Cases reported "Ophthalmoplegia"

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1/50. A case of the subacute brainstem encephalitis.

    A case of brainstem encephalitis of undetermined etiology is reported in 66-year-old woman who had a sudden onset of illness with left abducens palsy, nystagmus and ataxia. The symptoms progressed to complete paralysis of eye movements, dysphagia and left hemiparesis with generalized hyperreflexia. Examination of CSF, CT scan and MRI of the brain were normal. The patient died 4 months after onset of disease. Neuropathologic study disclosed in the brainstem numerous perivascular and nodular inflammatory cell infiltrations composed predominantly of lymphocytes T and B. Most intensive inflammation concerned midbrain and pontine tegmentum and to a lesser degree medulla oblongata, pontine nuclei and cerebellar nuclei. basal ganglia, cerebral and cerebellar cortex were unaffected. Neuropathological finding was reminiscent of brainstem encephalitides related to viral infection or to paraneoplastic syndrome. However, HSV-1, EBV, and CMV antigens were not detected by immunohistochemistry, as well as evidences of malignancy were not present in this case.
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2/50. Perinatally ruptured dermoid cyst presenting as congenital oculomotor palsy.

    An eight-month-old girl presented with congenital exotropia and latent nystagmus. Further evaluation revealed congenital ptosis of the left eye and restriction of the elevation, depression and adduction of the left eye. A diagnosis of congenital oculomotor palsy was made. At the age of three months she had been examined by the neurologist because of retarded psychomotor development. All laboratory investigations were normal. At the age of eight months, a CT scan of the brain and orbit was found to be normal. The patient was treated for amblyopia. At the age of five, strabismus surgery was performed, and a large fibrous tumor encapsulating the superior and lateral rectus muscle was found. A biopsy was taken and pathology showed fibrous tissue containing a hair. Based on the clinical history, the diagnosis of a perinatally ruptured orbital dermoid cyst was made. review of the previous CT and an additional CT showed enlargement of the left lateral orbital wall with a notch in the lateral wall, indicative of a dermoid cyst.
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3/50. Ocular myasthenia mimicking a one-and-a-half syndrome.

    A 52-year-old patient developed an eye movement disorder first resembling a left internuclear ophthalmoplegia and subsequently a "one-and-a-half syndrome" as the presenting symptoms of ocular myasthenia gravis. No accompanying myasthenic features were present except for the fluctuation in the amplitude of dissociated nystagmus. This patient shows that an oculomotor disorder considered a typical pontine lesion may instead be caused by myasthenia gravis, even in the absence of other clinical and electrophysiologic features of neuromuscular deficit.
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4/50. Unilateral acquired upper eyelid ptosis and decreasing nystagmus.

    PURPOSE: To describe a patient with congenital nystagmus that decreased in one eye after the development of a cavernous sinus meningioma. DESIGN: Interventional case report. methods: A 45-year-old man with congenital nystagmus, albinism, and high myopia presented with right upper eyelid ptosis that was corrected surgically on three occasions during a 3-year period. An episode of syncope was followed by diagnostic evaluation that disclosed a cavernous sinus meningioma. He commented that his right eye nystagmus progressively decreased. He was found to have ophthalmoplegia on the right eye due to nerve compression by the meningioma. RESULTS: A magnetic resonance imaging scan disclosed a cavernous sinus meningioma extending into the parachiasmal and clival areas. A biopsy specimen of the lesion demonstrated a meningioma. CONCLUSIONS: Any patient with acquired ptosis should have a complete ophthalmic examination, including evaluation of extraocular motility.
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5/50. ophthalmoplegia and dissociated nystagmus in adetalipoproteinemia.

