Oculomotor Disturbances Research Articles

Central Oculomotor Disturbances and Nystagmus

Oculomotor disturbances and nystagmus are seen in many diseases of the nervous system, the vestibular apparatus, and the eyes, as well as in toxic and metabolic disorders. They often indicate a specific underlying cause. The key to diagnosis is systematic clinical examination of the patient's eye movements. This review deals mainly with central oculomotor disturbances, i.e., those involving smooth pursuit, saccades, gaze-holding, and central types of nystagmus. We searched the current literature for relevant publications on the diagnosis and treatment of oculomotor disturbances and nystagmus, and discuss them selectively in this review along with the German Neurological Society's guidelines on the topic. A detailed knowledge of the anatomy and physiology of eye movements usually enables the physician to localize the disturbance to a specific area in the brainstem or cerebellum. The examination of eye movements is an even more sensitive method than magnetic resonance imaging for the diagnosis of acute vestibular syndromes and for the differentiation of peripheral from central lesions. For example, isolated dysfunction of horizontal saccades is due to a pontine lesion, while isolated dysfunction of vertical saccades is due to a midbrain lesion. Generalized gaze-evoked nystagmus (GEN) has multiple causes; purely vertical GEN is due to a midbrain lesion, while purely horizontal GEN is due to a pontomedullary lesion. Internuclear ophthalmoplegia involves a constellation of findings, the most prominent of which is impaired adduction to the side of the causative lesion in the ipsilateral medial longitudinal fasciculus. The most common pathological types of central nystagmus are downbeat and upbeat nystagmus (DBN, UBN). DBN is generally due to cerebellar dysfunction, e.g., because of a neurodegenerative disease. This short review focuses on the clinical characteristics, pathophysiology and current treatment of oculomotor disorders and nystagmus.

Reversible vision loss secondary to malnutrition in a woman with severe anorexia nervosa, purging type, and alcohol abuse

To report a case of severe reversible vision loss in a woman with a 7-year history of anorexia nervosa, purging type, alcohol abuse and a severely restricted, vitamin-deficient diet. Psychiatric, ophthalmologic, and medical records were reviewed, and a literature search was performed on visual complications associated with anorexia nervosa and malnutrition. Ophthalmologic complications of malnutrition are rare but include both oculomotor and visual sensory disturbances. Thiamine deficiency can cause both types of disorders. Vitamin B12 and folate deficiencies are typically associated with optic neuropathy. Clinicians treating eating disorders should be aware of the potential for vitamin deficiencies and associated visual loss in patients with anorexia nervosa. This case highlights the importance of a detailed dietary history to guide vitamin rehabilitation and to minimize or reverse nutritional visual loss.

Topography of Cerebellar Deficits in Humans

The cerebellum is a key-piece for information processing and is involved in numerous motor and nonmotor activities, thanks to the anatomical characteristics of the circuitry, the enormous computational capabilities and the high connectivity to other brain areas. Despite its uniform cytoarchitecture, cerebellar circuitry is segregated into functional zones. This functional parcellation is driven by the connectivity and the anatomo-functional heterogeneity of the numerous extra-cerebellar structures linked to the cerebellum, principally brain cortices, precerebellar nuclei and spinal cord. Major insights into cerebellar functions have been gained with a detailed analysis of the cerebellar outputs, with the evidence that fundamental aspects of cerebrocerebellar operations are the closed-loop circuit and the predictions of future states. Cerebellar diseases result in disturbances of accuracy of movements and lack of coordination. The cerebellar syndrome includes combinations of oculomotor disturbances, dysarthria and other speech deficits, ataxia of limbs, ataxia of stance and gait, as well as often more subtle cognitive/behavioral impairments. Our understanding of the corresponding anatomo-functional maps for the human cerebellum is continuously improving. We summarize the topography of the clinical deficits observed in cerebellar patients and the growing evidence of a regional subdivision into motor, sensory, sensorimotor, cognitive and affective domains. The recently described topographic dichotomy motor versus nonmotor cerebellum based upon anatomical, functional and neuropsychological studies is also discussed.

