Synergistic convergence and split pons in horizontal gaze palsy and progressive scoliosis in two sisters.

Synergistic convergence is an ocular motor anomaly where on attempted abduction or on attempted horizontal gaze, both the eyes converge. It has been related to peripheral causes such as congenital fibrosis of extraocular muscles (CFEOM), congenital cranial dysinnervation syndrome, ocular misinnervation or rarely central causes like horizontal gaze palsy with progressive scoliosis, brain stem dysplasia. We hereby report the occurrence of synergistic convergence in two sisters. Both of them also had kyphoscoliosis. Magnetic resonance imaging (MRI) brain and spine in both the patients showed signs of brain stem dysplasia (split pons sign) differing in degree (younger sister had more marked changes).

Neuropareidolia: diagnostic clues apropos of visual illusions / Neuropareidolia: pista diagnóstica a partir de uma ilusão visual

Diagnosis in neuroimaging involves the recognition of specific patterns indicative of particular diseases. Pareidolia, the misperception of vague or obscure stimuli being perceived as something clear and distinct, is somewhat beneficial for the physician in the pursuit of diagnostic strategies. Animals may be pareidolically recognized in neuroimages according to the presence of specific diseases. By associating a given radiological aspect with an animal, doctors improve their diagnostic skills and reinforce mnemonic strategies in radiology practice. The most important pareidolical perceptions of animals in neuroimaging are the hummingbird sign in progressive supranuclear palsy, the panda sign in Wilson's disease, the panda sign in sarcoidosis, the butterfly sign in glioblastomas, the butterfly sign in progressive scoliosis and horizontal gaze palsy, the elephant sign in Alzheimer's disease and the eye-of-the-tiger sign in pantothenate kinase-associated neurodegenerative disease.

O diagnóstico em neuroimagem envolve o reconhecimento de padrões específicos indicativos de doenças particulares. Pareidolia, é a perceção equivocada de algo claro e distinto a partir de um estímulo vago e obscuro, por vezes benéfico a quem interpreta exames de imagem na procura do diagnóstico. A este propósito, alguns animais podem pareidolicamente ser reconhecidos em neuroimagens associadas a determinadas doenças específicas, promovendo mais rapidez na habilidade diagnóstica e naturalmente reforçando estratégias mnemônicas individuais na prática do diagnóstico neuroradiológico. Alguns dos sinais de neuroimagens relacionados a percepções pareidolicas de animais são: o sinal do beja-flor na paralisia supra nuclear progressiva; o sinal do panda na doença de Wilson; o sinal do panda na sarcoisdose; o sinal da borboleta no glioblastoma; o sinal da borboleta no escoliose progressiva e paralisia do olhar horizontal; o sinal do elefante na doença de Alzheimeir; e o sinal do olho de tigre na doença degenerativa ligada a pantothenato kinase.

A Case of Horizontal Gaze Palsy with Ipsilateral Esotropia due to Pontine Hemorrhage

PURPOSE: We describe a patient with a horizontal pontine gaze palsy and a concurrent esotropia and discuss the etiology and surgical management of this condition. METHODS: A 42-years-old male visited the hospital with dipopia and left ocular pain. He had a history of pontine hemorrhage a year ago. Because of left ficial palsy, lagophthalmos was noted on the left eye and slit-lamp examination showed filament keraititis and superficial punctate keratitis. Left face turn was noted and a left horizontal gaze palsy with inability to abduct the left eye and to adduct the right eye past the midline was also noted. By Krimsky estimation, 40 prism diopter (PD) left esotropia was present in the primary position. Brain magnetic resonance imaging carried a yeas ago showed a large lobulated hyperdense lesion and the central lesion is on the left dorsal portion of pons. RESULTS: The patient underwent a right lateral rectus muscle recession of 5 mm, a left medial rectus muscle recession of 6 mm and a parial tendon transfer of the left superior and inferior rectus muscles th the left lateral rectus muscle insertion (modified Hummelsheim operation with augmentation). A lateral tarsorrhaphy was also performed. 3 months after operation, he didn't complain diplopia and orthotropia was noted in the primary position by Hirshberg test. His head posture was also right. The lagophthalmos and keratitis was markely improved and ocular pain was not complained. CONCLUSIONS: The lesion in this patient caused by potine hemorrhage may be involve the left PPRF, the left sixth nerve nucleus and the left facial nerve fascicles. Strabismus surgery on the patient improved diplopia and face turn.

A Case of Bilateral Horizontal Gaze Palsy due to Pontine Hemorrhage

PURPOSE: To report a case of bilateral horizontal gaze palsy associated with pontine hemorrhage. METHODS: A 51-year-old man developed a sudden decrease in consciousness. Computed tomography reveal a pontine hemorrhage and infarctions of both basal ganglia. The mentality was recovered after conservative treatment, but he complained of difficulty in moving his eyes. We performed ophthalmic and radiologic evaluations to confirm a diagnosis of bilateral horizontal gaze palsy and to reveal the condition. RESULTS: On ophthalmic examination, his eye showed complete bilateral horizontal gaze palsy, but convergence and vertical eye movements were normal. Radiologic evaluation revealed a pontine hemorrhage, and the hemorrhagic area was consistent the bilateral paramedian pontine reticular formation (PPRF).

Neuro-Behcet's Disease Presenting with Foville Syndrome : A Case Report

A Foville syndrome is rare. A 46-year-old man with a 3-year history of Behcet's disease presented with a right facial palsy and a limitation in the rightward movements of the eyes. On neurologic examination, there was no movement of eyes in the right hemifield during all kinds of eye movements, including saccade, smooth pursuit, vestibulo-ocular reflex, and optokinetic nystagmus. However, all the eye movements were normal in the left hemifiled except for slowed saccades toward the right. Additional features were right infranuclear facial paresis and extensor plantar response on left side. Brain MRI revealed high signal intensity lesions in the right pons both in the dorsal tegmentum and in the basis. Neurological signs resolved gradually with steroid treatment. Different clinical features between abducens nucleus syndrome and paramedian pontine reticular formation syndrome are briefly discussed. To the authors' knowledge, this is the first case report of a Foville syndrome caused by neuro-Behcet's disease.