Isolated nuclear oculomotor nerve syndrome due to mesencephalic hematoma

Unilateral third nerve palsy with bilateral superior rectus paresis and bilateral ptosis is a typical condition for nuclear oculomotor nerve syndrome. We report a case of nuclear oculomotor nerve syndrome due to midbrain hemorrhage, as a rare cause. A 73-year-old man presented with an abrupt onset of double vision and difficulty opening his eyes. He had uncontrolled hypertension in his history. Neurological examination revealed right oculomotor palsy with impairment of bilateral upward gaze and bilateral ptosis. MRI showed a mesencephalic area of increased T1 signal and decreased T2 signal consistent with a subacute hematoma. It is emphasized that isolated mesencephalic hemorrhage may be the cause of the nuclear oculomotor nerve syndrome without associated neurological signs.     

Bilateral ptosis and upgaze palsy with right hemispheric lesions

Bilateral ptosis is reported with unilateral hemispheric lesions, suggesting partial lateralization of the control of the levator palpebrae superioris. There is a tight synkinesis between vertical eye and eyelid movements, but a similar, lateralized control of vertical gaze has not been previously described. We report 3 patients with right hemispheric infarctions, in whom bilateral ptosis was accompanied by impaired upward gaze. We postulate that this laterlization of ocular motor function reflects the special contribution that the nondominant hemisphere makes to attention.     

Tonic downward and inward ocular deviation ipsilateral to pontine tegmental hemorrhage

A 61-year-old man presented with coma and left hemiparesis. He was found to have tonic downward and inward deviation of the right eye, and a right lateral gaze palsy. He also had occasional downward bobbing movements of the right eye, and a partial bilateral upgaze paresis. CT showed a right pontine tegmental hemorrhage extending to the ipsilateral midbrain. Tonic ocular downward and inward deviation associated with pontine tegmental hemorrhage may be due to irritation of mesencephalic downgaze and convergence centers by rostral extension of the hematoma. Although 'eyes seeming to peer at the tip of the nose' is characteristic of thalamic hemorrhage, it may also be seen secondary to pontine tegmental hemorrhage.     

A case of midbrain and thalamic infarction showing Collier's sign in one side and blepharoptosis in the other side

A 68-year-old man was admitted to the hospital complaining of aphasia and right hemiparesis. Neurological examination revealed aphasia, right hemiparesis and ataxia in the left upper extremity. He showed striking ocular symptoms: intense retraction of the right eyelid (Collier's sign), complete ptosis of the left eyelid which might conceal Collier's sign, marked upward gaze palsy and slight downward gaze palsy. Vertebral angiography and MRI revealed an infarction in the left medial area and the left crus cerebri in the upper midbrain, the left subthalamic area inferomedially extending from the adjacent area to the posterior commissure and the medial area in the left thalamus resulting from occlusion of the left mesencephalic artery. From these MRI findings and the reports on pathological findings of patients with Collier's sign, we suggest that damage in the posterior commissure or its adjacent area may be responsible for the sign. Upward gaze palsy is often associated with Collier's sign because fibers mediating upward gaze may decussate in the posterior commissure. Third nerve palsy is also associated with the Collier's sign in some patients. The mechanism creating Collier's sign may be a disturbance of inhibitory fibers leading to the motoneuron pool of the levator muscle in the posterior commissure or its adjacent area.     

A case of left third nerve palsy and contralateral vertical gaze palsy with medial midbrain infarction]

An 81-year-old man developed oculomotor nerve palsy of the left eye and vertical gaze palsy of the right eye due to left medial midbrain infarction. His left eyelid was ptotic and the pupil was dilated. His right eye showed normal horizontal movement and Bell's phenomenon was preserved although the oculocephalic reflex was incomplete. There were no other abnormal neurological findings. The brain MRI revealed a high-intensity lesion in left medial midbrain on T2 weighted image. This lesion involved the oculomotor nerve nucleus, the interstitial nucleus of Cajal, and the rostral intersititial nucleus of the medial longitudinal fasciculus (riMLF). We thought that upward gaze palsy of the right eye was resulted from the infarction of the left riMLF or disruption of the axonal collateral of upward gaze fibers in the left oculomotor nucleus. Downward gaze palsy was resulted from the damage of the downward gaze fibers before their decussation, or the damage of the left interstitial nucleus of Cajal. This case provides evidence that unilateral lesion of the midbrain could cause contralateral vertical gaze palsy.     

