Skip to main content

Tolosa-Hunt Syndrome

Tolosa-Hunt Syndrome


OVERVIEW

Tolosa-Hunt syndrome is a rare disorder characterized by severe periorbital headaches, along with decreased and painful eye movements (ophthalmoplegia). Symptoms usually affect only one eye (unilateral). In most cases, affected individuals experience intense sharp pain and decreased eye movements. Symptoms often will subside without intervention (spontaneous remission) and may recur without a distinct pattern (randomly). Affected individuals may exhibit signs of paralysis (palsy) of certain cranial nerves such as drooping of the upper eyelid (ptosis), double vision (diplopia), large pupil, and facial numbness. The affected eye often abnormally protrudes (proptosis). The exact cause of Tolosa-Hunt syndrome is not known, but the disorder is thought to be associated with inflammation of specific areas behind the eye (cavernous sinus and superior orbital fissure).

Signs & Symptoms

Many individuals with Tolosa-Hunt syndrome experience the sudden onset of severe periorbital headache, followed by painful and decreased eye movements (ophthalmoplegia). In some cases of severe ophthalmoplegia, the eye itself is unable to move or look in various directions (frozen globe).

The major symptoms of Tolosa-Hunt syndrome include chronic periorbital headache, double vision, paralysis (palsy) of certain cranial nerves, and chronic fatigue. Affected individuals may also exhibit protrusion of the eye (proptosis), drooping of the upper eyelid (ptosis) and diminished vision. In most cases, symptoms associated with Tolosa-Hunt syndrome affect only one side (unilateral). Symptoms will usually subside without intervention (spontaneous remission) and may recur without a distinct pattern (randomly).

Causes

While the exact cause of Tolosa-Hunt syndrome is unknown, one theory is an abnormal autoimmune response linked with an inflammation in a specific area behind the eye (cavernous sinus and superior orbital fissure). In some cases, inflammation may be due to a clumping of a certain type of cell (granulomatous inflammation). Autoimmune disorders are caused when the body’s natural defenses against “foreign” or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons. Other possible causes may include generalized inflammation and constricted or inflamed cranial blood vessels.

Disorders with Similar Symptoms

Symptoms of the following disorders can be similar to those of Tolosa-Hunt syndrome. Comparisons may be useful for differential diagnosis:

Orbital cellulitis is characterized by inflammation of the tissues within the cavity which holds the eyeball. Symptoms include extreme pain, impaired eye movement, swelling, fever and a general feeling of discomfort. Possible complications may include impaired vision, vein abnormalities and spread of the inflammation to the entire orbit, brain or the membranes surrounding the brain.

Cavernous sinus thrombosis is an ophthalmologic disorder usually caused by infection and clotting in veins behind the eyeball. It can be a complication of Orbital Cellulitis or infections of facial skin. Swelling and protrusion of both eyes, fever, headache, poor eye movements, droopy eyelids, facial numbness, and an overly sick appearing patient are symptoms of this deadly disorder. Prompt treatment with antibiotics and blood thinners and rest is recommended.

Migraine headaches usually involve one side of the head like the Tolosa-Hunt syndrome. Individuals who suffer from these intense headaches may have a genetic predisposition to them. Often associated with these painful attacks are irritability, nausea, vomiting, constipation or diarrhea, and sensitivity to light. Medical researchers believe constriction of the cranial arteries may precede migraine headaches in some cases. Poor eye movements are not characteristic of migraine headaches and should alert physicians to the possibility of Tolosa-Hunt syndrome.

Diagnosis

The diagnosis of Tolosa-Hunt syndrome has been codified by the International Headache Society. The following criteria must be met for a definitive diagnosis of this disorder: Eye pain on one side of the head that persists for at least eight weeks if untreated; associated irritation or damage to the third, fourth, or sixth cranial nerves; relief of pain within 48 hours upon the administration of steroids; and specialized testing that rules out other conditions such as neoplasm, infection or aneurysm.

The diagnosis of Tolosa-Hunt syndrome is suspected based upon the presence of characteristic physical features (e.g., pain, headache, ophthalmoplegia). The diagnosis may be confirmed by a thorough clinical evaluation, detailed patient history, and a variety of specialized radiologic tests including computed tomography (CT) scan, and magnetic resonance imaging (MRI). These examinations may reveal characteristic enlargement or inflammation of the areas behind the eye (cavernous sinus and superior orbital fissure).

Treatment

In most cases, the pain associated with Tolosa-Hunt syndrome subsides with short-term use of steroid drugs. Pain is usually reduced in untreated cases within fifteen to twenty days. With steroid treatment, pain typically briskly subsides within twenty-four to seventy-two hours – and this brisk steroid response aids in the diagnosis. Affected individuals may be vulnerable to recurrent future attacks.

TYPE OF DOCTOR AND DEPARTMENT: Neurologist SPECIALIST CAN DIAGNOSES THIS DISEASE.

Comments

Post a Comment

Popular posts from this blog

Charge Syndrome

Overview CHARGE syndrome is a recognizable genetic syndrome with known pattern of features. It is an extremely complex syndrome, involving extensive medical and physical difficulties that differ from child to child. CHARGE syndrome is correlated with genetic mutation to CHD7 and the prevalence of CHARGE syndrome is 1:10,000-1:15,000 live births. Babies with CHARGE syndrome are often born with life-threatening birth defects. They spend many months in the hospital and undergo many surgeries and other treatments. Swallowing and breathing problems make life difficult even when they come home. Most have hearing two little girls sitting on a carpet, one girl has a trach and is biting her finger.loss, vision loss, and balance problems that delay their development and communication. Despite these seemingly insurmountable obstacles, children with CHARGE syndrome often far surpass their medical, physical, educational, and social expectations. One of the hidden features of CHARGE syndrome is the ...
7 comments
Read more

Legg–Calve–Perthes disease

  Legg–Calve–Perthes disease Overview Legg-Calve-Perthes (LEG-kahl-VAY-PER-tuz) disease is a childhood condition that occurs when blood supply to the ball part (femoral head) of the hip joint is temporarily interrupted and the bone begins to die. This weakened bone gradually breaks apart and can lose its round shape. The body eventually restores blood supply to the ball, and the ball heals. But if the ball is no longer round after it heals, it can cause pain and stiffness. The complete process of bone death, fracture and renewal can take several years. To keep the ball part of the joint as round as possible, doctors use a variety of treatments that keep it snug in the socket portion of the joint. The socket acts as a mold for the fragmented femoral head as it heals. Symptoms Symptoms of Perthes disease include: Limping. Pain or stiffness in the hip, groin, thigh or knee. Limited range of motion of the hip joint. Pain that worsens with activity and improves with rest. Perthes diseas...
Post a Comment
Read more

Kernicterus

  Kernicterus Overview Kernicterus is a rare condition that affects your baby’s brain when they have too much bilirubin in their blood (hyperbilirubinemia). Bilirubin is a yellow waste product that your body makes. Sometimes, your liver can’t remove enough bilirubin to keep you healthy. Too much bilirubin can cause jaundice. This is when your skin, the whites of your eyes and your gums or the area underneath your tongue (mucous membranes) appear yellow. Symptoms of kernicterus progress in stages. In addition to jaundice, symptoms usually affect newborns and include irritability, poor feeding and seizures. Complications can lead to hearing loss and permanent brain damage. If you notice changes to your newborn’s behavior or appearance, contact their healthcare provider immediately. You may hear your healthcare provider call kernicterus “bilirubin encephalopathy.” Jaundice is common in newborns. Healthcare providers will monitor newborn jaundice to decrease your baby’s risk of develop...
Post a Comment
Read more