Acute myeloid leukaemia presenting as gaze palsy

The most frequent initial ocular manifestation of acute myeloid leukaemia (AML) is retinal involvement. Here, we report an unusual case of AML associated with a pontine chloroma presenting with gaze palsy as the initial symptom. A 77-year-old Caucasian man presented to the Eye Casualty complaining of a one-day history of blurred vision. On examination, his face was turned to the left, both eyes were fixed in dextroversion and the patient demonstrated left gaze palsy associated with left motor neurone VII palsy. Baseline blood investigations revealed leucocytosis with 60% circulating myeloblasts. A bone marrow biopsy confirmed the diagnosis of myelomonocytic leukaemia. A CT scan showed a well-circumscribed lesion in the dorsal pons, most likely representing a chloroma. Chloromas or myeloblastomas related to AML are localised extramedullary tumours composed of leukaemic myeloid cells. Chloromas may be present at the time of the initial diagnosis of leukaemia or may precede the diagnosis by 1 month to 2 years; however, their occurrence in the central nervous system is rare, comprising 1-6% of all chloromas. This case illustrates the many different ways that AML can manifest itself in the eyes, and ophthalmologists should be aware of the great variety of presenting symptoms in undiagnosed AML.     

One-and-a-half syndrome. Type II: a case with rostral brain stem infarction

This article is about a patient who suddenly developed a right hemiparesis and stupor. Her neuro-ophthalmological examination revealed a left-sided ptosis and a combined vertical gaze palsy in addition to right esotropia, right conjugate gaze palsy, and abduction paralysis of the left eye on attempted gaze to the left with adduction nystagmus of the right eye. The horizontal eye movement disorder was similar to that in one-and-a-half syndrome, except for an abduction paralysis and an adduction nystagmus. MRI showed a rostral brain stem infarction on the left side. Left-sided ptosis and adduction paralysis were attributed to a left oculomotor fascicular involvement in the presence of a right hemiparesis. Right-sided esotropia and abduction paresis were consistent with a ‘pseudo-abducens palsy’, which has been reported in thalamo-mesencephalic lesions. Combined vertical gaze palsy is a classical finding of rostral brain stem infarction. Left-sided abduction paralysis with adduction nystagmus on the right side on attempted gaze to the left is thought to be due to involvement of the para-MLF on the left side and is called internuclear ophthalmoplegia of abduction (INO-abd). To our knowledge, this type of horizontal eye movement disorder has never been reported before with rostral brain stem infarction.     

Síndrome del uno y medio de Fisher a causa de un cavernoma bulbo-protuberancial

The one and a half Fisher syndrome is produced by an unilateral lesion in the dorsal and inferior part of the pons located specifically in the pontine tegmentum. This is an area that contains the medial longitudinal fasciculus (LMF), the paramedian pontine reticular formation (PPRF) and the nucleus of the sixth cranial nerve, responsible for the horizontal movements of the gaze. Clinically it manifests with conjugate gaze palsy with internuclear ophthalmoplegia.The case is reported on a 60 year-old patient, who presented with binocular diplopia, ophthalmoplegia and paralysis of the gaze conjugated to the left. Imaging studies were performed that showed a hemi-protuberant haematoma secondary to a cavernoma. As expected, he presented with several episodes of re-bleeding with clinical worsening: headache, diplopia, tetraparesis, and hypoaesthesia in the right side of the face, as such that the microsurgical exeresis of the protuberant cavernoma was considered.     

Near Reflex Substituting for Acquired Horizontal Gaze Palsy: A Case Report

Background Some patients with acquired horizontal gaze palsy overcome the adduction palsy by utilizing convergence. This substitution phenomenon is very rare. We report a patient with horizontal gaze palsy who was able to use convergence to compensate for the lack of adduction in the left eye.Case The patient was a 31-year-old woman with an arteriovenous malformation in the fourth ventricle. She suffered right gaze palsy and right abducens palsy after tumor surgery and radiation therapy.Observations Three years after the vascular accident, she was found to be able to adduct the left eye, in association with the adduction of the right eye. At the same time, constriction of both pupils and globe retraction of the left eye were observed. When she shifted the gaze direction of her left eye from left to right, an 11 and 8 diopter increase of myopia in the right and left eyes, respectively, was confirmed by objective refractometry.Conclusions The existence of convergence substituting for adduction in this patient with horizontal gaze palsy was confirmed by refraction change in addition to pupillary change. Jpn J Ophthalmol 2004;48:584–586 © Japanese Ophthalmological Society 2004     

Regressive pattern of horizontal gaze palsy

The different phases of recovery of horizontal bilateral gaze palsy are described. The main features are a Millard Gubler syndrome homolateral to the lesion, paralysis of abduction of the contralateral eye and adducting movements of both eyes in the various directions of gaze. The lesion responsible for the symptomatology was a benign pontine hematoma localized in the tegmentum of the pons by means of Nuclear Magnetic Resonance.     