    A characteristic pattern of acquired exotropia, progressive paresis of the medial rectus muscles, and dissociated nystagmus on lateral gaze was found in three patients with abetalipoproteinemia. Study with electronystagmography of the eye movements of one patient revealed abnormally slow voluntary saccades and slow or absent fast components of vestibular nystagmus, optokinetic nystagmus, and jerk-type, disassociated nystagmus. Defects in central nervous system centers generating saccadic eye movements are postulated.
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keywords = nystagmus
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6/50. ophthalmoplegic migraine.

    ophthalmoplegic migraine is a rare presentation of migraine complicated by an isolated oculomotor paresis. vasodilation of extracranial vessels is believed to underlie the headache, and vasoconstriction to account for the ophthalmoplegia. Whether the vascular insult involves the central or peripheral portions of the oculomotor nerve is still uncertain. We describe a child who presented with ophthalmoplegic migraine and was demonstrated to have a deficiency of the near triad documented by eye movement and pupillary recordings. Voluntary conjugate eye movements--saccades, smooth pursuit, and optokinetic nystagmus--were normal. Vergence amplitudes appropriate to fixation distance were elicited for Snellen optotypes but not to a point source of light. Concurrent measures of pupillary diameter failed to reveal significant modulation to either stimulus. brain magnetic resonance imaging scan was normal, and there was no contrast enhancement of the oculomotor nerve at its exit from the midbrain. Both the oculomotor paresis and concurrent presence of a deficiency of the near triad localized the vascular insult to the oculomotor nerve complex in the brainstem.
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7/50. Fisher's syndrome without total ophthalmoplegia.

    A 16-year-old boy with acute ophthalmoplegic polyneuritis (Fisher's syndrome) exhibited bilateral internal ophthalmoplegia, but only lateral rectus and superior oblique eye movements were affected. Gaze-evoked nystagmus was also present on left lateral gaze. Pupillary reflex recovered 7 weeks after onset, but neither cholinergic supersensitivity nor light-near dissociation were present during the recovery process. This is the first such case to be reported in which the site of the lesion responsible for the pupillomotor impairment was the Edinger-Westphal nucleus.
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keywords = nystagmus
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8/50. Internuclear ophthalmoplegia of abduction: clinical and electrophysiological data on the existence of an abduction paresis of prenuclear origin.

    Three patients showed unilateral and five bilateral abduction paresis. Five had associated adduction nystagmus of the contralateral eye. Electrophysiological testing of masseter and blink reflexes indicated an ipsilateral rostral pontine or mesencephalic lesion, and excluded a lesion of the infranuclear portion of the abducens nerve. Abduction paresis was attributed to impaired inhibition of the tonic resting activity of the antagonistic medial rectus muscle. The prenuclear origin of the disorder is based on morphological and neurophysiological evidence of an ipsilateral inhibitory connection between the paramedian pontine reticular formation and the oculomotor nucleus running close to but separated from the medial longitudinal fasciculus.
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9/50. Acute ophthalmoplegia and nystagmus in infants fed a thiamine-deficient formula: an epidemic of wernicke encephalopathy.

    In 2003, an epidemic of wernicke encephalopathy (WE) developed in Israeli infants fed a thiamine-deficient soy-based formula. Approximately 20 infants were affected out of an estimated 3500 fed the vitamin-deficient formula. The finding of gaze abnormalities in a single infant by neuro-ophthalmologists led to the unraveling of the epidemic. In this report, the findings in three infants are described. early diagnosis and treatment with parenteral thiamine led to complete neurologic recovery in two infants; in the third infant, delayed diagnosis may have been responsible for severe lingering deficits. This is the first reported epidemic of WE secondary to thiamine-deficient infant formula. early diagnosis and treatment are critical to avoid persistent neurologic impairment.
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ranking = 4
keywords = nystagmus
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10/50. Ophthalmoparesis in idiopathic intracranial hypertension.

    PURPOSE: To report unusual ocular motility disturbances in the setting of idiopathic intracranial hypertension (IIH). DESIGN: Interventional case series. methods: Two cases with IIH and unusual ophthalmopareses are reported. RESULTS: Two patients with confirmed IIH presented with headache, diplopia, and papilledema. The first patient had bilateral sixth nerve palsies and a partial right third nerve palsy, which resolved rapidly after a cerebro spinal fluid (CSF) shunting procedure; the second patient had alternating skew deviation and upbeat nystagmus. Both cases had normal brain magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) and normal CSF contents, ruling out a secondary cause of intracranial hypertension. CONCLUSIONS: These exceptional vertical and horizontal ophthalmopareses in the setting of IIH may be related directly to very elevated CSF pressures and may be secondary to altered CSF flow in the posterior fossa.
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