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In the United States, testing for "horizontal gaze nystagmus" is one of a battery of field sobriety tests used by police officers to determine whether a suspect is driving under the influence of alcohol. The test involves observation of the suspect's pupil as it follows a moving object, noting
1. lack of smooth pursuit,
2. distinct and sustained nystagmus at maximum deviation, and
3. the onset of nystagmus prior to 45 degrees.
The horizontal gaze nystagmus test has been highly criticized and major errors in the testing methodology and analysis found. However, the validity of the horizontal gaze nystagmus test for use as a field sobriety test for persons with a blood alcohol level between 0.04–0.08 is supported by peer reviewed studies and has been found to be a more accurate indication of blood alcohol content than other standard field sobriety tests.
Nystagmus is very noticeable but rarely recognized. Nystagmus can be clinically investigated by using a number of non-invasive standard tests. The simplest one is the caloric reflex test, in which one ear canal is irrigated with warm or cold water or air. The temperature gradient provokes the stimulation of the horizontal semicircular canal and the consequent nystagmus.
Nystagmus is often very commonly present with Chiari malformation.
The resulting movement of the eyes may be recorded and quantified by special devices called electronystagmograph (ENG), a form of electrooculography (an electrical method of measuring eye movements using external electrodes), or even less invasive devices called videonystagmograph (VNG), a form of video-oculography (VOG) (a video-based method of measuring eye movements using external small cameras built into head masks) by an audiologist. Special swinging chairs with electrical controls can be used to induce rotatory nystagmus.
Over the past forty years, objective eye-movement-recording techniques have been applied to the study of nystagmus, and the results have led to a greater accuracy and understanding of the condition.
Orthoptists may also use an optokinetic drum, or electrooculography to assess a patient's eye movements.
Nystagmus can be caused by subsequent foveation of moving objects, pathology, sustained rotation or substance use. Nystagmus is not to be confused with other superficially similar-appearing disorders of eye movements (saccadic oscillations) such as opsoclonus or ocular flutter that are composed purely of fast-phase (saccadic) eye movements, while nystagmus is characterised by the combination of a smooth pursuit, which usually acts to take the eye off the point of regard, interspersed with the saccadic movement that serves to bring the eye back on target. Without the use of objective recording techniques, it may be very difficult to distinguish between these conditions.
In medicine, the presence of nystagmus can be benign, or it can indicate an underlying visual or neurological problem.
According to a Cochrane review of 2012, controversies remain regarding type of surgery, non-surgical intervention and age of intervention.
The aims of treatment are as follows:
The elimination of any amblyopia
A cosmetically acceptable ocular alignment
long term stability of eye position
binocular cooperation.
Controversy has arisen regarding the selection and planning of surgical procedures, the timing of surgery and about what constitutes a favourable outcome.
1. Selection and planning
Some ophthalmologists, notably Ing and Helveston, favour a prescribed approach often involving multiple surgical episodes whereas others prefer to aim for full alignment of the eyes in one procedure and let the number of muscles operated upon during this procedure be determined by the size of the squint.
2. Timing and outcome
This debate relates to the technical anatomical difficulties of operating on the very young versus the possibility of an increased potential for binocularity associated with early surgery. Infants are often operated upon at the age of six to nine months of age and in some cases even earlier at three or four months of age. Some emphasize the importance of intervening early such as to keep the duration of the patient's abnormal visual experience to a minimum. Advocates of early surgery believe that those who have their surgery before the age of one are more likely to be able to use both eyes together post-operatively.
A Dutch study (ELISSS) compared early with late surgery in a prospective, controlled, non-randomized, multicenter trial and reported that:
"Children operated early had better gross stereopsis at age six as compared to children operated late. They had been operated more frequently, however, and a substantial number of children in both [originally-recruited] groups had not been operated at all."
Other studies also report better results with early surgery, notably Birch and Stager and Murray et al. but do not comment on the number of operations undertaken. A recent study on 38 children concluded that surgery for infantile esotropia is most likely to result in measureable stereopsis if patient age at alignment is not more than 16 months.
Another study found that for children with infantile esotropia early surgery decreases the risk of dissociated vertical deviation developing after surgery.
Aside the strabismus itself, there are other aspects or conditions that appear to improve after surgery or botulinum toxin eye alignment. Study outcomes have indicated that after surgery the child catches up in development of fine-motor skills (such as grasping a toy and handling a bottle) and of large-muscle skills (such as sitting, standing, and walking) in case a developmental delay was present before. Evidence also indicates that as of the age of six, strabismic children become less accepted by their peers, leaving them potentially exposed to social exclusion starting at this age unless their eye positioning is corrected by this time ("see also:" Psychosocial effects of strabismus).
