A case of post-infectious wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome]

The patient is a 72-year-old man who had tonsillitis and underwent incision on March 6, 1998. He complained headache and nausea from March 10 and double vision from March 12. He was admitted to our hospital on March 14. Neurological examination on March 18 revealed bilateral internuclear ophthalmoplegia with exotropia (WEBINO), bilateral ptosis and disturbance of convergence. Lumbar puncture revealed moderately elevated protein (46 mg/dl) with normal pressure and cells. The T2-weighted images of head MRI showed multiple high intensity lesions in anterior commissure and right pons. After he was treated with methylprednisolone, his headache and ptosis disappeared. The lesion in anterior commissure on MRI disappeared also. But WEBINO remained at the time of discharge. WEBINO syndrome is caused by lesion of pons and midbrain. The most common cause of bilateral internuclear ophthalmoplegia is multiple sclerosis in the European and American countries, but the frequency of vascular origin is higher in Japan than in the European and American countries. All the cases of WEBINO syndrome reported in Japan are vascular origin. This is the first case of WEBINO syndrome not of vascular origin.     

Wall-eyed bilateral internuclear ophthalmoplegia from lesions at different levels in the brainstem.

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is a rarely reported syndrome. There is dispute about whether WEBINO is caused by a pontine or a midbrain lesion and whether the medial rectus subnuclei are implicated. In a study of the clinical and imaging features of four patients with WEBINO, we found that that three of four lesions involved the midbrain but not necessarily the medial rectus subnuclei.     

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome in a patient with neuromyelitis optica spectrum disorder and anti-aquaporin-4 antibody

This report describes, for the first time, an occurrence of wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) in a 19-year-old female with neuromyelitis optica (NMO) spectrum disorder, who had anti-aquaporin-4 (AQP4) antibody. A high signal intensity lesion on T2-weighted MRI was detected in the midbrain tegmentum adjacent to the aqueduct, and presumably involved the medial longitudinal fasciculus bilaterally at the caudal levels. Plasma exchange resolved both WEBINO syndrome and the midbrain lesion. Although WEBINO syndrome is occasionally reported in multiple sclerosis patients, diagnosis of NMO should not be excluded in patients with WEBINO syndrome, because AQP4 is expressed abundantly around the periaqueductal region.     

MRI characteristics of the MLF in MS patients with chronic internuclear ophthalmoparesis

OBJECTIVE: The authors imaged the medial longitudinal fasciculus (MLF) in 58 patients with MS and chronic internuclear ophthalmoparesis (INO) to determine which MRI technique best shows the characteristic lesion associated with this ocular motor syndrome. METHODS: Using quantitative infrared oculography, the authors determined the ratios of abduction to adduction for velocity and acceleration, to confirm the presence of INO and to determine the severity of MLF dysfunction in 58 patients with MS and INO. Conventional MRI techniques, including proton density imaging (PDI), T2-weighted imaging, and fluid-attenuated inversion recovery (FLAIR) imaging, were used to ascertain which technique best shows MLF lesions within the brainstem tegmentum. T1-weighted imaging was performed to determine the frequency of brainstem tegmentum hypointensities. RESULTS: All patients studied had evidence of an MLF lesion hyperintensity on PDI, whereas T2-weighted imaging and FLAIR imaging showed these lesions in 88% and 48% of patients, respectively. With PDI, dorsomedial tegmentum lesions were seen in the pons in 93% of patients and in the midbrain of 66% of patients. Lesions were observed at both locations in 59% of patients. One patient had an MLF lesion with a corresponding T1 hypointensity. CONCLUSIONS: PDI best shows the MLF lesion in patients with MS and INO.     

A Case Report of Wall-Eyed Bilateral Internuclear Ophthalmoplegia with Bilateral Facial Palsy

Case reports of bilateral facial palsy with horizontal gaze restriction are rare. A 62-year-old woman experienced sudden onset of bilateral adduction deficits, bilateral abducting nystagmus accompanied with facial diplegia. We confirmed acute ischemic stroke in the midline dorsal pons, where medial longitudinal fasciculus (MLF) and facial nerve fascicles are located. This can be explained by vascular variation of pontine perforating arteries.     

“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.     