Supranuclear vertical gaze palsy: bilateral thalamo-mesencephalic lesions demonstrated by MRI

The exact location for the major control center of the vertical gaze, which is referred to as rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), has been documental in a few autopsy studies. However, CT in some of the case reports of vertical gaze palsy have not clearly delineated this nucleus and its nearby complex anatomical structures. Discrete small, bilateral, symmetric infarcts in the thalamo-mesencephalic junction were demonstrated by MRI in a 59-year-old female patient who had sustained vertical gaze palsy over a period of nine years. Neurological examination revealed severe supranuclear combined downward and upward vertical gaze palsy, incomplete convergence, and slight limitation in adduction of the right eye. There was neither ptosis, pupillary disturbance, nor any further abnormalities of horizontal eye movements. The vertical vestibulo-ocular reflex was maintained, being similar to the other reported cases of vertical gaze palsy. MRI has advantages over CT in demonstrating lesions in the thalamo-mesencephalic junction in the cases of vertical gaze palsy, because clearer sagittal images can be obtained.     

Functional correlates of vertical gaze palsy and other ocular motor deficits in PSP: An FDG-PET study

ObjectiveTo determine the functional correlates of vertical gaze palsy and other ocular motor deficits in patients with progressive supranuclear palsy (PSP) using [¹⁸F]fluorodeoxyglucose (FDG-)PET.MethodsTwenty-six patients with PSP underwent clinical examination of vertical gaze combined with FDG-PET scans to assess regional cerebral glucose metabolism as a marker of neuronal activity. Of these, eighteen PSP patients were also investigated by electrical nystagmography to determine horizontal ocular motor deficits. Statistical parametric mapping analyses were performed to correlate regional neuronal activity with ocular motor functions.ResultsIn categorical comparisons, patients with downward gaze palsy showed a significantly reduced glucose metabolism in bilateral anterior cingulate gyrus and right lingual gyrus compared to those without downward gaze palsy. Maximum velocity of horizontal saccades was positively correlated with glucose metabolism of the rostral vermis and lingual gyrus; regional metabolism of oculomotor vermis was associated with peak velocity of the optokinetic reflex. Analysis of smooth pursuit eye movement amplitude and peak velocity of corrective saccades showed positive correlation with metabolism in bilateral inferior parietal lobe and inferior part of the frontal eye field. All paradigms of smooth pursuit showed positive association with glucose metabolism in V5.ConclusionsOcular motor functions in PSP are correlated with neuronal activity in distinct anatomical regions. These include the anterior cingulate gyrus (downward gaze palsy), rostral cerebellum (saccades), oculomotor vermis (optokinetic reflex) and inferior parietal as well as temporal regions and frontal eye field (smooth pursuit). These findings provide a deeper insight into the pathophysiology of PSP-associated ocular motor abnormalities.     

Monocular elevation paresis and contralateral downgaze paresis from unilateral mesodiencephalic infarction.

A 26 year old woman presented with monocular elevation paresis of the right eye, contralateral paresis of downward gaze, and subtle bilateral ptosis. Magnetic resonance imaging disclosed a unilateral embolic infarction restricted to the mesodiencephalic junction involving the left paramedian thalamus. Preserved vertical oculocephalic movements and intact Bell's phenomenon suggested a supranuclear lesion. This rare "crossed vertical gaze paresis" results from a lesion near the oculomotor nucleus affecting ipsilateral downward gaze and contralateral upward gaze fibres, originating in the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF).     

Diagnostic usefulness of a hot test in patients with mild ocular myasthenia gravis

The diagnosis of myasthenia gravis (MG) can usually be made on the basis of the characteristic clinical history and signs, improvement by the use of anticholinesterase drugs, decremental responses in repetitive nerve stimulations, and assay of anti-acetylcholine receptor (AchR) antibody titers. We, however, have difficulty to make diagnosis of ocular MG patients with mild symptoms because muscular weakness is minimal and ancillary tests are negative. In the present communication, we report clinical usefulness of a hot test to provoke ptosis by warming the eyelid in ocular MG patients with minimal fatigability. Patient 1, a 27-year-old housewife, developed drooping of the right upper eyelid in May 1985. The ptosis was absent in the morning, but became apparent and worsened later. Neurological examination carried out 3 months after the onset revealed mild right ptosis, but fatigability of the levator palpebrae superioris could not be elicited by the provocative procedures such as sustained upward gaze or repeated opening and closing of eyelids. Both Tensilon and cold tests yielded negative responses. Repetitive nerve stimulations produced no decremental responses. Titers of anti-AChR antibody and antistriational antibody were within normal limits. In order to find a possible neuro-muscular blockade, we warmed the right upper eyelid by applying hot water of about 45 degrees C in a vinyl bag for 3 minutes. The hot test worsened the right ptosis and induced mild left ptosis. Tensilon administration reversed the eyelids to the previous position. Patient 2 was a 12-year-old boy with a typical history and clinical signs of ocular MG. His symptoms remitted spontaneously without any medication 3 weeks after the onset.(ABSTRACT TRUNCATED AT 250 WORDS)     

