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Status Migrainosus, also known as Status Migraine, Intractable Migraine and Pernicious Migraine is defined as a severe migraine headache without aura, lasting longer than 72 hours and is classified as a complication of a migraine. There are about 100 million people with headaches in the U.S.; about 37 million of these people have migraines. The World Health Organization suggests that 18 percent of women and 7 percent of men in the U.S. suffer from migraines. A migraine headache can cause intense throbbing or a pulsing sensation in one area of the head and is commonly accompanied by nausea, vomiting, and extreme sensitivity to light and sound All migraines are categorized in two ways, migraines with auras and migraines without auras. Auras are sensory events which may precede a migraine, most are visual but others can involve other senses. A typical migraine can sometimes turn into status migrainosus.The term “status migrainosus” was coined by David Taverner in 1978, at which time he described patients with prolonged, resistant migraine attacks that resulted in incapacitation. These patients could have severe dehydration and electrolyte depletion secondary to nausea and vomiting. "Severe and prolonged, or frequently repeated migraine," Taverner wrote, "may amount to status migrainosus." While migraines are not considered fatal, status migraines are considered to be a risk factor for stroke or suicide.

Signs and symptoms

There are numerous types of migraines and seemingly countless combinations of symptoms. Symptoms can vary from one individual to the next or any individuals migraines can vary from one to the next. As for status migranosus, the only real distinction is longevity and treatment. Status migrainosus, defined by its duration, has different causes. Making it an independent entity serves mainly to clarify a protocol for management. There are four stages through which a migraine may progress. However, migraine sufferers may not always experience all four stages or some individuals may never experience certain stages. The four stages are: Prodrome, Aura, Attack, and Postdrome.

Prodrome

The prodrome phase is a group of vague symptoms that may precede a migraine attack by several hours, or even a day or two. Prodrome symptoms may include:

• Sensitivity to light or sound
• Changes in appetite, including decreased appetite or food cravings
• Thirst
• Fatigue and drowsiness
• Mood changes, including depression, irritability, or restlessness

Aura

All migraines are categorized in two ways, migraines with auras and migraines without auras. As stated earlier, status migraines are usually without Araus. Auras are sensory disturbances that occur before the migraine attack in 1 in 5 patients. Visually, auras are referred to as being positive or negative:

• Positive auras include bright or shimmering light or shapes at the edge of the field of vision called scintillating scotoma. They can enlarge and fill the line of vision. Other positive aura experiences are zigzag lines or stars.
• Negative auras are dark holes, blind spots, or tunnel vision (inability to see to the side).
• Patients may have mixed positive and negative auras. This is a visual experience that is sometimes described as a fortress with sharp angles around a dark center.

Other neurologic symptoms may occur at the same time as the aura, although they are less common. They may include:

• Speech disturbances
• Tingling, numbness, or weakness in an arm or leg
• Perceptual disturbances such as space or size distortions
• Confusion

Attack

A typical migraine attack produces the following symptoms:

• Throbbing pain on one side of the head. Pain also sometimes spreads to affect the entire head.
• Pain worsened by physical activity
• Nausea, sometimes with vomiting
• Visual symptoms
• Facial tingling or numbness
• Extreme sensitivity to light and noise
• Looking pale and feeling cold

Less common symptoms include tearing and redness in one eye, swelling of the eyelid, and nasal congestion, including runny nose. (Such symptoms are more common in certain other headaches, notably cluster headaches.)

Postdrone

After a migraine attack, there is usually a postdrome phase, in which patients may feel exhausted and mentally foggy for a while.

Causes

In general, most migraines including status migrianosus are considered idiopathic, meaning the precise cause is unknown. Both twin studies and population-based Epidemiological surveillance strongly suggest that migraine withou t aura is a multifactorial disorder, caused by a combination of genetic and environmental factors. Currently, migraine is considered a http://www.klinikum.uni-muenchen.de/Neurochirurgische-Klinik-und-Poliklinik/en/Specialities/Neurovascular_diseases/index.html involving the trigeminovascular system. The trigeminal system innervates the meninges, and provides sensory innervation to the Intracranial vessels. The Neuronal Bodies of these sensory neurons lie within the trigeminalganglia. Upon stimulation impulses travel antidromically to Dural tissue causing dilatation of the meningeal blood vessels and local release of neuropeptides – Substance P, nitric oxide, vasoactive intestinal polypeptide, 5-HT, Neurokinin A and CGRP, a potent vasodilator. This leads to plasma protein extravasation and initiation of sterile neurogenic inflammation. Regaurdless of the underlying cause, there are precipitating factors that can lead to typical migraines; some of those can be specific triggers for a Status migraine. A typical migraine can sometimes turn into status migrainosus if not treated early in the course of the attack, not treated correctly and/or headache medication is overused.

