Parinaud syndrome is vertical gaze palsy. Paralysis of the eye muscles is caused by lesions in the midbrain and the nerve nuclei located there, such as can be caused by tumors, strokes and inflammation. The treatment of the symptom complex depends on the triggering disease.
What is Parinaud Syndrome?
In Parinaud’s syndrome, vertical gaze paralysis is present in combination with other neurological symptoms due to damage to the cranial nerves. The symptom complex is also known as dorsal midbrain syndrome and was first described by Parinaud in the 19th century. With this phenomenon, convergence movements of the eyes are only possible to a limited extent. Command movements up can no longer be executed at all. See ezhoushan for What does L1CAM Syndrome Stand for.
Parinaud’s disease must be distinguished from Parinaud’s syndrome. It is an infectious disease caused by the bacterium Francisella tularensis. As part of this disease, the so-called oculologlandular syndrome Parinaud can occur. This means conjunctivitis with swollen lymph nodes under the jaw and in front of the ear. This phenomenon occurs not only in the context of Parinaud’s disease, but also in the context of tuberculosis, fungal infections or syphilis.
Causes
Damage to the cranial nerves is the cause of vertical gaze palsy in the form of Parinaud’s syndrome. More specifically, the cranial nerve nuclei are damaged. In most cases, these are lesions in the area of the midbrain, i.e. the brainstem between the pons and the diencephalon. Most of the eye muscles are controlled from the midbrain. As a rule, the causative lesion of Parinaud’s syndrome is ventrocaudal to the quad.
A pineal tumor is most commonly responsible for lesions of this type. These tumors are growths of the pineal gland and usually occur in childhood. A stroke in the midbrain area can also cause lesions near the four ridges. In addition to these causes, inflammatory diseases of the brain are also possible, such as encephalitis or multiple sclerosis.
Symptoms, Ailments & Signs
Parinaud syndrome is primarily characterized by upward gaze paralysis. Within the framework of the doll’s head effect, reflex movements in the paralyzed direction are possible. The pupils of the patients are usually dilated and show no reflexes. Close-up is no longer possible or only possible to a limited extent.
The pupils are usually fixed to light, but other pupillary disorders can also occur. There are also convergence disorders of the bulbi. In addition, nystagmus can occur, i.e. an involuntary trembling of the eyes. Parallel eye movement does not necessarily have to be limited in Parinaud syndrome. It is difficult to give a uniform definition of the symptoms for Parinaud’s syndrome. The cause of the triggering midbrain lesion plays a role in the symptoms actually occurring. In the case of a tumor in the pineal gland, for example, liquor builds up and intracranial pressure increases.
Diagnosis & course of disease
The symptoms are assessed by a neurologist and assessed, among other things, by exams of the eye motor function. After this initial assessment, an imaging procedure is usually initiated. This can be either a CT or an MRI. Brain pressure measurements supplement the imaging. The underlying disease behind Parinaud’s syndrome must be elucidated upon diagnosis.
Inflammatory diseases of the central nervous system can be detected by analyzing the liquor. Tumors such as the pineal tumor appear relatively typically in the MRI and are therefore easy to differentiate from inflammatory diseases. In addition, tumor markers are found in the cerebrospinal fluid for some tumor types. The prognosis for Parinaud syndrome varies depending on what caused the midbrain lesions.
Complications
Those affected by Parinaud syndrome suffer from visual problems. The further course of this disease depends very much on its cause, so that a general prediction of the complications is also not possible in general. In many cases, however, treatment is no longer possible if the nerves have been irreversibly damaged.
The patients can no longer see properly in a certain direction. Focusing on nearby objects is also usually no longer possible due to Parinaud’s syndrome, which means that the person affected has significant limitations in their everyday life. In many cases, Parinaud’s syndrome also leads to significantly increased pressure in the brain. This often leads to severe headaches or unpleasant feelings of pressure in the head and ears.
The treatment of Parinaud syndrome depends on the underlying disease, although not every case leads to a positive course of the disease. Without treatment, it can also lead to inflammation of the meninges, which can be fatal for those affected. Parinaud’s syndrome may also reduce the patient’s life expectancy.
When should you go to the doctor?
Parinaud’s syndrome is a rare condition that occurs, among other things, in connection with Parkinson’s disease. People suffering from a nervous system disorder should call their doctor if they experience the typical up or down gaze paralysis. A reduced pupillary reflex and nystagmus are associated symptoms that need to be investigated. The same applies to a dilated pupil or unusual eye movements such as squinting or exophoria. People who suffer from multiple sclerosis, Parkinson’s disease, encephalitis or a neurological disorder are particularly at risk.