Selective and sequential therapy of oculomotor and palpebral sequelae resulting from biopsy of dorsal midbrain sarcoidosis

BACKGROUND: Biopsy of a dorsal mesencephalon lesion often leads to neuro-ophthalmological complications, namely dorsal mesencephalic syndrome, skew deviation, IV (th) palsy, or Horner's syndrome. HISTORY AND SIGNS: A 26-year-old man underwent a biopsy of the quadrigeminal plate for a lesion which revealed to be neurosarcoidosis. Post-operative complications included a left homonymous hemianopia, a left III (rd) nucleus palsy, a bilateral IV (th) palsy and a bilateral internuclear ophthalmoplegia. Palpebral fissures were narrowed to 2 mm on both sides. In primary gaze there was 24 degrees of exotropia, 17 degrees of right hypertropia and 30 degrees of exyclorotation. THERAPY AND OUTCOME: After 24 months of stable evolution, three oculomotor surgical procedures were performed including: surgery on both horizontal muscles of the left eye; shortening of both superior oblique muscles; resection of the right lateral rectus muscle, and posterior fixation of an inferior rectus muscle. Bilateral brow suspension on the frontal muscle of both superior eyelids was then performed. Evolution was favorable with a binocular single field of vision measuring 17 degrees horizontally and 20 degrees vertically centered on the primary gaze. Palpebral fissures measured 7 to 8 mm in primary gaze. CONCLUSIONS: The neuro-ophthalmological sequelae which followed the biopsy of a neurosarcoidosis lesion of the quadrigeminal plate were so severe that this patient was completely handicapped. A four-step surgery correcting the horizontal, torsional, vertical components, then the bilateral ptosis allowed the patient to recover both a satisfying function and esthetical aspect.     

Ocular myasthenia presenting as superior oblique weakness

We diagnosed ocular myasthenia in a 39-year-old man whose presenting symptom was vertical diplopia. Unrecognized weakness of the right superior oblique and secondary overaction of the right inferior oblique caused a right hypertropia, which was worse on left gaze. One week later left blepharoptosis, right medial rectus weakness, and bilateral obicularis oculi weakness were found on ocular examination. All findings were partially reversed by the injection of Tensilon. Superior oblique muscle palsy simulating a fourth cranial nerve palsy is infrequently reported in patients with ocular myasthenia. Pseudofourth nerve palsy is another sign of myasthenia gravis.     

Gaze-evoked amaurosis produced by intraorbital buckshot pellet

OBJECTIVE: To report the first case of gaze-evoked amaurosis secondary to an intraocular foreign body and to highlight the characteristic clinical findings of patients with this symptom. DESIGN: Case report and review of the literature. METHODS: Case review, clinical history, electrophysiologic testing, and follow-up. MAIN OUTCOME MEASURES: Visual acuity, automated perimetry, and visual fields. RESULTS: A case of gaze-evoked amaurosis as a result of an intraorbital foreign body is described, and 19 additional cases of gaze-evoked amaurosis are reviewed from the English language literature. These cases share certain characteristics including good vision in primary position with deterioration of vision in eccentric gaze; concurrent objective pupillary abnormalities in eccentric gaze; stereotypic onset and recovery of vision; and funduscopic abnormalities consisting of disc edema and chorioretinal folds. CONCLUSIONS: Gaze-evoked amaurosis is a reliable sign of intraconal mass lesion. We report the first case of gaze-evoked amaurosis secondary to an intraorbital foreign body.     

Surgery for Bilateral Superior Oblique Palsy

Following head trauma, because the patient has no overt vertical or horizontal tropia to account for the complaint of diplopia, the symptoms are dismissed, when in fact cyclodiplopia resulting from the excyclotropic feature of bilateral superior oblique palsy is the cause. A prospective study of nine patients with bilateral superior oblique muscle palsy caused by head trauma and managed by an identical surgical regimen are presented. All patients had symptomatic cyclodiplopia that increased in downgaze. Cover testing performed in various gaze positions and in left and right head tilt positions plus the double Maddox rod tests confirmed the diagnosis. The four expected findings are left hypertropia (LHT) in right gaze and right hypertropia (RHT) in left gaze, RHT on right head tilt and LHT in left tilt, V pattern, and excyclodeviation. All patients received symmetrical bilateral superior oblique tendon surgery, using a modified technique originally described by Harada and Ito, which consisted of advancing the anterior tendon half along the equator of the globe toward the superior border of the lateral rectus muscle. Elimination of the cyclodiplopia symptom was achieved in all cases by reducing the quantity of excyclotropia.     