Precipitating Factors (Triggers)

Researchers have found some contributing factors thought to be responsible for triggering status migrainosus included emotional stress, depression, abuse of medications, diet, hormonal factors, and multiple nonspecific factors.

Emotional Stress / Anxiety

Stress at work or home or general anxiety can cause status migraines.

Depression

Depression can causes status migraines.

Abuse of Medications

Abuse of medications, especially those prescribed to prevent or relieve migraines can lead to status migraines.

Diet

Aged cheeses, salty foods and processed foods may trigger migraines. Skipping meals or fasting also can trigger status migraines.

Hormonal Factors

Fluctuations in estrogen seem to trigger headaches in many women with known migraines. Women with a history of migraines often report headaches immediately before or during their periods, when they have a major drop in estrogen. Others have an increased tendency to develop migraines during pregnancy or menopause. Hormonal medications, such as oral contraceptives and hormone replacement therapy, also may worsen migraines. Some women, however, may find their migraines occur less often when taking these medications.

Diagnosis

Diagnosis of a status migraine is generally a process of elimination. First an accurate diagnosis of a migraine headache vs any other cause must be made. An extensive neurologic evaluation, including CT or MRI scan and lumbar puncture, is indicated in patients presenting with their first or worst prolonged headache, particularly if it is of sudden onset or associated with focal neurologic signs, stiff neck, or changes in cognition. All patients presenting with severe, sudden-onset headache should be evaluated for a suspected aneurysm with angiography, even if the CT, MRI, and lumbar puncture show no evidence of a subarachnoid hemorrhage. Although several prospective studies have shown that a thunderclap headache is usually benign and angiography probably not necessary if neurologic examination, CT or MRI, and CSF examination performed at the time of the ictus are normal, other evidence clearly shows that an unruptured aneurysm can cause a thunderclap headache.

Differential Diagnosis of Status Migrainosus*

For each type of headache occurrence, the following is a list of maladies which need to be ruled out prior to the diagnosis of a status migrainosus. *(List is not all inclusive.)

Acute single headache

• Cerebral venous sinus thrombosis
• First attack of migraine
• Glaucoma
• Hypertensive encephalopathy
• Meningitis
• Pituitary apoplexy
• Postconcussion or post-traumatic syndrome
• Retroclival hematomas
• Sinusitis
• Spontaneous intracranial hypotension
• Subarachnoid hemorrhage
• Systemic infection

Acute recurrent headache

• Cerebral tumors
• Cerebrovascular insufficiency
• Idiopathic intracranial hypertension
• Intermittent hydrocephalus
• Migraine
• Subarachnoid hemorrhage
• Subacute headache (days or weeks)

Brain abscess

• Pseudotumor cerebri
• Subdural hematoma
• Temporal arteritis (in elderly)
• Tumor
• Chronic daily headache (months or years)

Analgesic rebound

• Chronic tension-type headache
• Pseudotumor cerebri
• Psychiatric state
• Tumor

Secondly, the attack needs to have been continuous and refractory to standard treatments for in excess of 72 hours. Finally, the pain of attack is characterized as severe.

Treatment

The treatment of status migraines is difficult and not well defined. Status migraine attacks do not respond to standard treatments for migraines and often require hospitalization. Acute inpatient hospitalization for the treatment of headache without a serious underlying organic medical condition should be considered rarely, and the goals of admission should be clearly defined. Patients with a severe, persistent headache (status migrainosus or chronic daily headache) may have headache despite, or because of, the use of analgesics, narcotics, ergots, and triptans. The pathophysiology responsible for this type of evolution is still subject to much controversy and due to the common failure of the usual treatment in such circumstances, various approaches in bouts of severe migraine with anesthetic drugs have been made, from local endovenous (intravenous) anesthetics to opioids, and also with hypnotics as propofol. This latter strategy has come to very interesting results, and this alleged action would be the interaction with the central GABA receptors, with a mechanism similar to other anesthetic agents, including inhaled isoflurane not necessarily in the same receptor subtypes. Therefore, other drugs with similar mechanism of action in these receptors also have been a target of research for the treatment of migraine. Ergotamine's role (in common migraines) since the introduction of triptans is now less certain. However, Ergotamine may still be helpful for patients with status migrainous or those with frequent recurring headaches.