Clinical Study of Paranasal Sinus Cyst with Orbital Manifestations

Paranasal sinus cyst may cause orbital manifestations such as visual disturbance, diplopia, and oculomotor disturbance. We treated 15 cases—11 men and 4 women—of paranasal cyst with orbital symptoms from January 2005 to December 2009. We assessed the chief orbital complaint, lesion site, case history, history of sinus surgery, first diagnostic visit, and prognosis. Four of the 15 with visual disturbance were analyzed further. Subjects were 31 to 71 years old (mean; 61.3 years). Of the 15, 11 (73.3%) had postoperative cysts and 4 (26.7%) had idiopathic cyst. Among paranasal sinus cysts, 4 were maxillary, 3 each anterior ethmoid and frontoethmoid, 2 sphenoethmoid, 1 each posterior ethmoid, frontal, and sphenoid sinuses. Of the 15, 12 had first seen ophthalmologists and 2 an internist. Time from orbital symptom onset to the first diagnostic visit averaged 5.9 (0 to 44) days. Chief complaints were visual disturbance in 4, diplopia in 7, and oculomotor disturbance in 4. All underwent endoscopic sinus surgery, after which orbital symptoms disappeared in most. Visual acuity returned to expected levels after surgery in the 4 with visual disturbance. Of orbital manifestations, visual disturbance was considered the most serious clinical feature because vision loss may become permanent without immediate surgical exploration. We consider specifically treating the involved sinuses soon after diagnosis to be of primary concern.

Steroid-responsive encephalopathy in autoimmune thyroiditis: Clinical spectrum and MRI observations in three cases

Hashimoto's encephalopathy (H.E.) is probably of autoimmune etiology, and manifests with seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms, myoclonus. It is presumed to be autoimmune in origin with high serum titers of antithyroid peroxidase antibodies (anti-TPA). Thyroid function might often be normal. The diagnosis is arrived at by excluding other toxic, metabolic and infectious causes of encephalopathies, supportive clinical profile, elevated thyroid antibodies and optimum steroid response. We present the characteristic phenotypic manifestations, magnetic resonance imaging and electroechography observations and response to immunomodulation with follow-up in three cases of H.E. All the three cases manifested with subacute to chronic progressive encephalopathy, cerebellar dysfunction, seizures, behavioral abnormalities and oculomotor disturbances and had evidence of hypothyroidism, elevated titers of anti-TPA and positive thyroid anti-microsomal antibodies. Atypical and uncommon presentations are known. This report emphasizes that a high index of suspicion is often required in cases with “investigation negative encephalopathy” for early diagnosis of H.E.

Specific Culomotor Disturbance in a Patient With Amyotrophic Lateral Sclerosis

A 67-year-old male patient was admitted to the hospital for pneumonia and respiratory insufficiency. Neurological examination showed lower and upper motor system involvement. One year and eight months after the initiation of artificial ventilation, he presented specific oculomotor disturbances. He predominantly showed slow eye movement: saccade speed was approximately 67 degree/second. Sometimes, he showed blink and neck rotation before he initiated saccades, or his saccades were undershoot or did not reach visual objects. He sometimes showed almost normal saccades, especially when he initiated saccades spontaneously (automatically). In this study, we have reported slow eye movement (saccades disturbances) with automatico-voluntary dissociation of an amyotrophic lateral sclerosis (ALS) patient. J Neurol Res. 2011;1(3):109-112 doi: https://doi.org/10.4021/jnr25w

Long-Term Outcome after Coil Embolization of Cavernous Sinus Arteriovenous Fistulas: Fig 1.