A rostral lesion within the midbrain may affect the convergence center thus causing bilateral divergence of the eyes which is known as the WEBINO syndrome (Wall Eyed Bilateral INO) as each eye looks at the opposite "wall".
If the lesion affects the PPRF (or the abducens nucleus) and the MLF on the same side (the MLF having crossed from the opposite side), then the "one and a half syndrome" occurs which, simply put, involves paralysis of all conjugate horizontal eye movements other than abduction of the eye on the opposite side to the lesion.
The disorder is caused by injury or dysfunction in the medial longitudinal fasciculus (MLF), a heavily myelinated tract that allows conjugate eye movement by connecting the paramedian pontine reticular formation (PPRF)-abducens nucleus complex of the contralateral side to the oculomotor nucleus of the ipsilateral side.
In young patients with bilateral INO, multiple sclerosis is often the cause. In older patients with one-sided lesions a stroke is a distinct possibility. Other causes are possible.
If an optokinetic drum is available, rotate the drum in front of the patient. Ask the patient to look at the drum as you rotate it slowly. If an optokinetic drum is not available, move a strip of paper with alternating 2-inch black and white strips across the patient's visual field. Pass it in front of the patient's eye at reading distance while instructing the patient to look at it as it rapidly moves by. With normal vision, a nystagmus develops in both adults and infants. The nystagmus consists of initial slow phases in the direction of the stimulus (smooth pursuits), followed by fast, corrective phases (saccade). Presence of nystagmus indicates an intact visual pathway.
Another effective method is to hold a mirror in front of the patient and slowly rotate the mirror to either side of the patient. The patient with an intact visual pathway will maintain eye contact with herself or himself. This compelling optokinetic stimulus forces reflex slow eye movements.
OKN can be used as a crude assessment of the visual system, particularly in infants. When factitious blindness or malingering is suspected, check for optokinetic nystagmus to determine whether there is an intact visual pathway.
The optokinetic response is a combination of a slow-phase and fast-phase eye movements. It is seen when an individual follows a moving object with their eyes, which then moves out of the field of vision at which point their eye moves back to the position it was in when it first saw the object. The reflex develops at about 6 months of age.
Optokinetic nystagmus (OKN) is nystagmus that occurs in response to a rotation movement. It is present normally. The optokinetic response allows the eye to follow objects in motion when the head remains stationary (e.g., observing individual telephone poles on the side of the road as one travels by them in a car, or observing stationary objects while walking past them).
The eye findings of Parinaud's Syndrome generally improve slowly over months, especially with resolution of the causative factor; continued resolution after the first 3–6 months of onset is uncommon. However, rapid resolution after normalization of intracranial pressure following placement of a ventriculoperitoneal shunt has been reported.
Treatment is primarily directed towards etiology of the dorsal midbrain syndrome. A thorough workup, including neuroimaging is essential to rule out anatomic lesions or other causes of this syndrome. Visually significant upgaze palsy can be relieved with bilateral inferior rectus recessions. Retraction nystagmus and convergence movement are usually improved with this procedure as well.
Pendular nystagmus is a sinusoidal oscillation, which refers to the waveform of involuntary eye movements that may occur in any direction. It is characterized by the multidimensional slow eye movements of the eyes (1 Hz frequency) with an equal velocity in each direction that resembles the trajectory of a pendulum. These pattern of these movements may differ between the two eyes. Depending upon the pattern of movements, pendular nystagmus has been divided into different subtypes such as congenital nystagmus, acquired pendular nystagmus, and amaurotic nystagmus.
There have been cases of improvement in extra-ocular movement with botulinum toxin injection.
Typically a coloboma appears oval or comet shaped with round end towards the centre. There may be a few vessels (retinal or choroidal) at the edges. The surface may have irregular depression.
Colobomas of the iris may be treated in a number of ways. A simple cosmetic solution is a specialized cosmetic contact lens with an artificial pupil aperture. Surgical repair of the iris defect is also possible. Surgeons can close the defect by stitching in some cases. More recently artificial iris prosthetic devices such as the Human Optics artificial iris have been used successfully by specialist surgeons. This device cannot be used if the natural lens is in place and is not suitable for children. Suture repair is a better option where the lens is still present.
Vision can be improved with glasses, contact lenses or even laser eye surgery but may be limited if the retina is affected or there is amblyopia.
Amaurotic nystagmus is defined as the nystagmus associated with blindness or the central vision defects. It is characterized by the pendular or jerky movements of the eyes in the patients who have visual impairement for a long period of time.