Correlation of clinical and neuroradiological findings in down-gaze palsy

Background: Isolated down-gaze palsy is the least common pathology of vertical gaze. Patients with low-gaze palsy may consult an ophthalmologist with difficulty in reading and this may be the only ocular finding of a central nervous system lesion. Methods: A 43-year-old man with isolated down-gaze palsy was examined. The medical history of the patient revealed that he had had myocardial infarction. Result: Magnetic resonance imaging disclosed an ischemic area at the right thalamus. Conclusion: Down-gaze palsy may be an important sign for the diagnosis of thalamic infarctions due to embolic syndrome. Received: 1 March 1999 Revised: 15 September 1999 Accepted: 15 September 1999     

Horizontal Gaze Palsy with Progressive Scoliosis: A Case Report and Literature Review

Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare autosomal recessive disorder. The ROBO 3 gene mutation is responsible for the disease. We present a boy aged 12 years who was admitted for scoliosis surgery who had also had horizontal gaze palsy since birth. His brainstem abnormalities were compatible with the syndrome of HGPPS. HGPPS is one of the rare congenital diseases of childhood. Horizontal gaze palsy, ametropia, and progressive scoliosis are the main findings of the disease. This syndrome should be kept in mind for both ophthalmologists and orthopaedic surgeons in patients who present with gaze palsy and scoliosis. Early diagnosis of scoliosis makes it possible to treat the disease at an early stage, and early diagnosis of ametropia is important in the prevention of amblyopia.     

Abnormal ocular motility with brainstem and cerebellar disorders.

The disorders of ocular motility seen in association with brainstem or cerebellar disorders may point to rather specific anatomical or pathological correlations. Pontine gaze palsy reflects involvement of the pontine paramedian reticular formation. Internuclear ophthalmoplegia signifies a lesion in the medial longitudinal fasciculus. Skew deviation may result from a lesion anywhere in the posterior fossa. Ocular bobbing typically results from a pontine lesion. The Sylvian aqueduct syndrome is characteristic of involvement in the upper midbrain-pretectal region, usually a pinealoma. Cerebellar lesions may be manifested by gaze paresis, skew deviation, disturbances of saccadic or smooth pursuit movements, ocular myoclonus, or several characteristic forms of nystagmus. Familiarity with these disorders may be of great help to the physician dealing with a patient with a possible posterior fossa lesion.     

Cryptic disseminated tuberculosis presenting as gaze palsy

We present a case of gaze palsy caused by a pontine tuberculoma associated with cryptic disseminated tuberculosis. The only symptoms in this patient were those caused by her gaze abnormality despite the presence of disseminated tuberculosis that was later confirmed at autopsy.     

Congenital oculomotor nerve palsy, cerebellar hypoplasia, and facial capillary hemangioma.

We saw two infants with the unusual combination of oculomotor nerve palsy, facial capillary hemangioma, cerebellar hypoplasia, and apparent gaze palsy. Systematic imaging of children with congenital oculomotor nerve palsy may lead to the recognition of more associated neurologic abnormalities that are not clinically apparent, as was the case in our patients.     

Etiology and surgical management of horizontal pontine gaze palsy with ipsilateral esotropia.

INTRODUCTION: An esotropia ipsilateral to a horizontal pontine gaze palsy has been infrequently reported. We discuss the etiology and review the surgical management of this ocular motility defect. METHODS: Four patients with radiographically documented dorsal pontine lesions and persistent horizontal gaze palsy with esotropia ipsilateral to the gaze palsy were treated. In each patient, the esotropia was present in attempted primary gaze, producing symptomatic diplopia. An anomalous face turn was required to attain single binocular vision. RESULTS: All 4 patients underwent surgical correction to alleviate the anomalous head position and diplopia. Bilateral, asymmetric surgery was required to achieve a long-term successful result. Single binocular vision in the primary position with elimination or marked improvement of the compensatory head posture was initially achieved in all 4 patients. One patient, who had not undergone asymmetric strabismus surgery to reconcile incomitance produced by the esotropia, rapidly developed a residual esotropia. CONCLUSIONS: Ophthalmologists should recognize that concurrent esotropia may occur in patients with horizontal pontine gaze palsy. Single binocular vision in the primary position, an expanded binocular visual field, and alleviation of a compensatory head position are achievable with strabismus surgery.     