Cranial nerve palsies are regularly observed in patients with arteriovenous fistulas of the cavernous sinus. The purpose of our study was to determine the long-term clinical outcome-with a special focus on extra-ocular muscular dysfunctions-in patients who had undergone endovascular treatment of a cavernous sinus fistula with detachable coils. Sixteen patients were recalled for an ophthalmoneurologic control examination (mean interval of 4.4 years). The mRS and the EQ-5D questionnaire were used for the description of general outcome. Age, duration of symptoms, character of the fistula (direct, dural), and coil volume were tested to assess their relevance for persistent symptoms. All patients displayed complete regression of chemosis, exophthalmus, and pulsating tinnitus with no evidence of recurrences. Oculomotor disturbances persisted in 9 of 13 patients and caused permanent diplopia in 7 patients. In 15 patients a mRS score of 1 or 2 was achieved; however, 7 patients reported some limitations in life quality (EQ-5D). A significant correlation was found between coil volume and persistent diplopia (P = .032) and persistent cranial nerve VI paresis (P = .037). Coil embolization of the cavernous sinus led to durable closure of AVF and reliable regression of acute symptoms. However, long-term follow-up showed a 44% rate of persistent cranial nerve deficits with disturbances of oculomotor and visual functions. This may be explained by the underlying fistula size itself and/or the space-occupying effect of the coils. As neuro-ophthalmologic outcome is crucial for control of therapeutic success, patients should be routinely examined by ophthalmologists.

Effect of transsphenoidal surgery on decreased visual acuity caused by pituitary apoplexy

We treated 12 patients with pituitary apoplexy out of 103 patients with pituitary tumors from August 1994 to March 2008 in the Nishi-Kobe Medical Center. The male to female ratio was 1:2 and the average age was 43 years old, ranging from 19 to 73. The symptoms on presentation were a decrease of visual acuity in nine, headache in seven, endocrinological disturbance in six, visual field defect in seven, a febrile state in six, vomiting in four, oculomotor disturbance in two, abducens palsy in one, and transient altered consciousness in one. All patients underwent transsphenoidal surgery and, in four of these, surgery was conducted within 7 days after onset. All nine patients with a decrease in the visual acuity recovered (100%) and, in addition, complete or near-complete vision recovery was noted in six out of eight patients (75%), excluding one patient whom we were unable to examine accurately. Emergent surgery was performed for only two patients with an acute deterioration of the visual acuity, with one finally developing complete blindness. Based on this study, we conclude that decompressive surgery is very useful for decreased visual acuity caused by pituitary apoplexy, but it is not necessary to perform emergent surgery for pituitary apoplexy in the absence of severe visual deterioration.

Physiopathology and recording techniques of the ocular movements

A number of functional systems are involved in the control of eye movements. The vestibulo-ocular and optokinetic reflexes are automatic responses that compensate for the movements of the head and those of the visual environment in order to stabilize the retinal image on a given fixation point. The saccadic movements are quick displacements of fixation from one to another point in the visual field. The smooth pursuit movements consist in the gaze following a moving target. Finally, there are some involuntary movements of very small amplitude during fixation maintenance. Each functional modality of movement depends on specific neuronal circuits that work in a coordinated manner for encoding the contraction of the oculomotor muscles to reach an adequate position at every moment. These neuronal systems can be altered by many neurological processes of different kinds and localizations, causing a broad variety of oculomotor disturbances. The most salient aspects of the physiopathology and the recording systems of eye movements are reviewed.

Sensorimotor Function and Dizziness in Neck Pain: Implications for Assessment and Management

The term sensorimotor describes all the afferent, efferent, and central integration and processing components involved in maintaining stability in the postural control system through intrinsic motor-control properties. The scope of this paper is to highlight the sensorimotor deficits that can arise from altered cervical afferent input. From a clinical orthopaedic perspective, the peripheral mechanoreceptors are the most important in functional joint stability; but in the cervical region they are also important for postural stability, as well as head and eye movement control. Consequently, conventional musculoskeletal intervention approaches may be sufficient only for patients with neck pain and minimal sensorimotor proprioceptive disturbances. Clinical experience and research indicates that significant sensorimotor cervical proprioceptive disturbances might be an important factor in the maintenance, recurrence, or progression of various symptoms in some patients with neck pain. In these cases, more specific and novel treatment methods are needed which progressively address neck position and movement sense, as well as cervicogenic oculomotor disturbances, postural stability, and cervicogenic dizziness. In this commentary we review the most relevant theoretical and practical knowledge on this matter and implications for clinical assessment and management, and we propose future directions for research. Level 5.