Medication

Medications include both anti-emetics for vomiting as well as a variety of drugs to alleviate the headache.

Anti-emetics metoclopramide, (Metozolv, Reglan), chlorpromazine, prochlorperazine and (Compro, Procomp)

Headache Alleviation dihydroergotamine, Intervenous Anesthetics, opioids, propofol, isoflurane and fentanyl

Pregnancy –The major concern in managing the pregnant migraineur is the effect of both medication and migraine on the fetus. Severe intractable headaches associated with nausea and vomiting can lead to dehydration. These conditions not only are disruptive to the patient, but may pose a risk to the fetus that is greater than the potential risk of the medications used to treat the pregnant patient. Migraines frequently worsens during the first trimester and can result in status migrainosus. Because of the risk to the fetus, this condition should be treated aggressively. Intravenous fluids should be administered for hydration. Intravenous narcotics or corticosteroids can be added if necessary. Acetaminophen (alone or with codeine), codeine alone, or other narcotics can be used during pregnancy. Ergotamine, dihydroergotamine, and sumatriptan should be avoided. One case report was found in which labetalol was used successfully to reduce the frequency and intensity of migraine attacks in a pregnant woman with preeclampsia. Other medications often used in status migrainosus should be approached with caution due to their potential adverse effects on the fetus and the pregnancy, such as NSAIDS (preterm labor) and phenothiazines.

Prophylactic Management

Status migraines attacks do not generally respond to standard treatments for migraines, yet drugs designed to prevent migraines may still have benefits. If the headache is of long duration, depression, low frustration tolerance, and physical and emotional dependency on abortive medication may coexist. Overused drugs must be withdrawn for prophylactic medication to be effective. While these prophylactics may not completely prevent a migraine, the may reduce the severity of an attack, thus reducing the risk of it escalating to a status Migrainosous. These prophylactic medications include:

Beta-blockers. These drugs, which are also used for reducing blood pressure, are a common preventive treatment for people with frequent migraines. They include atenolol (Tenormin), metoprolol (Lopressor, Toprol), nadolol (Corgard), and propranolol (Inderal, InnoPran, Pronol).

Calcium Channel Blockers, which are also used for high blood pressure.

Antidepressants known as tricyclics (such as amitriptyline) and SNRIs (such as venlafaxine).

Anti-seizure medicines such as valproate and topiramate.

Botox. Though better known as a treatment for wrinkles, Botox has been approved by the FDA to prevent chronic migraines.

Recent Research

General Anesthetic to Treat Status Migrainosus

The use of general anesthetic has recently shown promise as a treatment for Status Migrainosus. A Female patient, aged 50 years, with status migrainosus, in the last five days with visits to the emergency department, medicated parenterally with various agents without result. Without comorbidities, dehydrated, described her pain as “well over 10” in Visual Numeric Scale (VNS). After consulting the literature, and given the apparent severity of the condition, we opted for a general anesthesia: induction with fentanyl, propofol, and vecuronium and maintenance with isoflurane and propofol for two hours. Following the treatment, in the postanesthetic recuperation (PAR), the patient related her pain as VNS 3, and was released after five hours with VNS 2. Subsequently, her preventive treatment was resumed.

Peripheral nerve blocks in the treatment of migraine in pregnancy

Peripheral nerve blocks were performed for pregnant status migrainosus patients. Before the peripheral nerve blocks were performed, oral medications failed for all patients and intravenous medications failed for most. The average pain reduction on a VNS was 4.0 (±2.6 standard deviation) (P<.001) immediately post-procedure and 4.0 (±4.4 standard deviation) (P=.007) 24 hours post-procedure in comparison to pre-procedure pain.

Status migrainosus as an initial presentation of multiple sclerosis

A status migraine headache could be the first presenting symptom of Multiple Sclerosis. A 25 year old female patient first presented with a status migrainosus per the criteria of International Classification of Headache Disorder, third edition (ICHD-3). She continued to suffer from status migraines. Although she continued to use standard abortive medications, standard prophylactic medications were discontinued due to ineffectiveness. However, she later presented with symptoms of MS and an MRI confirmed the diagnosis. She was started on oral fingolimod as disease modifying therapy. The headaches entered a remission phase for the next following months. Further research is needed to confirm or refute this possible connection. Status migrainosus in the presence of demyelinating MRI features at the initial presentation may pose diagnostic dilemma. Failure to respond to prophylactic migraine therapy in view of progressive demyelinating features on follow-up MRIs may point to another relatively common neurological disorder such as MS among young women.