The one and a half syndrome is a rare weakness in eye movement affecting both eyes, in which one cannot move laterally at all, and the other can move in only one lateral direction (inward or outward). More formally, it is characterized by ""a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other"". The most common manifestation of this unusual syndrome is limitation of horizontal eye movement to abduction (moving away from the midline) of one eye (e.g. right eye in the diagram on the right) with no horizontal movement of the other eye (e.g. left eye in the diagram on the right). Nystagmus is also present when the eye on the opposite side of the lesion is abducted. Convergence is classically spared as cranial nerve III (oculomotor nerve) and its nucleus is spared bilaterally.
There is generally no treatment to cure achromatopsia. However, dark red or plum colored filters are very helpful in controlling light sensitivity.
Since 2003, there is a cybernetic device called eyeborg that allows people to perceive color through sound waves. Achromatopsic artist Neil Harbisson was the first to use such a device in early 2004, the eyeborg allowed him to start painting in color by memorizing the sound of each color.
Moreover, there is some research on gene therapy for animals with achromatopsia, with positive results on mice and young dogs, but less effectiveness on older dogs. However, no experiments have been made on humans. There are many challenges to conducting gene therapy on humans. See Gene therapy for color blindness for more details about it.
Bruns nystagmus is an unusual type of bilateral nystagmus most commonly occurring in patients with cerebellopontine angle tumours. It is caused by the combination of slow, large amplitude nystagmus (gaze paretic nystagmus) when looking towards the side of the lesion, and rapid, small amplitude nystagmus (vestibular nystagmus) when looking away from the side of the lesion. It occurs in 11% of patients with vestibular schwannoma, and occurs mainly in patients with larger tumours (67% of patients with tumours over 3.5 cm diameter). Bruns nystagmus is also associated with an increased incidence of balance disturbance in patients with vestibular schwannoma. It may be caused by the compression of both flocculi, the vestibular part of the cerebellum, and improvement in both the nystagmus and balance problems occur commonly after removal of the tumour.
Bruns nystagmus is named for Ludwig Bruns (1858 – 1915).
The condition is diagnosed by the patient's history, and by performing the Dix–Hallpike test or the roll test, or both.
The Dix–Hallpike test is a common test performed by examiners to determine whether the posterior semicircular canal is involved. It involves a reorientation of the head to align the posterior semicircular canal (at its entrance to the ampulla) with the direction of gravity. This test will reproduce vertigo and nystagmus characteristic of posterior canal BPPV.
When performing the Dix–Hallpike test, patients are lowered quickly to a supine position, with the neck extended by the clinician performing the maneuver. For some patients, this maneuver may not be indicated, and a modification may be needed that also targets the posterior semicircular canal. Such patients include those who are too anxious about eliciting the uncomfortable symptoms of vertigo, and those who may not have the range of motion necessary to comfortably be in a supine position. The modification involves the patient moving from a seated position to side-lying "without" their head extending off the examination table, such as with Dix–Hallpike. The head is rotated 45 degrees away from the side being tested, and the eyes are examined for nystagmus. A positive test is indicated by patient report of a reproduction of vertigo and clinician observation of nystagmus. Both the Dix–Hallpike and the side-lying testing position have yielded similar results, and as such the side-lying position can be used if the Dix–Hallpike cannot be performed easily.
The roll test can determine whether the horizontal semicircular canal is involved. The roll test requires the patient to be in a supine position with their head in 30° of cervical flexion. Then the examiner quickly rotates the head 90° to the left side, and checks for vertigo and nystagmus. This is followed by gently bringing the head back to the starting position. The examiner then quickly rotates the head 90° to the right side, and checks again for vertigo and nystagmus. In this roll test, the patient may experience vertigo and nystagmus on both sides, but rotating towards the affected side will trigger a more intense vertigo. Similarly, when the head is rotated towards the affected side, the nystagmus will beat towards the ground and be more intense.
As mentioned above, both the Dix–Hallpike and roll test provoke the signs and symptoms in subjects suffering from archetypal BPPV. The signs and symptoms patients with BPPV experience are typically a short-lived vertigo, and observed nystagmus. In some patients, though rarely, the vertigo can persist for years. Assessment of BPPV is best done by a medical health professional skilled in management of dizziness disorders, commonly a physiotherapist, audiologist or other physician.
The nystagmus associated with BPPV has several important characteristics which differentiate it from other types of nystagmus.
- Latency of onset: there is a 5–10 second delay prior to onset of nystagmus.
- Nystagmus lasts for 5–120 seconds.
- Positional: the nystagmus occurs only in certain positions.
- Repeated stimulation, including via Dix–Hallpike maneuvers, cause the nystagmus to fatigue or disappear temporarily.
