Tolosa-Hunt syndrome
Encyclopedia
Tolosa-Hunt syndrome is a rare disorder
characterized by severe and unilateral headaches with extraocular palsies
, usually involving the third, fourth, fifth, and sixth cranial nerves, and pain around the sides and back of the eye, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles.
In 2004, the International Headache Society provided a definition of the diagnostic criteria which included granuloma
.
and superior orbital fissure).
In addition, affected individuals may experience paralysis of various facial nerves and drooping of the upper eyelid (ptosis
). Other signs include double vision
, fever
, chronic fatigue, vertigo
or arthralgia
. Occasionally the patient may present with a feeling of protrusion of one or both eyeballs (exophthalmos
).
, thyroid
function tests and serum protein electrophoresis
. Studies of cerebrospinal fluid
may also be beneficial in distinguishing between THS and conditions with similar signs and symptoms.
MRI scans of the brain and orbit
with and without contrast, magnetic resonance angiography
or digital subtraction angiography
and a CT scan of the brain and orbit with and without contrast may all be useful in detecting inflammatory
changes in the cavernous sinus, superior orbital fissure and/or orbital apex. Inflammatory change of the orbit on cross sectional imaging in the absence of cranial nerve palsy is described by the more benign and general nomenclature of orbital pseudotumor.
Sometimes a biopsy
may need to be obtained to confirm the diagnosis, as it is useful in ruling out a neoplasm.
Differentials to consider when diagnosing THS include craniopharyngioma
, migraine
and meningioma
.
) and immunosupressive agents (such as Methotrexate
or Azathioprine
). Corticosteroids act as analgesia and reduce pain (usually within 24–72 hours), as well as reducing the inflammatory mass, whereas immunosupressive agents help reduce the autoimmune response. Treatment is then continued in the same dosages for a further 7–10 days and then tapered slowly.
Radiotherapy has also been proposed.
.
and internationally. In New Zealand, there is only one recorded case . Both genders, male and female, are affected equally, and it typically occurs around the age of 60.
Rare disease
A rare disease, also referred to as an orphan disease, is any disease that affects a small percentage of the population.Most rare diseases are genetic, and thus are present throughout the person's entire life, even if symptoms do not immediately appear...
characterized by severe and unilateral headaches with extraocular palsies
Palsy
In medicine, palsy is the paralysis of a body part, often accompanied by loss of sensation and by uncontrolled body movements, such as shaking. Medical conditions involving palsy include cerebral palsy , brachial palsy , and Bell's palsy ....
, usually involving the third, fourth, fifth, and sixth cranial nerves, and pain around the sides and back of the eye, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles.
In 2004, the International Headache Society provided a definition of the diagnostic criteria which included granuloma
Granuloma
Granuloma is a medical term for a tiny collection of immune cells known as macrophages. Granulomas form when the immune system attempts to wall off substances that it perceives as foreign but is unable to eliminate. Such substances include infectious organisms such as bacteria and fungi as well as...
.
Causes
The exact cause of THS is not known, but the disorder is thought to be, and often assumed to be, associated with inflammation of the areas behind the eyes (cavernous sinusCavernous sinus
The cavernous sinus , within the human head, is a large collection of thin-walled veins creating a cavity bordered by the temporal bone of the skull and the sphenoid bone, lateral to the sella turcica.-Contents:...
and superior orbital fissure).
Signs and Symptoms
Symptoms are usually limited to one side of the head, and in most cases the individual affected will experience intense, sharp pain and paralysis of muscles around the eye. Symptoms may subside without medical intervention, yet recur without a noticeable pattern.In addition, affected individuals may experience paralysis of various facial nerves and drooping of the upper eyelid (ptosis
Ptosis (eyelid)
Ptosis is a drooping of the upper or lower eyelid. The drooping may be worse after being awake longer, when the individual's muscles are tired. This condition is sometimes called "lazy eye", but that term normally refers to amblyopia...
). Other signs include double vision
Diplopia
Diplopia, commonly known as double vision, is the simultaneous perception of two images of a single object that may be displaced horizontally, vertically, or diagonally in relation to each other...
, fever
Fever
Fever is a common medical sign characterized by an elevation of temperature above the normal range of due to an increase in the body temperature regulatory set-point. This increase in set-point triggers increased muscle tone and shivering.As a person's temperature increases, there is, in...
, chronic fatigue, vertigo
Vertigo (medical)
Vertigo is a type of dizziness, where there is a feeling of motion when one is stationary. The symptoms are due to a dysfunction of the vestibular system in the inner ear...
or arthralgia
Arthralgia
Arthralgia literally means joint pain; it is a symptom of injury, infection, illnesses or an allergic reaction to medication....
. Occasionally the patient may present with a feeling of protrusion of one or both eyeballs (exophthalmos
Exophthalmos
Exophthalmos is a bulging of the eye anteriorly out of the orbit. Exophthalmos can be either bilateral or unilateral . Measurement of the degree of exophthalmos is performed using an exophthalmometer...