Parinaud’s syndrome also frequently occurs in connection with a brain tumor or a cerebral infarction. People who notice the symptoms mentioned and also suffer from one of these diseases should speak to the responsible doctor immediately. Depending on the causative disease, either the neurologist, the general practitioner or an internist is responsible. Oncologists and psychologists can also be involved in the treatment. Parinaud syndrome is treated depending on the cause and usually requires medication. Eye training and special glasses can support the therapy.
Treatment & Therapy
The treatment of Parinaud syndrome depends on the causative disease. For example, a pineal tumor must be surgically removed. If liquor builds up as part of this disease, the increased intracranial pressure must first be regulated. Before the operation, liquor is drained. The doctor achieves this goal, for example, as part of an endoscopic ventriculocisternostomy, in which the third cerebral ventricle is opened at the bottom.
After that, the doctor prefers to remove the tumor microsurgically, especially in the case of carcinomas, choriocarcinomas and teratomas. The surrounding tissue must be spared as much as possible during this procedure. In the case of a malignant tumor, care must be taken to ensure that tumor cells are not tolerated in other tissue parts during the operation. In the case of germinoma, there is no surgery, but primary radiation.
For other malignant tumors, the head and spine are irradiated after microsurgical removal of the tumor to avoid metastasis through the cerebrospinal fluid. In the case of inflammatory causes, the patient is usually given intravenous cortisone. If multiple sclerosis is suspected to be the cause, acute treatment with cortisone is usually followed by long-term treatment with immunosuppressive drugs.
In the acute phase of meningitis caused by bacteria, antibiotics are administered instead of cortisone. Depending on the accompanying symptoms, the treatment of Parinaud’s syndrome may vary.
Outlook & Forecast
The outlook and prognosis of Parinaud syndrome depends on the causative disorder. If the course is severe, damage to the eyes occurs on both sides and ultimately blindness. Severe courses are to be expected as a result of brain tumors, cerebral infarction or multiple sclerosis.
The prognosis is better if the Parinaud syndrome is based on increased intracranial pressure. If treated early, recovery is likely. Aqueduct stenosis and encephalitis also offer a good prognosis if treated quickly. Life expectancy can be limited by Parinaud syndrome. Many patients die prematurely from side effects of the causative disease.
Parinaud syndrome itself does not negatively affect life expectancy. However, the quality of life is limited by the limited vision and the side effects. The specialist doctor responsible provides the outlook and prognosis. Usually an internist or neurologist is responsible. Parinaud syndrome itself is evaluated in the context of the underlying disease and the prognosis must be adjusted accordingly as the disease progresses. Vertical gaze paralysis can lead to accidents and falls in everyday life. As a rule, those affected are dependent on help and are no longer able to work as before.
Prevention
Vaccines are available for some brain infections caused by infection. Strokes can be prevented by a healthy diet, sufficient exercise and not smoking and alcohol. Multiple sclerosis, on the other hand, cannot be prevented because the causes of this disease are still unclear. Midbrain tumors can also hardly be prevented.
Aftercare
In most cases, those affected by Parinaud’s syndrome have only limited and few direct follow-up measures available. Since it is a case of paralysis of the eyes, which can usually no longer be treated completely, those affected are primarily dependent on an early and quick diagnosis of the disease. This is the only way to prevent further complications or other complaints.
The sooner a doctor is contacted, the better the further course of the disease. Affected people are dependent on various interventions to remove the tumor that is responsible for this disease. Those affected should definitely take it easy and rest after such an operation.
Efforts should be avoided. Regular check-ups and examinations by a doctor are also very important in order to detect further tumors at an early stage. When taking antibiotics, it is always important to ensure that they are taken regularly and that the correct dosage is used. These should also not be taken with alcohol. In many cases, Parinaud syndrome reduces the life expectancy of those affected.
You can do that yourself
Children and adolescents who suffer from Parinaud’s syndrome usually suffer from permanent damage. The extent of the visual disturbance can be limited by immediately consulting a doctor.
After the diagnosis, it must be ensured that the eye can recover well. This is achieved by wearing special glasses that correct the gaze. Visual exercises, such as those used to treat strabismus, help some patients. The affected children should remain under the supervision of their parents to prevent an accident or fall. In the case of chronic complaints, changes must be made in the household. Since the child cannot see properly, the stairs must be secured. With a mildly pronounced Parinaud syndrome, it is sufficient to help the child with everyday tasks in the first few months. After a while, activities such as cycling or climbing stairs can also be carried out alone.
Parinaud’s syndrome does not require further treatment. Patients can lead a normal life with the necessary support without the need for further therapy. However, if the symptoms increase, the responsible ophthalmologist must be consulted.