From amaurosis fugax to asymptomatic bithalamic infarct

Bilateral paramedian thalamic infarctions are usually associated with impaired consciousness, oculomotor disturbances and neuropsychological changes. A 44-year-old healthy woman presented with amaurosis fugax of the right eye immediately after a Valsalva maneuver. Neurological examination, in particular visual acuity, vigilance and ocular movements, was normal. Blood pressure, ECG and angio-CT of the neck and head vessels were normal, but MRI/DWI and T2 sequences showed recent bi-thalamic ischemic lesions in the paramedian territories. Doppler sonography and transesophageal echocardiography showed a large right-to-left shunt due to an atrial communication, with septum aneurysm. Twenty-four-hours cardiac monitoring was normal but prior to an eventual closure of the cardiac defect she underwent an ambulatory 7-day ECG monitoring which revealed several paroxystic short lasting passages into atrial fibrillation, unnoticed by the patient. The interest in this case is threefold: (1) bilateral paramedian thalamic infarction which usually presents with a devastating clinical picture may occur clinically silent; (2) monocular amaurosis fugax which is usually associated with ipsilateral carotid disease may be the consequence of cardiac embolism, and (3) atrial fibrillation is never completely ruled out, here it was caught only in a 7-days ambulatory R-test, and consequently prevented closure of a possibly asymptomatic patent foramen ovale.

Fisiopatología y técnicas de registro de los movimientos oculares

A number of functional systems are involved in the control of eye movements. The vestibulo-ocular and optokinetic reflexes are automatic responses that compensate for the movements of the head and those of the visual environment in order to stabilize the retinal image on a given fixation point. The saccadic movements are quick displacements of fixation from one to another point in the visual field. The smooth pursuit movements consist in the gaze following a moving target. Finally, there are some involuntary movements of very small amplitude during fixation maintenance. Each functional modality of movement depends on specific neuronal circuits that work in a coordinated manner for encoding the contraction of the oculomotor muscles to reach an adequate position at every moment. These neuronal systems can be altered by many neurological processes of different kinds and localizations, causing a broad variety of oculomotor disturbances. The most salient aspects of the physiopathology and the recording systems of eye movements are reviewed.

Use of Porous Polyethylene With Embedded Titanium in Orbital Reconstruction: A Review of 106 Patients

To evaluate our initial experience with a porous polyethylene implant with embedded titanium in orbital reconstruction. A retrospective review of the charts was performed for patients receiving at least 1 orbital porous polyethylene implant with embedded titanium from October 2004 through April 2006. Patient demographics, implant type and size, method of fixation, postoperative complications, and length of follow-up were recorded. One-hundred six patients received at least one embedded titanium implant (80 men, 26 women). Age ranged from 3 years to 85 years (mean, 31 years). Indications included orbital fracture repair (102 patients), correction of enophthalmos (3 patients), and reconstruction after tumor resection (1 patient). The average floor implant used was 22.3 mm wide by 27.9 mm long. The average medial wall implant was 13.6 mm tall by 22 mm long. Implants were fixated with 1 or 2 microscrews in 75 patients (70.8%) and placed without fixation in 31 patients (29.2%). Four postoperative complications were noted: a retrobulbar hemorrhage on postoperative day 3 that resolved without sequelae, a vertical overcorrection requiring removal of the implant, a transient oculomotor disturbance, and a screw placed in proximity to the infraorbital nerve canal resulting in hypesthesia and chronic pain that required removal of the screw. In the 3 complications potentially related to the implant, the embedded titanium allowed postoperative implant visualization and guided subsequent management. No implant extrusions or postoperative infections were noted. Porous polyethylene implants with embedded titanium provide a new alternative to alloplastic implant materials for orbital reconstruction with a profile that combines several advantages of porous polyethylene and titanium implants.

CAVERNOUS HEMANGIOMA OF THE CAVERNOUS SINUS, SKIN, AND RETINA

There are several reports concerning cavernous hemangiomas of the skin and central nervous system. Additional retinal involvement has also been reported. The authors report a 69-year-old woman with a giant extra-axial cavernous hemangioma of the right cavernous sinus involving the supra- and parasellar region, retina, and skin. Shrinkage of its cutaneous part lead to subsequent increase of the volume of the intracranial part. Owing to compression of the optic and the oculomotor nerves, oculomotor disturbances, ptosis, and visual impairment to 0.2 occurred. Via a pterional approach microsurgical removal of the tumor except for a remnant of the intracavernous part was performed. Hemodynamic connection between cutaneous, retinal, and intracranial hemangiomas should be considered.