Brainstem infarcts presented medial longitudinal fasciculus (MLF) syndrome and cerebellar ataxia--report of three cases

Vascular disorders of the brainstem manifest characteristic neurologic symptoms according to their localization. The medial longitudinal fasciculus (MLF) lies in the paramedian portion of the upper brainstem tegmentum, and the lesion in this fasciculus shows specific abnormality of the eye movement pattern which is known as the MLF syndrome. Although the MLF syndrome was reported frequently to be accompanied by varieties of other neurologic deficits according to the lesion extending into the adjacent structures in the brainstem, the combination of MLF syndrome and cerebellar ataxia has attracted less attention of neurologists. Recently we experienced three cases of cerebellar ataxia arisen simultaneously with MLF syndrome. Magnetic resonance imaging (MRI) tomography or X-ray computed tomography visualized lacunar lesion(s) in the upper brainstem tegmentum in all of three cases. It was obvious that the lesion included the MLF and caused the MLF syndrome in these cases. It was speculated that ventro-caudal extension of the lesion involved the decussation of the superior cerebellar peduncle, and was responsible for the association of cerebellar ataxia with MLF syndrome. According to the recent discussion about the feeding arteries in the brainstem, we decided that the damage of the paramedian branches diverging from the basilar tip resulted in the lacunar lesion(s). The development of diagnostic procedures especially MRI enables the diagnosis of the vascular disorders in the brainstem with considerable accuracy.     

A variant of WEBINO syndrome after top of the basilar artery stroke

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is an uncommon neuro-ophthalmologic syndrome consisting of both eyes primary position exotropia and bilateral internuclear ophthalmoplegia. It is thought to be caused by medial midbrain lesions involving both bilateral medial longitudinal fasciculi and medial rectus subnuclei. We report the clinical and neuroimaging findings of a WEBINO syndrome associated to bilateral ptosis, non-reactive mydriasis and complete vertical gaze palsy in a 55-year-old man who suffered a top of the basilar artery stroke causing tegmental midbrain infarction.     

One and a half syndrome and MRI--a case report and functional neuroanatomy of the syndrome

This is a case report of 63 year old men who presented one and a half syndrome with ipsilateral peripheral type facial palsy due to lacunar infarct. MRI taken one week after the onset (TR 2000, TE 38) demonstrated small high intensity lesion, 4 mm in diameter, located at the dorsal portion of pontine tegmentum, contacting with the floor of 4th ventricle. This MRI coincides with the lesion limited to the abducens nucleus and genu of facial nerve. Traditionally, projections from the PPRF to the ipsilateral abducens nucleus and opposite MLF was postulated. Recently, however, experiment on monkey and autopsy cases showed projection from abducens nucleus, instead of PPRF, to the opposite MLF has been proposed. MRI findings in this case support the latter hypothesis. It is expected that, with the advent of MRI, more meticulous functional neuroanatomy will be developed.     

Ataxic hemiparesis with one and half syndrome

We report a case in whom clinical entity of ataxic hemiparesis (AH) is associated with one and half syndrome. The later finding favours a lesion in the dorsal pons while a lesion in the ventral pons will cause AH. Computerised axial tomography (CT) showed a pontine infarction over right side. The patient recovered within two weeks of onset in spite of diffuse (ventral and dorsal) pontine involvement.     

Wall-eyed bilateral internuclear ophthalmoplegia in central nervous system cryptococcosis.

Only one case of wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) has been described in central nervous system cryptococcosis. The disorder was initially unilateral, then became bilateral with skew deviation and vertical upgaze deficit. We report a case of WEBINO in central nervous system cryptococcosis in a patient with acquired immune deficiency syndrome. Magnetic resonance imaging revealed high signal on T2 images in the right midbrain, left frontal vertex, left splenium, and cerebellum. With treatment, the internuclear ophthalmoplegia improved; however, the convergence insufficiency remained. Disruption of input from cortical supranuclear locations or the region of the rostral interstitial nucleus of the medial longitudinal fasciculus has been proposed as a mechanism in the absence of convergence. This correlates in our patient with the lesions seen on magnetic resonance images.     

