 Unique Neurological Conditions
Frequently
Asked Questions
Click here to read
the content disclaimer
Unique Neurological Conditions (by MHNI Staff)
Arnold-Chiari malformation
More than a tongue-twister, Arnold-Chiari malformation is a well-described congenital
(present at birth) abnormality of the cerebellum and brain stem. While four different
types have been described, types 1 and 2 are most common. Types 2, 3, and 4 are
typically diagnosed in infancy because of abnormalities involving the skull or spinal
cord. However, type 1, considered a milder form of the condition, may not cause
symptoms until adulthood.
In all types, changes affect the posterior part of the brain and spinal cord. Instead
of fitting into the posterior fossa, a portion of the cerebellum (the "balance center"
of the brain) squeezes through the base of the skull next to the spinal cord. This
can cause changes within the spinal cord, ranging from a relative flattening of
the cord to a collection of fluid within the cord (called a syrinx). Increased fluid
in the brain (hydrocephalus) can also develop.
The headache associated with Arnold-Chiari may take on various forms, including
an intermittent form or a persistent one. The pain may be frontal (as in the forehead
area) or in the neck or back of the head area. Characteristically, but not always,
the pain may worsen with neck movement, coughing, or straining movements. Associated
neck pain is a frequent complaint. Upon examination, other problems may be found
including incoordination, difficulty swallowing, impaired eye movements, sensory
disturbances, and others. Arnold-Chiari is diagnosed by an MRI of the brain and
spinal cord but cannot be identified by a CT scan.
If a severe malformation is present, neurosurgery may be needed to eliminate pressure
on various parts of the brain and spinal cord and relieve associated symptoms. If
surgical intervention is not required, treatment is typically based upon symptoms.
For example, daily or periodic (abortive) medications are administered for headaches
in a manner similar to treatment for headaches caused by other factors.
Back to Top | Back to FAQs
Benign paroxysmal positional
vertigo
Benign paroxysmal positional vertigo, or BPPV, is probably the most common single
cause of vertigo, a sense of spinning, in the United States. It has been estimated
that at least 20% of all patients who complain of vertigo have BPPV. However, because
it is frequently misdiagnosed, this figure is most likely an underestimation.
BPPV, which was first described by Barany in 1921, causes vertigo due to debris
collecting within a part of the inner ear. This debris, or "ear rocks," is comprised
of small crystals of calcium carbonate. The debris is believed to derive from structures
in the ear called "otoliths" that may have been damaged by head injury, infection,
disorders of the inner ear, or degenerated because of advanced age. However, in
about half of the people affected, the cause of BPPV remains unknown.
In the most common variety of BPPV, symptoms are usually triggered by movement of
the head, such as when turning rapidly, rotating in bed, or sitting up or lying
down. Symptoms may begin suddenly and can persist for quite some time or dissipate
within 20-30 seconds. The major symptoms of BPPV are vertigo, lightheadedness, imbalance,
and nausea. The offending position may sometimes lead to a sense of fluttering of
the eyes (nystagmus) which is also a common component of the vertigo.
A diagnosis of BPPV can be made through a comprehensive history, examination, and
a review of vestibular and auditory test results. Other diagnostic studies such
as an electronystagmyography (ENG) may be required. An MRI may be performed to rule
out other causes of vertigo.
Various treatment options are available to address BPPV. Many experts argue that
since BPPV is benign and can resolve on its own in weeks to months, simple observation
is all that is needed. However, symptoms can be disabling and may necessitate intervention
for many sufferers.
For some people, certain medications may be very helpful. However, they often provide
only minimal relief, do not solve the underlying problem, and may lead to significant
side effects including sleepiness.
Various kinds of physical maneuvers have been found to be effective in treating
BPPV. The two most common repositioning techniques are the "Seamont method" and
the canalith reposition procedure (CRP), or "Epley method." Both involve rotating
the head to particular positions to rearrange and displace the "ear rocks." The
Seamont maneuver involves rapid and vigorous side-to-side head and body movements.
The CRP or Epley maneuver is a simple, noninvasive, office treatment which is designed
to actually cure BPPV in 1-2 sessions. This therapy, when performed by experienced
professionals, has been significantly successful for some patients with BPPV. However,
surgery is sometimes required.
For many sufferers, especially those suffering from classic BPPV, certain modifications
in daily activities may be necessary. Patients should avoid sleeping on their "bad"
side and should rise slowly and sit on the edge of the bed for a minute when awakening
in the morning. In addition, those who suffer this condition should avoid rapid
bending down, such as to pick things up, or extending their necks, such as when
looking upward.