).
Diagnosis
THS is usually diagnosed via exclusion, and as such a vast amount of laboratory tests are required to rule out other causes of the patient's symptoms. These tests include a complete blood countComplete blood count
A complete blood count , also known as full blood count or full blood exam or blood panel, is a test panel requested by a doctor or other medical professional that gives information about the cells in a patient's blood...
, thyroid
Thyroid
The thyroid gland or simply, the thyroid , in vertebrate anatomy, is one of the largest endocrine glands. The thyroid gland is found in the neck, below the thyroid cartilage...
function tests and serum protein electrophoresis
Serum protein electrophoresis
Serum protein electrophoresis is a laboratory test that examines specific proteins in the blood called globulins. Blood must first be collected, usually into an airtight vial or syringe...
. Studies of cerebrospinal fluid
Cerebrospinal fluid
Cerebrospinal fluid , Liquor cerebrospinalis, is a clear, colorless, bodily fluid, that occupies the subarachnoid space and the ventricular system around and inside the brain and spinal cord...
may also be beneficial in distinguishing between THS and conditions with similar signs and symptoms.
MRI scans of the brain and orbit
Orbit
In physics, an orbit is the gravitationally curved path of an object around a point in space, for example the orbit of a planet around the center of a star system, such as the Solar System...
with and without contrast, magnetic resonance angiography
Magnetic Resonance Angiography
Magnetic resonance angiography is a group of techniques based on Magnetic Resonance Imaging to image blood vessels. Magnetic resonance angiography is used to generate images of the arteries in order to evaluate them for stenosis , occlusion or aneurysms...
or digital subtraction angiography
Digital subtraction angiography
Digital subtraction angiography is a type of fluoroscopy technique used in interventional radiology to clearly visualize blood vessels in a bony or dense soft tissue environment. Images are produced using contrast medium by subtracting a 'pre-contrast image' or the mask from later images, once...
and a CT scan of the brain and orbit with and without contrast may all be useful in detecting inflammatory
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...
changes in the cavernous sinus, superior orbital fissure and/or orbital apex. Inflammatory change of the orbit on cross sectional imaging in the absence of cranial nerve palsy is described by the more benign and general nomenclature of orbital pseudotumor.
Sometimes a biopsy
Biopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
may need to be obtained to confirm the diagnosis, as it is useful in ruling out a neoplasm.
Differentials to consider when diagnosing THS include craniopharyngioma
Craniopharyngioma
Craniopharyngioma is a type of brain tumor derived from pituitary gland embryonic tissue, that occurs most commonly in children but also in men and women in their 50s and 60s....
, migraine
Migraine
Migraine is a chronic neurological disorder characterized by moderate to severe headaches, and nausea...
and meningioma
Meningioma
The word meningioma was first used by Harvey Cushing in 1922 to describe a tumor originating from the meninges, the membranous layers surrounding the CNS ....
.
Treatment
Treatment of THS is usually completed using corticosteroids (often PrednisonePrednisone
Prednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant drug. It is used to treat certain inflammatory diseases and some types of cancer, but has significant adverse effects...
) and immunosupressive agents (such as Methotrexate
Methotrexate
Methotrexate , abbreviated MTX and formerly known as amethopterin, is an antimetabolite and antifolate drug. It is used in treatment of cancer, autoimmune diseases, ectopic pregnancy, and for the induction of medical abortions. It acts by inhibiting the metabolism of folic acid. Methotrexate...
or Azathioprine
Azathioprine
Azathioprine is a purine analogue immunosuppressive drug. It is used to prevent organ rejection following organ transplantation and to treat a vast array of autoimmune diseases, including rheumatoid arthritis, pemphigus, inflammatory bowel disease , multiple sclerosis, autoimmune hepatitis, atopic...
). Corticosteroids act as analgesia and reduce pain (usually within 24–72 hours), as well as reducing the inflammatory mass, whereas immunosupressive agents help reduce the autoimmune response. Treatment is then continued in the same dosages for a further 7–10 days and then tapered slowly.
Radiotherapy has also been proposed.
Prognosis
The prognosis of THS is usually considered good. Patients usually respond to corticosteroids, and spontaneous remission can occur, although movement of ocular muscles may remain damaged. Roughly 30%-40% of patients who are treated for THS experience a relapseRelapse
Relapse, in relation to drug misuse, is resuming the use of a drug or a dependent substance after one or more periods of abstinence. The term is a landmark feature of both substance dependence and substance abuse, which are learned behaviors, and is maintained by neuronal adaptations that mediate...
.
Epidemiology
THS is uncommon in both the United StatesUnited States
The United States of America is a federal constitutional republic comprising fifty states and a federal district...
and internationally. In New Zealand, there is only one recorded case . Both genders, male and female, are affected equally, and it typically occurs around the age of 60.