Ocular motor abnormalities in progressive supranuclear palsy

Eleven patients, 7 males and 4 females, of progressive supranuclear palsy (PSP) were examined neuro-otologically for the purpose of elucidating the characteristics of ocular motor abnormalities. All cases were admitted to our hospital and age at onset was from 52 to 71 years old, duration of illness was 2 to 11 years. Range of voluntary eye movements and abnormal eye movements including nystagmus were examined on naked eyes and with electronystagmography (ENG). Smooth pursuit movements and saccadic eye movements were tested both horizontally and vertically by using visual tracking method with ENG recordings. Optokinetic nystagmus test and caloric test with visual suppression test were also performed. These neurotological examinations were made repetitively in 5 cases and their progressions were observed. Vertical gaze palsy and convergence palsy were observed in all cases as the initial symptom. In this study downward gaze was more severely disturbed than upward gaze. Using ENG, saccadic eye movements (saccades) were disturbed earlier than smooth pursuit movements. Hypometric saccades and decreased saccadic velocity were common abnormalities. In the later stage of the disease, horizontal eye movements were also disturbed. In four cases bilateral adduction palsy was added to vertical gaze paralysis so that the lesion of the MLF to oculomotor nucleus was suggested to exist. These voluntary eye movements were worsened gradually as the disease progressed. By using ENG we could find so called abnormal eye movements more frequently than the previous reports. Eight patients demonstrated horizontal gaze nystagmus, and rebound nystagmus were observed in four cases.(ABSTRACT TRUNCATED AT 250 WORDS)     

Bilateral palpebral ptosis with unilateral paralysis of upward gaze: a case report

The authors report a case of bilateral ptosis with miosis, paralysis of upward gaze and of smooth pursuit movements to the right, as well as a slowly regressive state of lassitude. This syndrome followed a haemorrhagic brain stem infarction. After a review of the pathophysiology of ptosis and of vertical eye movements, together with analysis of data provided by computerized tomography studies--since the case was not fatal--explanations are offered for each clinical sign and it is proposed that the lesion, a paramedian, slightly deviated to the right haemorrhagic focus in the midbrain, with a diameter of 15 mm, might have involved several structures, namely the rostral interstitial nucleus of the medial longitudinal fasciculus, the mesencephalic reticular formation, as well as cortico-nuclear and sympathetic pathways.     

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.     

An unusual variant of the dorsal midbrain syndrome in MS: clinical characteristics and pathophysiologic mechanisms

Patients with MS exhibit a broad diversity of ocular motor syndromes. We describe a patient with relapsing-remitting MS who developed an unusual variation of the dorsal midbrain syndrome, characterized by monocular convergent-retraction nystagmus in the right eye, accompanied by divergent-retraction nystagmus in the fellow eye upon attempted upward gaze. Examination also revealed a skew deviation with a left hyperdeviation and severe adduction limitation in the left eye during attempted right gaze. We propose that a left INO accounted for the inability of the left eye to adduct (and result in convergent-retraction) during attempted upward saccades. We consider the pathophysiologic mechanisms responsible for our observations and review important details of the dorsal midbrain ocular motor circuitry.     

Isolated nuclear oculomotor nerve palsy due to mesencephalic infarction

A patient with unilateral nuclear oculomotor palsy due to midbrain infarction is described. A 46-year-old man was admitted because of difficulty in opening right eye and double vision noticed when he awoke in that morning. On admission, neurological examination revealed total right oculomotor palsy with slight impairment of left upward gaze. There were no other neurologic abnormalities at all. Brain CT and cerebral angiograms were also normal. Magnetic resonance imaging (MRI) performed on the ninth day, however, demonstrated high signal intensity in the right tegmentum of the mesencephalon on T2-weighted images, which was shown more clearly after the administration of Gadolinium-DPTA. He was diagnosed as nuclear third nerve palsy caused by midbrain infarction. The majority of isolated oculomotor nerve palsy has been reported to be caused by extraaxial lesion. When the oculomotor palsy is caused by intraaxial ischemic lesion, it is usually accompanied by other brain stem signs, because abundant nuclei and fibers are present adjacent to the oculomotor nucleus and nerve in the mesencephalon. The present case clarified that such a small infarct disclosed only by MRI can cause isolated oculomotor nerve palsy. It is emphasized that the intraaxial ischemic lesion should be ruled out by using the sophisticated diagnostic aid before making diagnosis of peripheral lesion. This is the first report of the isolated third nerve palsy resulting from mesencephalic ischemic lesion in the Japanese.     