Congenital ocular motor apraxia: Clinical and neuroradiological findings, and long-term intellectual prognosis

The severity of intellectual sequelae and prognosis varies in patients with congenital ocular motor apraxia (COMA). Here, we explored this phenomenon with regard to the accompanying oculomotor signs and gross motor development, as well as the subtentorial structure defects. Ten patients diagnosed with COMA (M:F = 4:6, 4–37 years old) were reviewed. Four individuals who gained the ability to walk at 2 years or earlier showed normal intellect and social skills. Those who walked later often showed accompanying intellectual (5/6) and speech (6/6) disabilities. In this latter group, atypical oculomotor signs for COMA (presence of nystagmus, mild limitation of vertical gaze, slower head thrust, and marked improvement of lateral saccade during early childhood) were often noted (4/6). Minor anomalies of fingers and toes were also common in this group. Neuroimaging was conduced in nine patients (pneumoencepharography 1; computed tomography: 8, magnetic resonance imaging: 2). Dilatation of the fourth ventricle, mainly at the level of the midbrain or upper pons ( n = 7), and hypoplastic cerebellar vermis ( n = 6) were commonly observed in both the early- and late-walking groups. ‘Molar tooth’ signs ( n = 3) were exclusively noted in the late-walking group, and often accompanied by atypical oculomotor signs (3/3) and intellectual disabilities (2/3). Vermian hypoplasia and dilatation of the fourth ventricle at the upper brainstem level in COMA patients, with or without intellectual disabilities, suggested that the cardinal lesion for OMA may exist in these areas. The presence of a subset of ‘atypical’ COMA patients may suggest that COMA with subtle infratentorial abnormality represents a heterogeneous disease category, showing similar oculomotor disturbance. This review indicated that clinical and neuroradiological inspection might be valuable for prediction of long-term intellectual prognosis in COMA patients.

SURGERY FOR TEMPORAL MEDIOBASAL TUMORS

Abstract OBJECTIVE To describe the clinical characteristics, diagnosis, various approaches, and outcomes in a retrospective review of a large series of temporomediobasal (TMB) tumors. METHODS Charts from 235 patients with TMB tumors were identified from the glioma and epilepsy surgery database and from the electronic operations log. Preoperative magnetic resonance imaging scans were available for all patients and postoperative follow-up was available for 155 of these patients (mean follow-up period, 59 mo; range, 2–172 mo). Preoperative symptoms, approaches, technical problems, and surgical complications are described. RESULTS Two hundred and thirty-five patients with intra-axial TMB tumors (mean age, 35 yr) were collected during an 11-year period. The largest tumor groups were astrocytomas (38.0%), gangliogliomas (29.8%), dysembryoplastic neuroepithelial tumor (11.1%), and glioblastomas (11.1%). The most frequent tumor location was the mesial Type A tumor (45.1%), with this type also showing the highest proportion of benign (World Health Organization Grades I and II) histological features (91.3%). Of all tumors, 76.2% were benign. Larger tumor size was associated with higher frequency of malignant histopathological findings. The leading symptom was epilepsy in 91% of patients, followed by drug-resistant epilepsy in 71.5%. Significant preoperative neurological deficits, such as hemiparesis or aphasia, were seen in 3.8% of the patients; another 12% had visual field deficits. Thirty-eight patients with low-grade tumors had undergone surgery previously. Several surgical approaches were chosen: transsylvian in 28%, anterior two-thirds temporal lobe resection in 23%, temporal pole resection in 15.3%, subtemporal in 19%, and transcortical in 6%. The most frequent neurological complications were transient: dysphasia (4.2%), hemiparesis (5%), and oculomotor disturbance (2.5%). Permanent nonvisual neurological complications occurred in fewer than 2% of the patients and significant new hemianopic defects were found in another 5.4% of the patients. The most severe complication was one intraoperative internal carotid artery lesion. One patient died. CONCLUSION Small tumor size, magnetic resonance imaging, and microsurgery have made resection of mostly benign TMB tumors possible in a large number of patients. This series supports the conclusion that these tumors can be operated on with a relative degree of safety for the patient, provided that the anatomy of the mesial temporal lobe and the variety of approaches are well known to the surgeon. However, because of the complex anatomic structures in the vicinity, transient neurological deterioration is not infrequent and certain neurological disturbances (e.g., quadrantanopia) even seem to be unavoidable, whereas permanent significant deficits are rare.