Back to Top | Back to FAQs
Chronic paroxysmal hemicrania
Chronic paroxysmal hemicrania (CPH) is a unique headache syndrome that falls into
a category known as "indomethacin responsive headaches." It is very similar to cluster
headache but is less common. In fact, some authors have labeled CPH as "atypical
cluster headache."
CPH patients have multiple headaches per day (on average greater than 5), more than
what is seen in cluster headache. Each attack is usually short-lived, with most
lasting between 5 and 30 minutes. The pain is always one-sided, very severe, and
seems to be concentrated around the eye, temple, and forehead region. There are
associated symptoms of watering of the eye and eye redness; even drooping of the
eyelid has been reported.
Unlike cluster headache, CPH is usually seen in females. Children are not immune
to this condition-it has been reported to occur in girls as young as six years old.
Although patients affected may wake up with an attack, this does not seem to occur
as regularly as it might in persons with cluster headache. In addition, the nausea
and vomiting which seem to be so prevalent for patients with migraine are not seen
in CPH.
Individuals who experience CPH may have remissions of their attacks and pain, at
times lasting many months. They may also not be able to identify specific triggers
for onset of another attack.
While we have not yet identified the specific pathophysiology (underlying physical
cause) behind CPH, it seems that the trigeminal nerve is involved. Unfortunately,
many treatments that are effective in treating other forms of pain caused by a similar
mechanism do not seem to work when treating CPH. One medication that will always
eliminate CPH is indomethacin; hence the term "indomethacin responsive headaches."
For patients who are unable to use this medication, other treatment strategies are
being evaluated. However, other headache medications, such as triptans, usually
do not have an effect on CPH.
Although MHNI physicians are usually able to make a diagnosis of CPH based on a
person's history and clinical symptoms, there are underlying lesions in the brain
that can lead to similar symptoms and attacks (e.g., tumors, arteriovenous malformation).
Because of this, various tests of the brain are recommended to exclude structural
abnormalities.
Back to Top | Back to FAQs
Cyclic vomiting syndrome
Cyclic vomiting syndrome (CVS) is an uncommon disorder affecting primarily children
and some adults. The onset of CVS occurs most commonly between the ages of 3 and
7, equally in males and females. It is characterized by recurrent, prolonged episodes
of severe nausea, vomiting, confusion, and extreme exhaustion.
Episodes often begin at night or upon awakening and usually last 1-4 days with vomiting
occurring at frequent intervals. Episodes can recur several times a month to several
times a year. Between episodes, the patient feels and appears well.
Although some attacks occur for no apparent reason, CVS can be triggered by emotional
distress, excitement, illness, food sensitivities, menstruation, injury or pain.
Often there is a family history of migraine headache. Frequently in adolescence
CVS evolves into migraine headaches or may disappear altogether.
Diagnosis of CVS is made more difficult because there are no medical tests, such
as blood tests or x-rays, which diagnose it definitively, and many other disorders
have vomiting as a symptom.
Early treatment of CVS is important to prevent dehydration which, if severe, may
require hospitalization and IV fluids. Medicines that have traditionally been used
to control vomiting (anti-emetics) are usually not effective. However, newer anti-emetics
that block the actions of brain serotonin at certain receptors (5-HT3) may be helpful.
Medications used to treat migraines, such as tricyclic antidepressants, beta blockers,
and the "triptans," may also be effective. Investigational drugs that block the
effects of certain brain chemicals (tachykinins) also hold hope for more effective
treatment in the future.
In addition, stress management, biofeedback, and the avoidance of known triggers
can be quite helpful.
Back to Top | Back to FAQs
Dizziness
"Dizziness" is a symptom that frequently motivates a patient to seek medical attention.
The sensation of dizziness may be experienced as light-headedness, spinning of the
room or the body, swaying, visual blurring, or feelings of unreality.
It is important that descriptions of "dizziness" include the specific sensations
experienced, since neurologists distinguish true vertigo (a sensation of rotation
or spinning of the environment or the head) from non-vertiginous forms of dizziness.
Causes of non-vertiginous dizziness can include low blood pressure, abnormal heart
function, infections, medication side effects, or even severe anemia.
True vertigo may be caused by disease of the inner ear, brain, or brainstem disease.
Inner ear disease may involve hearing loss, tinnitus (ringing in the ears), or ear
pain, as well as certain physical exam and laboratory findings. Central nervous
system diseases of the brain or brainstem that cause vertigo may involve poor motor
coordination or loss of consciousness, as well as certain laboratory abnormalities.