Paraneoplastic cerebellar degeneration associated with thymic germinoma

We present a 69 year-old man with hypertension who developed the sudden onset of horizontal binocular diplopia and stuttering of speech. On examination, bilateral exotropia (i.e. 'wall-eyed') was observed in the primary position. Attempted horizontal saccades revealed bilateral internuclear ophthalmoplegia; all consistent with the wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome. Convergence, vertical saccades and vestibular ocular reflexes were likewise impaired. Pupillary and levator palpebrae superioris functions were intact. Mild left-sided dysmetria, intention tremor and dysdiadochokinesia were elicited. Conspicuously, further characterization of the patient's history revealed that he had stuttered as a child, but it had resolved in adolescence. Brain MRI revealed an acute infarction of the mesencephalic and upper pontine tegmentum involving the periaqueductal gray region and the medial longitudinal fasciculus bilaterally with greater involvement of the left. Like the WEBINO syndrome, re-emergent developmental stuttering is a rare neurologic phenomenon. To our knowledge, this is the first case report of a mesencephalic and upper pontine infarction causing both syndromes. We discuss the pathobiological underpinnings of the WEBINO syndrome and neurogenic stuttering and in relationship to this unusual case.     

Bilateral internuclear ophthalmoplegia in association with basilar artery occlusive disease

A 56 year-old man presented with vertigo and the right sided weakness. Neurological examination revealed a lethargic man with good orientation to three spheres. His neck was supple. He had anisocoria, the right pupil being larger than the left by 1.5 mm with sluggish light reaction bilaterally. He had exotropia of the right eye in primary gaze. The abduction of both eyes were full with terminal horizontal nystagmus. The adduction of both eyes were quite limited in each eye. He had a limited upward gaze with poor convergence. These were interpreted as the syndrome of the medial longitudinal fasciculus (MLF) bilaterally. He had a depressed gag reflex on the right side with tongue deviation to the right. He had a mild weakness of the right side limb and also had the right sided hemihyperesthesia including his face to pain and temperature. Twenty four hours after the onset, the left brachial angiography revealed a complete occlusion of the rostral portion of the basilar artery without visualization of the posterior cerebral and superior cerebellar arteries bilaterally. CT scans three days after the onset revealed a low density area in the mid pons with extension rostrally up to the mesencephalon. Four days later he became quadriplegic with bilateral horizontal gaze palsy. No more internuclear ophthalmoplegia is noted on both sides. The midline location of the MLF in the pons, and the separate blood supplies by different paramedian branches of the basilar artery, form the anatomical explanation for the frequent unilaterality of vascular and bilaterality of demyelinating lesions. Bilateral MLF syndrome has been considered almost pathognomonic of multiple sclerosis.(ABSTRACT TRUNCATED AT 250 WORDS)     

The trajectory of the medial longitudinal fasciculus in the human brain: A diffusion imaging‐based tractography study

The aim of this study is to investigate the trajectory of medial longitudinal fasciculus (MLF) and explore its anatomical relationship with the oculomotor nerve using tractography technique. The MLF and oculomotor nerve were reconstructed at the same time with preset three region of interests (ROIs): one set at the area of rostral midbrain, one placed on the MLF area at the upper pons, and one placed at the cisternal part of the oculomotor nerve. This mapping protocol was tested in an HCP‐1065 template, 35 health subjects from Massachusetts General Hospital (MGH), 20 healthy adults and 6 brainstem cavernous malformation (BCM) patients with generalized q‐sampling imaging (GQI)‐based tractography. Finally, the 200 μm brainstem template from Center for In Vivo Microscopy, Duke University (Duke CIVM), was used to validate the trajectory of reconstructed MLF. The MLF and oculomotor nerve were reconstructed in the HCP‐1065 template, 35 MGH health subjects, 20 healthy adults and 6 BCM patients. The MLF was in conjunction with the ipsilateral mesencephalic part of the oculomotor nerve. The displacement of MLF was identified in all BCM patients. Decreased QA, RDI and FA were found in the MLF of lesion side, indicating axonal loss and/or edema of displaced MLF. The reconstructed MLF in Duke CIVM brainstem 200 μm template corresponded well with histological anatomy. The MLF and oculomotor nerve were visualized accurately with our protocol using GQI‐based fiber tracking. This GQI‐based tractography is an important tool in the reconstruction and evaluation of MLF.     

Internuclear ophthalmoplegia and cerebellar ataxia: report of one case.

