Abducens neuromyotonia due to internal carotid artery aneurysm

Ocular neuromyotonia refers to paroxysmal involuntary contraction of one or more ocular muscles resulting in paroxysmal diplopia and strabismus. A 73-year-old woman reported spells of horizontal diplopia that usually developed after leftward gaze and mostly lasted less than one minute. Between the episodes, the extraocular movements were normal without aberrant regeneration. The diplopic paroxysms could be triggered by leftward gaze for several seconds. During the episode, the left eye was exotropic with mild adduction limitation. Brain imaging revealed an aneurysm in the left internal carotid artery. The episodes resolved with carbamazepine. Aneurysm of the internal carotid artery is a rare cause of abducens neuromyotonia. A compressive lesion may give rise to ocular neuromyotonia even in the absence of prior irradiation.     

Bilateral oculomotor paralysis due to neurosyphilis

Bilateral third nerve paresis attributed to neurosyphilis has not been documented in the absence of associated neurological deficits. We describe a patient with isolated bilateral third nerve paresis, positive serological findings in blood and cerebrospinal fluid, CSF pleocytosis, and increased protein content. Resolution of these abnormalities occurred when the patient was treated with penicillin.     

A case of partial fascicular oculomotor paresis caused by midbrain infarction]

We report a 74-year-old man with an ischemic lesion in the ventral midbrain. He presented with contralateral ptosis and marked upward gaze paresis of the right eye. Neurological examination revealed partial oculomotor nerve palsy caused by impairment of the right levator palpebrae, superior rectus and inferior oblique muscles. This finding is highly suggestive of a possible lesion in the midbrain affecting the oculomotor fascicular fibers. Magnetic resonance images showed an ischemic lesion in the paramedian area of the right midbrain tegmentum. The coronal view of T 2-weighted imaging clearly demonstrated to be the site of lesions below the red nucleus. It seemed to be coincidental with the impaired site of involving the caudal part of oculomotor fascicular fibers emerging from the nucleus. This report is considered to be a typical case of partial fascicular oculomotor paresis based on impairment of the caudal part of oculomotor fascicles for the levator palpebrae, superior rectus, and inferior oblique muscles. This is a valuable case to be documented in which neurological site of lesions are consistent with those found in radiological study.     

Case of ocular neuromyotonia with transient and gaze-induced diplopia]

A 71-year-old man had recurrent spells of transient diplopia for about four years. Vertical diplopia were induced by downward gaze, mostly to the lower-left, and lasted for about ten seconds. During the spells, limitation in elevation of the left eye was observed. Other neurological examinations were normal except for a minor paresis of the left trochlear nerve revealed only by detailed neuroophthalmological examination. The patient was treated with carbamazepine 200 mg daily, which abolished the spells. He was diagnosed to have idiopathic ocular neuromyotonia of the left third or fourth nerve. Ocular neuromyotonia is a rare disturbance of ocular motility, characterized by paroxysmal monocular involuntary spasms of one or more extraocular muscles supplied by the third, fourth or sixth cranial nerves. Past studies showed that most patients had radiation therapy to the parasellar or sellar regions. Slight injury to the peripheral nerves such as one caused by radiation may have an important role in the development of ocular neuromyotonia. Furthermore, patients with ocular neuromyotonia often benefit from the use of carbamazepine, a membrane-stabilizing agent. We report a case of idiopathic ocular neuromyotonia without history of damage to the cranial nerves. It is the first report in Japan.     

Isolated bilateral oculomotor paralysis due to a ruptured aneurysm

A patient presented with sudden headache and isolated bilateral third nerve palsy. Neuroradiologic examination disclosed a giant aneurysm of the right internal carotid artery. At postmortem examination, the aneurysm compressed and stretched both oculomotor nerves.     

Locked-in syndrome with bilateral ptosis: combination of bilateral horizontal pontine gaze paralysis and nuclear oculomotor nerve paralysis

Summary A patient with a classical locked-in syndrome is described, in whom bilateral ptosis was observed on the 2nd day. On the 4th day, ophthalmoplegia was complete with the exception of a voluntary downward movement of the right eye. The syndrome can be explained by the association of bilateral horizontal pontine gaze palsy and a unilateral nuclear oculomotor nerve palsy, and approximates the total locked-in syndrome.     

