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Rare Migraine Variants – Migraine Imitators – Diagnostic Testing in Migraine
Neurologist is the best specialist for diagnosis and treatment of migraine. Migraine is often misdiagnosed as a “sinus pain” because of frequent association with pain and swelling in the face. Sinusitis pain does not have periodic pattern. Sinusitis is often associated with fever and hot, red, and painful to touch forehead and the upper face. Sinus related pain is worse at night or upon awakening and lessens during the day and it is typically head position dependent.
Glaucoma (increased pressure in the eye) sometimes imitates migraine. Acute viral disease and meningitis often cause typical migraine-like throbbing pain with sensitivity to light and vomiting. Head injuries sometimes followed by migraine attacks even in those people who normally do not have migraines.
Giant cell arteritis (Temporal arteritis) is an autoimmune inflammation of large and medium caliber head and neck blood vessels. Headache in giant cell arteritis may involve either one or both sides of the head. The pain is felt rather in the scalp and often has allodynia like qualities. Temporal arteritis is typical after age 55. Jaw often gets tired while chewing. Giant cell arteritis requires urgent care because it can cause permanent loss of vision.
AVMs or Arteriovenous Malformations (abnormal blood vessels in the brain) may simulate migraine with aura. Strokes don’t cause migraine-like pain, but migraine aura is often hard to differentiate from stroke.
Vestibular migraine can be imitated by multiple conditions described in Recurrent vertigo section. Some rear genetic disorders have migraine as a symptom.
CADASIL or Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy
CADASIL is a rear inherited brain microvasculopathy (disease of small blood vessels). In some people this disease starts as a typical migraine with aura. Years down the road multiple recurrent strokes lead to depression and dementia (gradual cognitive decline). This disease was featured in Amenábar’s movie “Sea inside”. MRI reveals an extensive white matter disease of the brain. The diagnosis is made by genetic testing (Notch 3 gene mutation on chromosome 19).
Multiple mitochondrial disorders, such as MELAS, MERRF and Leber hereditary optic neuropathy have migraines as one of their symptoms.
Here is a typical clinical picture for CADASIL: gradual loss of motivation, depression, loss of interest to his routine activities, urine incontinence, and eventually inability to walk. This patient (Brain MRI image is on the right) never had migraine. His vision was preserved at the time of the last examination. Expressive speech, comprehension and other higher integrative functions were preserved. There was a strikingly poor attention, which was expected. Such an extensive and rapidly progressive microvascular disease is unlikely to be due to diabetes and high blood pressure. Symptoms started in early 60s and he reached the stated point within 2 years. Further history is not known. Genetic testing could not be performed due to insurance carrier denial of coverage.
Hemiplegic migraine is a rear disease. Some forms are inherited. Aura in this type of migraine is dramatic. It can present as weakness on one side of the body, weakness in both legs, or speech arrest. The aura can last from hours to days. Prolonged aura can cause confusion or even coma, fever and symptoms of meningitis. Hemiplegic migraine often starts in the childhood. Attacks may be brought up by minor head injuries. An individual patient may have from one to multiple attacks of hemiplegic migraine during one’s life time.
Headache may start during or after the aura. Severe and prolonged symptoms are less frequent than the milder ones. Patients with hemiplegic migraine usually have migraine attacks without aura or with different types of aura.
Isolated hemiplegic migraine aura without a headache, or migraine equivalent, is inevitably diagnosed as a “stroke”. There is one feature of hemiplegic migraine, which is very helpful in correct diagnosis. This is an extent of neurological symptoms. Ischemic strokes follow particular anatomy dictated patterns of brain circulation and are rarely associated with confusion early in the course. On the contrary, hemiplegic migraine has symptoms originating all over the brain at the same time. Neurological deficits are confusing in their localization. It is impossible to put all of them together.
Basilar migraine aura has multiple symptoms: loss of the half of visual field, which may later spread to the other side, vertigo, noise in the ears, loss of hearing, double vision, lack of balance, tingling and weakness on both sides of the body or confusion. Vomiting is common.
Basilar migraine aura may be followed by headache. Headache free attacks are not uncommon. Basilar migraine aura rarely lasts longer than an hour. Similar to hemiplegic migraine, stroke is the major concern in basilar migraine.
Ocular or Retinal Migraine
Ocular migraine starts with partial or complete loss of vision in one eye followed by a headache. Loss of vision may last for days to weeks or may be permanent. This type of aura is likely caused by a spasm of the retinal artery. Ocular migraine is most common in young adults. There is a phenomenon known well to ophthalmologist, which presents as periodic transient loss of vision in one eye caused by retinal arteries spasm. These two conditions probably have something in common.
Important: Don’t confuse “retinal migraine”, caused by retinal vasospasm with a “classical” visual aura, which is caused by brain dysfunction.
Abdominal migraine presents with stomachache, nausea and vomiting. Typical attack duration is from 1 to 3 days. This form of migraine is rear in adults.
Three quarters of children with abdominal migraine have or will develop a typical migraine within 10 years. Migraine can present in childhood by vomiting alone (cyclic vomiting) with development of head pain attacks later in life.
Diagnostic Testing in Migraine
Instrumental diagnostic testing is not required for typical migraine. Neurological evaluation is usually sufficient. There is no confirmatory test for migraine. Diagnostic testing is done to rule out secondary headaches.
Some headache features, such as sudden onset of the headache, prolonged aura, stiff neck, fever, or lack of typical migraine associated symptoms should trigger concerns over different than migraine reasons for the headache.
The most common diagnostic modalities are:
Brain MRI, Brain MRA, Neck MRA, CT head, Carotid Duplex, VNG, Ophthalmologist consultation; blood tests: ESR, CRP, ANA, RF; and a spinal tap.
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