirus cuya manifestación fue un síndrome del ocho y medio. (AU)

Acute disseminated encephalomyelitis is an immune mediated inflammatory-demyelinizing disease that usually manifests after infection or vaccination in school-age children. It typically presents a prodromal phase with flu-like symptoms, followed by a phase with varied clinical symptoms, neuro-ophthalmological alterations such as ophthalmoplegia or optic neuritis may occur.The differential diagnosis includes tumor, vascular, infectious, inflammatory and demyelinating diseases. Diagnosis is based on the clinical history and the characteristics of brain magnetic resonance imaging, the gold standard test. The study of the cerebrospinal fluid can help to guide the clinical picture.The prognosis is favorable, with an excellent response to corticosteroids and immunoglobulins, with minimal long-term sequelae in most cases.We report the case of an 8-year-old male with acute demyelinating disease due to adenovirus whose manifestation was an eight-and-a-half syndrome. (AU)

Eight-and-a-half syndrome as manifestation of acute disseminated adenovirus encephalomyelitis.

Acute disseminated encephalomyelitis is an immune mediated inflammatory-demyelinizing disease that usually manifests after infection or vaccination in school-age children. It typically presents a prodromal phase with flu-like symptoms, followed by a phase with varied clinical symptoms, neuro-ophthalmological alterations such as ophthalmoplegia or optic neuritis may occur. The differential diagnosis includes tumor, vascular, infectious, inflammatory and demyelinating diseases. Diagnosis is based on the clinical history and the characteristics of brain magnetic resonance imaging, the gold standard test. The study of the cerebrospinal fluid can help to guide the clinical picture. The prognosis is favorable, with an excellent response to corticosteroids and immunoglobulins, with minimal long-term sequelae in most cases. We report the case of an 8-year-old male with acute demyelinating disease due to adenovirus whose manifestation was an eight-and-a-half syndrome.

Eight-and-a-Half Syndrome Secondary to Neurotoxoplasmosis: A Rare Case Report.

Eight-and-a-half syndrome (EHS) is a neuro-ophthalmological condition characterised by horizontal gaze palsy, internuclear ophthalmoplegia, and ipsilateral facial palsy. Albeit rare, EHS is a well reported condition in the literature, with several reports presenting multiple aetiologies. Infarcts are the cause in more than half the cases. Human immunodeficiency virus (HIV)-related cases are rare, and are probably underreported in low- and middle-income countries. In this report, we describe EHS secondary to neurotoxoplasmosis in a 40-year-old HIV-positive Brazilian man. EHS secondary to neurotoxoplasmosis is a challenging diagnosis, with important differential diagnoses, notably for HIV patients.

An unusual presentation of hemiparesis with complex ophthalmoplegia: A 'nine' syndrome.

Nine syndrome is a rare clinical entity with core clinical signs of one and a half syndrome, ipsilateral facial palsy, and contralateral hemiparesis/hemianesthesia and localizing to the dorsal paramedian pontine tegmentum. The awareness of this sign helps in precise localization and consideration of relevant aetiologies. Here, we report a case consisting of right horizontal gaze palsy with right internuclear opthalmoplegia and right lower motor neuron type VII nerve palsy constituting eight-and-a-half syndrome. With the additional involvement of left upper and lower limb upper motor neuron weakness, it revealed the lesion responsible for the 'nine' syndrome in magnetic resonance imaging of the brain. It is our purpose to highlight the genesis of this combination of clinical signs.

A Rare Neuro-Ophthalmological Condition in a Patient with Lung Adenocarcinoma: The Eight-and-a-Half Syndrome, Case Report and Review of the Literature.

The eight-and-a-half syndrome is a rare neuro-ophthalmological condition caused by a structural lesion in the dorsal portion of the pons, involving critical areas of the brainstem, i.e., medial longitudinal fasciculus (MLF), abducens nucleus, facial genu, and colliculus. It is characterized by internuclear ophthalmoplegia with horizontal gaze palsy and peripheral facial palsy. Although the syndrome is most frequently caused by vascular or demyelinating diseases, several different underlying causes might occur. Herein, we describe a case of the eight-and-a-half syndrome caused by a lung adenocarcinoma metastasis localized in the lower pontine tegmentum. Then, we review the current literature on the underlying causes of the eight-and-a-half syndrome.