Presumed Metastasis of Breast Cancer to the Abducens Nucleus Presenting as Gaze Palsy

A 51-year-old woman with breast cancer presented with progressive diplopia. Neuro-ophthalmologic examination revealed right gaze palsy and peripheral facial nerve palsy. Brain magnetic resonance imaging (MRI) was normal. However, two months later a repeat brain MRI revealed an enhancing round nodular mass at the right facial colliculus of the lower pons, at the location of the abducens nucleus. Localized metastasis to the abducens nucleus can cause gaze palsy in a patient with breast cancer.     

Gaze palsy associated with viral syndrome

Four cases of gaze paresis occurred in association with viral disease. In three cases the viral prodrome preceded the oculomotor disturbance. In one, gaze palsy was followed several days later by a viral syndrome. One had isolated bilateral horizontal ophthalmoplegia; another patient had motility disturbance with blepharoptosis and peripheral areflexia. Two patients had clinical evidence of brain-stem involvement: unilateral gaze paresis and gaze-evoked nystagmus in one and Parinaud's syndrome in the other. No accompanying long-tract signs or changes in mental status were present in any case. Recovery was complete in all four patients.     

双上斜肌麻痹的临床特征与治疗

双眼上斜肌同时麻痹称为双上斜肌麻痹,近年来受到国内外眼科学者的重视。本文报告13例,均是我科近年来手术治疗并在术后观察3个月以上者。13例病人都无明显外伤史,发病在出生时及出生后早期,为先天性病变。手术次数1～2次。临床特征:不对称病变以单眼上斜视,代偿头位为主要表现,其中有一部分病人为潜在型双侧病变。对称型以两侧同向注视时的反向上斜视为主要表现,双侧Bielschowsky征为阳性。不少病人同时有V型斜视。需要手术治疗的病人一般应做双眼手术。可根据病情决定是同时做双侧手术,还是分次做,以及术式、术量。术后大部分病人能获得满意的头位及眼位。     

Lateral rectus muscle paralysis associated with closed-head trauma.

We examined 21 patients with closed-head trauma and resulting paralysis of the lateral rectus muscle. Clinical findings included laterally directed gaze palsy, some unconsciousness, and pseudo-duane's phenomenon suggesting a supranuclear lesion at the level of the upper pontine tegmentum, and pontine paramedial reticular formation. The surgical procedure of choice was a "midline operation," that is, the appropriate number of millimeters of recession and resection to achieve 0 to 5 degrees of exotropia in the primary position of gaze. Frequently, the medial rectus muscle must be recessed 10 nm or more and the lateral rectus muscle resected 10 nm or more to achieve this result. None of the 21 patients had diplopia after the midline procedure.     

Patterns of III nerve synkinesis

PURPOSE Various patterns of synkinesis have been observed following both congenital and acquired III nerve palsies. Aberrant regeneration is the most widely accepted mechanism which explains this synkinesis. Two models of aberrant regeneration have been proposed, namely central collateral axon sprouting, and misdirection of regenerating neurones at the site of nerve injury. We have studied the clinical patterns of III nerve recovery and synkinesis following III nerve palsies and relate the findings to the proposed models of synkinesis. MATERIALS AND METHODS Twelve patients with acquired and two with early onset III nerve synkinesis were reviewed. The type, rate and pattern of recovery of III nerve palsy was established from case notes. Observations and measurements of ocular motility, pupil movements, eyelid position and intraocular pressure changes were performed on each patient. Signs of contralateral synkinetic movements were also sought. RESULTS Upper lid retraction on attempted down gaze and in adduction was the most frequent synkinetic movement recorded. Observation of pupil size revealed evidence of pupillary-extraocular muscle synkinesis in all gaze positions except abduction. Similarly, intraocular pressure changes and signs of globe retraction showed widespread extraocular muscle co-contraction. Synkinetic levator innervation of extraocular muscles [i.e., LPS to MR, IR, SR] was also observed, a finding not previously reported in III nerve palsy but predicted by our model of misdirection of regenerating neurones at the site of nerve injury. There was no evidence of contralateral aberrant innervation. CONCLUSION Evidence of III nerve synkinesis in patients with III nerve palsy is widespread and the patterns of synkinesis observed were broadly similar for all patients irrespective of the aetiology. Random aberrant III nerve regeneration at the site of nerve injury best explains the patterns of synkinesis observed. Central collateral axon sprouting does not seem to play a major part in the synkinetic movements observed in acquired III nerve lesions.