Ptosis in patients with hemispheric strokes

BACKGROUND: Cerebral ptosis is considered rare; it has been reported with unilateral, usually right hemispheric lesions. However, the frequency of cerebral ptosis in patients with strokes has not received systematic study. OBJECTIVE: To determine the frequency of ptosis in patients with acute hemispheric stroke and to identify stroke features associated with ptosis. METHODS: Eyelid function was studied in 64 consecutive patients with acute hemispheric stroke and 40 age-matched subjects with no known neurologic disease. All underwent comprehensive neuro-ophthalmologic and general neurologic examination within 48 hours of admission, including measurement of palpebral fissures, marginal reflex distance, and range of upper lid movement. Only patients who could cooperate with eyelid testing were included. Brain CT scans were obtained for all patients who had had strokes. RESULTS: Twenty-four (37.5%) of the patients with strokes had neurogenic ptosis, which was bilateral in 10 and unilateral in 14. None of the control subjects had neurogenic ptosis. All patients with strokes with ptosis had a hemiparesis. Rightward gaze deviation and upgaze paresis were more common (p < 0.05) in the patients with ptosis compared with others who had had strokes. CT evidence of right-sided hemispheric cortical infarction was more common in patients with strokes with ptosis (p < 0.05). In five patients with large hemispheric infarction, complete bilateral or asymmetric ptosis was the first sign of imminent herniation, preceding pupillary dilation and ocular motor deficits. CONCLUSIONS: Ptosis occurs frequently in patients with hemispheric strokes, especially in association with right hemispheric lesions. Complete bilateral ptosis is usually caused by large infarctions and may be a premonitory sign of an impending herniation.     

Bilateral ptosis, bilateral upgaze and adduction paresis, and monocular downgaze paresis from a mesencephalic infarction]

We report a 57-year-old man with an ischemic lesion in the midbrain. In the acute stage, he presented with bilateral ptosis and markedly limited extraocular motion except for bilateral abduction and downward motion of the right eye. The pupillary reaction to light of his left eye also was impaired. He was admitted to our hospital, and brain MRI showed a small infarction extending from the left paramedian to the median tegmentum of the midbrain. Three weeks after admission, the ptosis and limited extraocular right eye motion had resolved. The pupillary reaction and downward motion of the left eye normalized gradually within 3 weeks. Two months after admission, ptosis and the limited left eye adduction were partially resolved, but the markedly limited upgaze of the left eye had not changed. Initial neuro-ophthalmologic findings suggested involvement of the caudal part of the oculomotor nucleus and the left oculomotor nerve within the midbrain. The pattern of neuro-ophthalmologic impairment seen in our patient led us to conclude that the caudal oculomotor nucleus and medial part of the fascicular fibers of the left oculomotor nerve probably recovered first, after which recovery of the fascicular fibers progressed laterally. The results of serial MRI were consistent with this interpretation.     

Bilateral ophthalmoplegia: an unusual sign of the top of the basilar artery syndrome

Bilateral ophthalmoplegia may be an unusual sign of vertebrobasilar ischemia. We report the cases of two patients (75 and 73 years old), who suddenly developed drowsiness, bilateral ophthalmoplegia with bilateral ptosis and mild right hemiparesis. In both patients, MRI revealed bilateral thalamic and midbrain infarcts, ECG showed the presence of atrial fibrillation and Doppler study of the extracranial and intracranial vertebral arteries found no significant alterations. Ischemia involving the midbrain and thalamic paramedian regions may cause bilateral ophthalmoplegia and consciousness disturbances. In these two cases, the most plausible etiologic mechanism was cardiac embolism, and the prognosis for bilateral ocular palsy was poor.The authors thank the Italian National Research Council (CNR) for its financial support for this study.     

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.     

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.     

Specificity of "peering at the tip of the nose" for a diagnosis of thalamic hemorrhage

BACKGROUND: Tonic inward and downward deviation of the eyes ("peering at the tip of the nose") is regarded as a unique feature of thalamic hemorrhage, but the mechanisms of this ocular finding remain obscure. OBJECTIVES: To describe 4 patients who showed tonic inward and downward deviation of the eyes from brainstem or thalamic lesions and to discuss the possible mechanisms involved. DESIGN: Case report. SETTING: Secondary and tertiary referral hospitals. RESULTS: One patient developed alternating esotropia with downward ocular deviation from thalamic hemorrhage compressing the midbrain. Two patients showed multiple infarctions in the territory of the posterior circulation with or without the involvement of the thalamus. Another patient had lateral pontine hemorrhage extending up to the midbrain tegmentum. Ocular bobbing preceded or accompanied tonic ocular deviation in 3 patients. CONCLUSIONS: Tonic inward and downward deviation of the eyes may develop in thalamic or brainstem lesions. Irritation or destruction of the neural structures involved in the vergence and vertical gaze may cause this ocular sign in mesodiencephalic lesions. Skew deviation and esotropia from abduction deficit may be involved in some patients. Ocular bobbing and tonic downward deviation may share a common pathogenesis.