True vertigo may also be caused by medications, toxins, seizures, Meniere's disease,
migraine, motion sickness, multiple sclerosis, anxiety, or trauma.
Treatment options depend on the underlying cause. Effective measures may include
medications (e.g., antibiotics for inner ear infections, diuretics for Meniere's
disease, anticonvulsants for seizure disorders, anti-anxiety medications or antidepressants),
psychotherapy, specific body positioning maneuvers for certain inner ear disorders,
specialized diets (e.g., low salt diet for Meniere's disease), or removal of an
offending medication.
A thorough examination and review by a knowledgeable physician is vital for the
proper diagnosis and treatment of dizziness. Assessment may include checking blood
pressure, performance of diagnostic and laboratory tests, or behavioral medicine
evaluation.
Back to Top | Back to FAQs
Exploding noises in the head
Sometimes the most awful symptom can be entirely harmless. Such is the case with
a rare condition called exploding head syndrome.
More than 55 cases have been reported in the medical literature, but it is likely
that hundreds, if not thousands, of patients have experienced this phenomenon.
The condition is characterized by a terrifying sense of explosive noise in the head,
and typically occurs within an hour or two after falling asleep. Individuals report
that they are awakened by a startling and frightening massive sound that can be
clearly distinguished from a dream. It occurs exclusively at night and is not accompanied
by any pain.
Activities prior to retiring at bedtime do not appear to have any relationship to
the sound, although three physicians who themselves suffered from these attacks
reported that the incidents occurred during periods of personal stress or when they
were particularly tired.
The attacks may develop at any time during life, even in childhood. Women are slightly
more likely to experience these events than men. Sometimes two or more attacks may
occur over a period of days or weeks, followed by total remission. Some individuals
experience only one such attack during a lifetime. A family history of similar attacks
has been reported by a few individuals, and some report a history of migraine or
epilepsy. After the attack, patients often feel a sense of terror, rapid heartbeat,
and anxiety.
No underlying cause has been identified to explain the condition, which was first
reported in 1988. Some authorities have suggested that it may have something to
do with a sudden movement of a middle ear component or a "springing open" of the
eustachian tube. Others have suggested that it may be related to a minor seizure
in the temporal lobe that contains the nerve cells for hearing.
All agree, however, that exploding head syndrome is an entirely benign condition,
is not caused by psychological disturbances, but is very real and frightening.
Back to Top | Back to FAQs
Hypnic headaches
Hypnic headache, or headache during sleep, is a rare primary headache disorder occurring
only in the senior population, with onset typically around the age of 60. Women
are more likely to experience hypnic headache than men. To be called "hypnic headache,"
the headache must occur exclusively at night or during the day during napping. The
attacks are very painful, often awakening the afflicted individual at the same time
each night. The patient may be awakened during a dream by diffuse pain and nausea.
Hypnic headache is throbbing in quality and occurs 2-4 hours into nighttime sleep,
although attacks after daytime napping are reported. The duration is usually short-lived,
lasting from 5 to 60 minutes. The throbbing and pain occurs on both sides of the
head.
Hypnic headaches typically respond to lithium carbonate at a dose of 300 mg to 600
mg, although caffeine and indomethacin are also helpful. Because hypnic headaches
affect only individuals over the age of 50, it has been suggested that there are
disturbances to the "biological clock" related to serotonin levels in the brain.
The absence of autonomic features that are often seen in migraine or cluster headache
distinguishes this illness from these conditions.
Back to Top | Back to FAQs
Meniere's
disease
Dizziness is a
common feeling, especially among headache sufferers. Some will experience true vertigo—the
sensation that the room is spinning, or that they themselves are spinning. Although
this very disturbing sensation can be caused by a number of illnesses, one possible
diagnosis is Meniere’s disease.
Meniere’s disease
typically begins with vertigo that builds over minutes, lasts hours, but can end
abruptly. Attacks may occur in clusters and then disappear for months or years,
recurring suddenly without provocation. Motion may make it worse; no position makes
it go away. Vertigo may be associated with ringing in the ear (tinnitus), ear pain,
and/or hearing loss, usually in one ear. Meniere’s may last 2 to 3 years, but can
recur years later. Common associated symptoms include compromised or permanent hearing
loss and imbalance.
Episodes of Meniere’s
are caused by distortion and eventual damage to the balance and hearing center in
the inner ear. Each bout of Meniere’s corresponds to an episode of damage. Repeated
damage causes the brain’s balance center to become impaired. As the brain “resets”
to the new set of signals, the vertigo ceases.