- Rotatory/Torsional component is present, or (in the case of lateral canal involvement) the nystagmus beats in either a geotropic (towards the ground) or ageotropic (away from the ground) fashion.
- Visual fixation suppresses nystagmus due to BPPV.
Although rare, CNS disorders can sometimes present as BPPV. A practitioner should be aware that if a patient whose symptoms are consistent with BPPV, but does not show improvement or resolution after undergoing different particle repositioning maneuvers — detailed in the Treatment section below — need to have a detailed neurological assessment and imaging performed to help identify the pathological condition.
Parinaud's Syndrome results from injury, either direct or compressive, to the dorsal midbrain. Specifically, compression or ischemic damage of the mesencephalic tectum, including the superior colliculus adjacent oculomotor (origin of cranial nerve III) and Edinger-Westphal nuclei, causing dysfunction to the motor function of the eye.
Classically, it has been associated with three major groups:
1. Young patients with brain tumors in the pineal gland or midbrain: pinealoma (intracranial germinomas) are the most common lesion producing this syndrome.
2. Women in their 20s-30s with multiple sclerosis
3. Older patients following stroke of the upper brainstem
However, any other compression, ischemia or damage to this region can produce these phenomena: obstructive hydrocephalus, midbrain hemorrhage, cerebral arteriovenous malformation, trauma and brainstem toxoplasmosis infection. Neoplasms and giant aneurysms of the posterior fossa have also been associated with the midbrain syndrome.
Vertical supranuclear ophthalmoplegia has also been associated with metabolic disorders, such as Niemann-Pick disease, Wilson's disease, kernicterus, and barbiturate overdose.
Persistent hyperplastic primary vitreous (PHPV), also known as Persistent Fetal Vasculature (PFV), is a rare congenital developmental anomaly of the eye that results
following failure of the embryological, primary vitreous and hyaloid vasculature to regress. It can be present in three forms: purely anterior (persistent tunica vasculosa lentis and persistent posterior fetal fibrovascular sheath of the lens), purely posterior (falciform retinal septum and ablatio falcicormis congenita) and a combination of both. Most examples of PHPV are unilateral and non-hereditary. When bilateral, PHPV may follow an autosomal recessive or autosomal dominant inheritance pattern.
Vertigo, a distinct process sometimes confused with the broader term, dizziness, accounts for about six million clinic visits in the United States every year; between 17 and 42% of these patients are eventually diagnosed with BPPV.
Other causes of vertigo include:
- Motion sickness/motion intolerance: a disjunction between visual stimulation, vestibular stimulation, and/or proprioception
- Visual exposure to nearby moving objects (examples of optokinetic stimuli include passing cars and falling snow)
- Other diseases: (labyrinthitis, Ménière's disease, and migraine, etc.)
Zonular cataract and nystagmus, also referred as Nystagmus with congenital zonular cataract is a rare congenital disease associated with Nystagmus and zonular cataract of the eye.
The primary vitreous used in formation of the eye during fetal development remains in the eye upon birth and is hazy and scarred. The symptoms are leukocoria, strabismus, nystagmus and blurred vision, blindness.
It has been suggested that the disease follows a x-linked pattern of inheritance though studies done on this particular disease are few.
The most important factor in diagnosing a patient with vertiginous epilepsy is the subject’s detailed description of the episode. However, due to the associated symptoms of the syndrome a subject may have difficulty remembering the specifics of the experience. This makes it difficult for a physician to confirm the diagnosis with absolute certainty. A questionnaire may be used to help patients, especially children, describe their symptoms. Clinicians may also consult family members for assistance in diagnosis, relying on their observations to help understand the episodes. In addition to the description of the event, neurological, physical and hematologic examinations are completed to assist in diagnosis. For proper diagnosis, an otological exam (examination of the ear) should also be completed to rule out disorders of the inner ear, which could also be responsible for manifestations of vertigo. This may include an audiological assessment and vestibular function test. During diagnosis, history-taking is essential in determining possible causes of vertiginous epilepsy as well as tracking the progress of the disorder over time.
Other means used in diagnosis of vertiginous epilepsy include:
- Electroencephalography (EEG)
- Magnetic resonance imaging (MRI)
- Positron emission tomography (PET)
- Neuropsychological testing
The EEG measures electrical activity in the brain, allowing a physician to identify any unusual patterns. While EEGs are good for identifying abnormal brain activity is it not helpful in localizing where the seizure originates because they spread so quickly across the brain. MRIs are used to look for masses or lesions in the temporal lobe of the brain, indicating possible tumors or cancer as the cause of the seizures. When using a PET scan, a physician is looking to detect abnormal blood flow and glucose metabolism in the brain, which is visible between seizures, to indicate the region of origin.