Stroke syndromes: A case of an eight and a half syndrome

INTRODUCTION@#Eight and a Half syndrome: a combination of ipsilateral cranial nerve seven palsy plus one and a half syndrome is rare. Exact prevalence of the syndrome has not been reported as of yet. This syndrome is mostly attributed to a vascular etiology such as a pontine tegmental infarction. @*OBJECTIVE@#To present a rare case of a stroke syndrome : eight and a half syndrome (peripheral cranial nerve seven palsy plus a one and an half syndrome) in an adult male. To present the importance of its early clinical recognition in correlation of radiologic imaging, and management. @*CASE REPORT@#This is a case of a sixty-two year old male, who had a one day history of sudden double vision. Cranial nerve examination revealed a frozen right eye; unable to perform any movement on horizontal gaze, and with right sided facial asymmetry. He was hypertensive for more than ten years. Left eye was exotropic, with no adduction. Right eye was frozen on horizontal gaze, and primary gaze was at midline. Right sided peripheral facial palsy was seen on examination. Cranial non-contrast magnetic resonance imaging with time of flight was done revealing an infarct in the right posterior pontine area, and a narrow right vertebral artery due to a probable occlusion. Patient was started on antiplatelet cilostazol 100mg/tab 1 tablet twice daily. Atorvastatin 40mg/tab 1 tablet was given. Anti-hypertensives were started on his fourth hospital day. Smoking cessation, dietary modifications, and compliance to medications were emphasized prior to discharge. @*DISCUSSION@#Here we have a stroke syndrome presenting as an ipsilateral lower motor neuron: seventh nerve palsy, and an ipsilateral horizontal gaze palsy with internuclear ophthalmoplegia of the contralateral eye (failure of adduction) termed as CN VII, + 1 ½ syndrome or Eight and a Half Syndrome. This is caused by a lesion involving the paramedian pontine reticular formation (PPRF) which sends signals towards the ipsilateral abducens nerve and contralateral medial longitudinal fasciculus. These structures lie in close proximity to the nucleus and intraaxial fascicles of cranial nerve VII manifesting as facial weakness of the ipsilateral side to the lesion. An occlusion in the tip of the paramedian pontine artery, a branch of the basilar artery, is the most common etiology.

Síndrome del ocho y medio secundario a vasculopatía lúpica: presentación de un caso / Eight-and-a-half syndrome secondary to lupus vasculopathy: a case presentation

RESUMEN Introducción: El manejo diagnóstico y terapéutico en los pacientes con lupus eritematoso sistémico que desarrollan una afectación neuropsiquiátrica representa un reto, debido a la heterogeneidad de las formas en que puede presentarse y la ausencia de criterios diagnósticos. Objetivo: Reconocer las formas clínicas de presentación de los síndromes neuroftalmológicos que traducen afectación pontina. Presentación del caso: Hombre de 71 años con antecedente de lupus eritematoso sistémico con afectación neuopsiquiátrica, que de forma aguda desarrolla un cuadro emético en el curso de una emergencia hipertensiva seguido de una parálisis de la mirada horizontal hacia la izquierda, una oftalmoplejía internuclear posterior derecha y una parálisis facial izquierda. En la neuroimagen se constata una afectación multifocal con marcado daño pontino. Conclusiones: Reconocer las formas clínicas de presentación de estos trastornos neuroftalmológicos raros que generalmente se presentan de forma aguda/subaguda permite al neurólogo realizar el diagnóstico topográfico de la lesión a nivel protuberancial con elevada precisión desde la Sala de Urgencias, así como reducir los posibles diagnósticos diferenciales a una etiología vascular, desmielinizante u ocupativa de espacio(AU)

ABSTRACT Introduction: The diagnostic and therapeutic management of patients with systemic lupus erythematosus who develop a neuropsychiatric involvement represents one of the biggest challenges due to the heterogeneity of the ways in which it can occur and the absence of diagnostic criteria. Objective: To recognize the clinical forms of presentation of neurophthalmological syndromes that express pontine involvement. Case presentation: Seventy-one-year-old man with history of systemic lupus erythematosus with neuropsychiatric involvement who acutely develops an emetic episode in the course of a hypertensive emergency followed by a paralysis of the horizontal gaze to the left, a right-sided posterior internuclear ophthalmoplegia and a left facial palsy. In the neuroimaging, a multifocal involvement with marked pontine damage is observed. Conclusions: Recognizing the clinical forms of presentation of these rare neurophthalmological disorders that generally occur in an acute or subacute form allows the neurologist to perform the topographic diagnosis of the lesion at a protuberancial level with high precision from the time when the patient attends the Emergency Department and reduces the possible differential diagnoses to a vascular, demyelinating or occupational etiology of space(AU)

Eight-and-a-half syndrome: a rare potentially life-threatening disease.

Purpose: To report a case of eight-and-a-half syndrome presenting with a conjugate horizontal gaze palsy, an ipsilateral internuclear ophthalmoplegia, and an ipsilateral lower motor neuron-like facial palsy. Methods: A 56-year-old male who came in for limitation of extraocular muscle motion - wherein the only remaining movement is abduction of the left eye - and right-sided facial weakness. Magnetic resonance imaging revealed a well-defined lesion resembling a cavernoma, at the level of the ponto-medullary junction. Results: Eight-and-a-half syndrome results from a lesion affecting the paramedian pontine reticular formation, the median longitudinal fasciculus, and the facial nerve fascicle on one side. In this case, a cavernoma at the level of the ponto-medullary junction was compressing the important pontine structures. Conclusion: Although eight-and-a-half syndrome is most commonly caused by an infarction or demyelination, in rare instances, a space-occupying lesion at the level of the pons can be the etiology. It is of utmost importance to recognize the features of this disease entity to be able to exhaust the proper diagnostic exams, localize the lesion and determine the proper treatment regimen catered to each patient.