Magnetic resonance imaging in horizontal oculomotor paralysis caused by infarction

Four patients with focal brainstem ischemic strokes and various types of horizontal oculomotor disturbances have been studied clinically and radiologically. One had a six nerve palsy, one a unilateral internuclear ophthalmoplegia, one a Fisher's one-and-a-half syndrome, and one a paramedian pontine reticular formation syndrome with a sixth nerve palsy. In all patients a C.T. Scan and a Magnetic Resonance Imaging (MRI) were obtained. The MRI study was performed on a CGR Magniscan 5000 with a superconducting magnet of 0.5 Tesla. In all patients a hypersignal in T2 weighted images was shown and corresponded to the brainstem infarct. MRI allowed accurate delineation of the lesion and clinico-radiologic correlations in three patients. But the low specificity of MRI does not permit to distinguish edema from necrosis, gliosis or demyelination in a region with a pathological MRI signal. In one patient this low specificity and perhaps partial volume effects decreased the accuracy of the clinico-topographic correlation.     

颅内动脉瘤与动眼神经麻痹的相关性分析

目的比较伴发动眼神经麻痹(OMNP)与非动眼神经麻痹(NOMNP)的颅内动脉(IA)瘤患者在各临床因素之间的差异性,并为其诊断及治疗提供依据。方法收集本院神经内科及神经外科2014年1月至2016年12月连续入组的224例经DSA确诊的单侧单发IA患者的临床资料,记录以下因素:性别、年龄、首发症状、受累眼肌表现、动脉瘤部位及最大直径、是否伴发蛛网膜下隙出血(SAH)、手术方式及预后等。对IA的部位进行统计,并将其中的后交通动脉瘤按照其首发症状分为动眼神经麻痹(OMNP)组及非动眼神经麻痹(NOMNP)组,分析两组之间的差异性。结果 (1)对动脉瘤部位分析统计可知,后交通动脉(74例,33.0%)最常见;(2)NOMNP组患者发生SAH的概率(84.91%)要明显高于OMNP组(19.05%)。结论以动眼神经麻痹为首发症状的患者,需排除动脉瘤的存在。对于继发于后交通动脉瘤的动眼神经麻痹患者,给予及时的干预措施,可预防动脉瘤破裂并改善预后。     

A patient with primary Sj?gren's syndrome featuring polyneuropathy and oculomotor paralysis and associated with asymptomatic left middle cerebral artery stenosis]

We report a case of primary Sj?gren's syndrome (primary SjS) with polyneuropathy and right oculomotor paralysis associated with middle cerebral artery stenosis. A 39-year-old woman developed progressive numbness and clumsiness of the limbs. Two months later, right third cranial palsy manifested itself and she was admitted to our hospital. A cranial MRA showed left middle cerebral artery stenosis confirmed by transcranial color doppler sonography. A nerve conduction study showed a decrease in the NCV and reduced CMAP, while sural nerve biopsy showed axonal degeneration and infiltration of inflammatory cells around the small blood vessel walls. The patient complained of dry mouth and a salivary gland biopsy revealed inflammatory changes, while salivary gland scintigraphy showed diminished secretion. These findings led to the diagnosis of Sj?gren's syndrome. Reports of primary SjS with involvement of large cerebral arteries are rare. In our case, polyneuropathy and oculomotor paralysis were the manifest symptoms, but middle cerebral artery stenosis was also observed. This indicates that, even in the absence of CNS symptoms, cerebral artery involvement may be present in primary SjS.     

Hereditary paroxysmal ataxia with neuromyotonia.

The clinical manifestations of a patient with hereditary paroxysmal ataxia and neuromyotonia are described. Generalized tremor, triggered by sudden movements, and spasms of hand and foot muscles were the main clinical findings. Electromyogram (EMG) and nerve blocking studies led to the diagnosis of neuromyotonia. Treatment with acetozolamide was of no therapeutic value, confirming previous observations about the difference in response of paroxysmal ataxia with and without neuromyotonia.     

Bilateral facial paralysis]

A case of bilateral facial paralysis following meningococcal meningitis and herpes simplex infection is reported. The author discusses the differential diagnosis of bilateral facial nerve paralysis which included several diseases and syndromes and concludes by herpetic aetiology.