Stiff-person syndrome with oculomotor and cerebellar disturbances

Stiff-person syndrome is a rare sporadic disorder of the central nervous system of unknown etiology. In the paper, a case of a woman with axial muscle rigidity, cerebellar and eye movement disturbances is presented. High level of autoantibodies to glutamic acid decarboxylase is detected, and electromyographic examination showed continuous motor unit activity of the axial muscles at rest. On electrophoretic investigation of muscle tissue proteins, it was shown for the first time destruction of titin and nebulin, the proteins determining elastic properties of the muscle.

Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is an acute, demyelinating polyneuropathy involving the spinal roots, peripheral nerves, and often the cranial nerves. Although its exact mechanism remains unclear, an autoimmune etiopathology is theorized. It is characterized by rapidly progressing, symmetrical muscular weakness starting in the legs and ascending to the trunk and arms. Additionally, deep tendon reflexes are lost. Approximately half of the patients with GBS have cranial nerve palsies, with unilateral or bilateral facial nerve (CN VII) palsy being the most common. Paralysis of the muscles of the tongue, lips, palate, larynx, and pharynx from lesions involving cranial nerves IX, X, and XI and weakness of the muscles of mastication (CN V) are the next most common cranial nerve abnormalities. Ocular muscle palsy is not common, only occurring in approximately 10% of patients. The Miller Fisher variant of GBS is a distinct syndrome in which the only neurologic deficits are oculomotor palsies, areflexia, and ataxia. We present the rare case of a 45-year-old woman with GBS whose ocular muscles were affected. The oculomotor disturbance and limb weakness occurred within a few days of one another. Basic anatomic considerations are reviewed along with the pathophysiology and clinical features of GBS.

Peduncular hallucinosis

Peduncular hallucinosis (PH) is a syndrome of hallucinations and brainstem symptoms which has only been described in single case reports. A detailed analysis of five patients was undertaken to investigate the clincial characteristics, hallucinations and behavioural abnormalities of PH in greater detail. Frequent clinical symptoms were oculomotor disturbances, impaired arousal, dysarthria and ataxia. In the chronic stage, sleep-wake cycle disturbances were common. Hallucinations were naturalistic, complex, scenic, mostly visual, but also combined visual-acoustic or visual-tactile and recurred stereotypically over months. Patients experienced their hallucinations as genuine and were unable to discriminate their percepts from reality. Neuropsychological testing disclosed severe impairments of episodic memory, occasionally coupled with confabulatory behaviour. By contrast, memory for hallucinations remained intact. Deficits of attentional and executive functions were found in a subgroup of patients. Associated abnormal behaviours were common, comprising confusion, delusional misidentification for persons and places, and loss of disease awareness. PH appeared after focal lesions in various regions, such as the midbrain, thalamus and pons. These findings document that subcortical, brainstem-related hallucinations are vivid, recurring percepts that have a strong naturalistic character and are often associated with cognitive and behavioural abnormalities. It seems likely that brainstem hallucinosis is caused by damage to ascending reticular systems and thalamocortical circuits. Available observations suggest that PH compromises cognitive functions which enable us to differentiate between illusionary percepts and reality, a reality monitoring system.

Clinical features of double infection with tick-borne encephalitis and Lyme borreliosis transmitted by tick bite

Background: In Latvia and other endemic regions, a single tick bite has the potential to transmit both tick-borne encephalitis (TBE) and Lyme borreliosis. Objective: To analyse both the clinical features...