Eight-and-a-Half Syndrome: A Combination of Intracranial Capillary Telangiectasia and Hematencephalon.

Eight-and-a-half syndrome, a combination of one-and-a-half syndrome and ipsilateral facial palsy, was first described by Eggenberger in 1998. Intracranial capillary telangiectasia (ICT) is a rare type of latent cerebral vascular malformation characterized by a number of small, dilated, and thin-walled blood capillaries with normal brain tissues between them. Susceptibility weighted imaging is the recommended diagnostic method to detect ICT. Oommen once reported 1 case about pontine hemorrhage causing Fisher one-and-a-half syndrome with facial paralysis. Here, we report a case with regard to pontine tegmentum ICT and hematencephalon presenting as eight-and-a-half syndrome.

Eight-and-a-half syndrome caused by a pontine haemorrhage: a case report and review of the literature.

BACKGROUND: Eight-and-a-half syndrome is caused by a lesion in the dorsal tegmentum of the caudal pons involving parapontine reticular formation and median longitudinal fasciculus, as well as the nucleus and/or the fasciculus of the facial nerve. It is characterized by one-and-a-half syndrome and an ipsilateral cranial nerve VII palsy. Also, many variants of eight-and-a-half syndrome have been described, including nine syndrome, thirteen-and-a-half syndrome and fifteen-and-a-half syndrome. METHODS: We describe a case of a 49-year-old man who presented with eight-and-a-half syndrome combined with contralateral hemiparesis. We reviewed the literature describing the related spectrum of eight-and-a-half syndrome associated with various etiologies. RESULTS: Brain computed tomography scan revealed a hyperdensity located in the left paramedian aspect of the dorsal pons. T2-weighted magnetic resonance imaging at the 11-month follow-up showed hyperintensity and enlargement of the inferior olivary nuclei, which were compatible with a diagnosis of hypertrophic olivary degeneration. In light of our observations and cases reported in the literature, we categorize the spectrum of eight-and-a-half syndrome into three types, namely classic eight-and-a-half syndrome, eight-and-a-half syndrome variants and eight-and-a-half plus syndrome. Besides, the clinical feature and outcome of the three types are discussed in this article. CONCLUSIONS: Recognition of the spectrum of eight-and-a-half syndrome allows precise anatomic localization of the lesion to pontine tegmentum region.

"Eight and a half" and "nine syndrome" rare presentation of pontine lesions; case reports and review of literature.

Background: Eight-and-a-half syndrome (EHS) is one-and-a-half syndrome [(conjugated horizontal gaze palsy and internuclear ophthalmoplegia (INO)] plus ipsilateral fascicular seventh cranial nerve palsy. Involvement of lower pontine tegmentum including the abducens nucleus, the ipsilateral medial longitudinal fasciculus (MLF), and the adjacent facial colliculus contribute to the clinical findings of EHS. Recently, nine syndrome with addition of hemiparesis or hemianesthesia to EHS (due to involvement of adjacent corticospinal tract or medial lemniscus) is suggested. Methods: Consecutive patients with presentation of EHS or nine syndrome were reviewed from referral neuro-ophthalmology and strabismus clinics. Results: Three cases of EHS were identified with different etiologies of intracerebral hemorrhage (ICH), demyelination, and neuromyelitis optica spectrum disorder. Moreover, one case of "nine syndrome" due to ICH was described. Brain magnetic resonance imaging (MRI) in all of them revealed lesion in lower tegmentum of pons. Conclusion: Apart from different etiologies, recognition of EHS or nine syndrome allows precise localization of the lesion to lower pontine tegmentum ipsilaterally.

“Nine” syndrome and its MRI brain findings

We describe a rare presentation of pontine infarction in a lady who was on dual antiplatelet therapy.Her presentation includes one and a half syndrome, left facial nerve palsy and contralateral hemiataxia.Magnetic resonance imaging (MRI) of the brain revealed acute infarction of the dorsal pons. A diagnosisof “nine” syndrome secondary to lacunar stroke was made. She continued dual antiplatelet therapyand her symptoms resolved completely over three months.

"Nine" syndrome: A new neuro-ophthalmologic syndrome: Report of two cases.

"Eight-and-a-half" syndrome is a rare condition involving the ipsilateral abducens nucleus or paramedian pontine reticular formation (PPRF), the ipsilateral medial longitudinal fasciculus (MLF), and the adjacent facial colliculus/facial nerve fascicle. The condition is often caused by a lesion (vascular or demyelinating) in the dorsal tegmentum of the caudal pons. There are new variants of this syndrome caused by extension of lesion to involve new adjacent structures in pontine tegmentum. We report two patients with different etiology presenting with clinical features suggestive of eight-and-a-half syndrome associated with hemiataxia representing "nine" syndrome (8½ + ½ = 9) adding new dimension to "eight-and-a-half" syndrome.