Deep hemispheric or brainstem small infarcts can lead to atypical lacunar syndromes. Unilateral internuclear ophthalmoplegia (INO) and cerebellar ataxia has not been reported previously. A 57-year-old hypertensive female presented with bilateral appendicular and left truncal cerebellar ataxia and right INO. Cranial MRI showed a right paramedian infarct of lacunar size located in the tegmentum of caudal mesencephalon. At this level the involvement of medial longitudinal fascicle (MLF) led to right INO and the lesion of brachium conjunctivum caused the bilateral cerebellar ataxia. Ipsilateral involvement of both cerebellofugal fibers, before and after decussation, was responsible for bilateral cerebellar ataxia.     

Hallucinose pédonculaire with left MLF syndrome caused by midbrain infarction--a case report]

A 65-year-old male suffering from left MLF syndrome accompanied by transient hallucinose pédonculaire caused by the midbrain infarction is reported. In the first night of his admission with MLF syndrome, he complained of sleeplessness followed by transient visual hallucination. The hallucination was vivid and colored scene in which many strangers were seen around the patient, and he was well oriented without dreadfulness at that time. A small infarction in the left tegmentum was revealed by MR imaging, which was supposed to cause the hallucination.     

Capsular ataxic tetraparesis

A 42-year-old man with ataxic tetraparesis is reported. Computerized tomography showed bilateral and symmetrical lacunar infarcts at the junctions of the posterior limb of the internal capsule and the corona radiata. Previously this clinical syndrome had been reported only with a lesion in the pons.     

Origin and distribution of brain-stem somatosensory evoked potentials in humans.

The distribution of somatosensory evoked potentials (SEPs) recorded from the brain-stem surface was studied to investigate their generator sources in 14 patients during surgical exploration of the posterior fossa. Two distinct SEPs of different morphologies and electrical orientation were obtained by median nerve stimulation. A small positive-large negative-late prolonged positive wave was recorded from the cuneate nucleus and its vicinity. There was a phase-reversal between the cuneate nucleus and the ventral surface of the medulla, depicting a dipole for dorso-ventral organization. From the pons and midbrain, triphasic waves with predominant negativity were obtained. This type of SEP had identical wave forms between the dorsal, lateral and ventral surface of the pons and midbrain. It showed an increase in negative peak latency as the recording sites moved rostrally, suggesting an ascending axial orientation. In a patient with pontine hemorrhage, the killed end potential, a large monophasic positive potential was obtained from the lesion. This potential occurs when an impulse approaches but never passes beyond the recording electrode. Therefore, the triphasic SEP from the pons and midbrain reflects an axonal potential generated in the medial lemniscal pathway.     

The course of direct projections from the abducens nucleus to the contralateral medial rectus subdivision of the oculomotor nucleus in the cat

We have used autoradiography (tritiated leucine) to investigate the projections of a number of nuclear groups of the cat pons. Some cells of the abducens nucleus have axons that cross the midline, ascend in the opposite median longitudinal fasciculus (MLF) and synapse on the cells of the oculomotor complex which have been identified by others as those innervating the medial rectus muscle.     

The correlation between vertebral artery asymmetry and pontine infarction--an MR angiography study]

The purpose of this study is to evaluate the correlation between variation of the vertebral artery (VA) and the incidence of pontine infarction. A total of 206 patients were examined using magnetic resonance imaging (MRI) and 3-dimension time-of-flight MR angiography (MRA) of the brain. Of these, 54 patients had pontine infarctions (23 symptomatic and 31 asymptomatic), and the majority of them were located in the pontine base. The sites of dominant lesion in the pons were right in 18 cases, left in 8 cases, and bilateral in 28 cases. The number of patients with VA asymmetry (the ratio of internal diameters 1:2 or more) were 89 (43.2%). Of these, 67 patients had small diametric VA of right side, and 22 of left side. Among the 117 patients with normal VA pattern, 19 (16.2%) had infarction, while among the 89 patients with VA asymmetry, 35 (39.3%) had infarction. The patients with small diametric VA of right side significantly had infarctions in the same side of the pons. The results of this study suggest that VA asymmetry is considered to be one of the risk factors of pontine infarction and that MRA can be useful in the examination of the cerebral artery as a valuable and non-invasive screening method.