Treatment usually
focuses on two initial strategies. The first step is relief of the acute symptoms
of vertigo and nausea with such medications as meclizine (Antivert®) and metoclopramide
(Reglan®). The second is to focus on decreasing excess
endolymph pressure—the fluid in the inner ear. A low salt diet, avoidance of caffeine,
and a diuretic such as acetazolamide
(Diamox®) are often recommended.
Surgical and pharmacological
treatments may help, including in severe cases destruction of the inner ear itself.
This stabilizes the input to the brain’s balance centers and eliminates the attacks,
but often at the cost of further hearing loss. Therefore, such steps must be considered
only after more conservative options have failed to provide relief of symptoms.
Meniere’s disease
is not the only cause of vertigo. Therefore, a comprehensive history and physical
by an experienced physician is needed to differentiate Meniere’s disease from other
look-alike syndromes. An accurate diagnosis is the key to establishing the proper
treatment for this aggravating but treatable disease.
Back to Top | Back to FAQs
Post-herpetic neuralgia
Herpes zoster (shingles) affects up to a million people per year, and 200,000 Americans
are affected by the painful aftermath (post-herpetic neuralgia) which is more likely
to strike those over the age of 60. The treatment of post-herpetic neuralgia, which
results from the herpes virus invading and destroying nerves and even spinal cord
tissue, is often complex. Treatments include medications that reduce the transmission
of pain impulses in the nerves as well as reducing the area of pain in skin and
nerve receptors.
Numerous medications are available and include analgesics, antidepressants (not
for depression but to treat the pain itself), antiseizure medicine (not for seizures
but to treat nerve pain), capsaicin, and most recently the drug Neurontin, which
at high dosages has shown itself to be very effective in treating the symptoms of
post-herpetic neuralgia. Sometimes nerve blocks are required and can be dramatically
helpful as well; electrical stimulation of the skin is another option.
A variety of skin patches are available, including those that contain lidocaine,
such as Lidoderm. Lidoderm is the first product ever approved for the relief of
pain specifically associated with post-herpetic neuralgia, and is thought to stabilize
nerve membranes and thus produce an analgesic effect.
The patch is applied to intact skin covering the most painful areas and causes a
reduction in pain under and surrounding the patch. It is not generally recommended
to be applied to areas of broken or seriously inflamed skin.
The advantages of the patch are that it is easy to apply and the medicine is generally
not absorbed into the system (although overdosing or using it for more than 12 hours
out of 24 can be toxic). The patch may also reduce irritation from clothing or inadvertent
stimulation. Modest to moderate relief can be expected. The drug is marketed by
Endo Laboratories.
Back to Top | Back to FAQs
Referred pain
Why does one part of the body hurt when the origin of pain is somewhere else?
The best known example of this phenomenon—called "referred pain"—is the development
of left arm or jaw pain as a result of coronary insufficiency (lack of blood flow
through the heart vessels), or angina. The physiological explanation is that the
brain cannot discern the true origin of the pain because of overlapping and intermingling
nerve pathways that can converge in the spinal cord or in other locations. Here
are some other important examples of referred pains:
Migraine, which originates in the brain, is often initially perceived
as neck pain or a stiff neck because of descending neural circuits that go deep
into the neck region.
Abdominal aortic aneurysm (ballooning of the aorta) is often perceived
as back pain.
Esophagus pain can be felt in the back between the shoulder blades
or even in the ear region.
Testicular pain is frequently perceived as abdominal pain because
the testicles' nerve supply originates in the abdomen. The testes are located in
the abdomen of the fetus and then descend into the groin.
Gas pain from intestines around the spleen in the left upper abdominal
area is often referred to the left shoulder, neck, or chest.
Upper neck disturbances may be first perceived as pain in the forehead
or within the eye.
Hip disease may be felt in the knee area.
Nasal or sinus pain may be perceived as a headache on the top of the
head.
Chest disease may be felt as pain in the ear or face area.
The effective evaluation and treatment of most painful disorders requires time and
diligence on the part of both the patient and the health professional.
Back to Top | Back to FAQs
Tinnitus
Tinnitus, defined as ringing or buzzing in the ears, is a frequent complaint. It
may be seen in patients with headache, or may occur unrelated to pain.
Tinnitus is often associated with hyperacusis, which is an intolerance
to moderate to loud sounds. Sounds are heard with exaggerated volume. Recent estimates
suggest that 40 to 50 million Americans suffer from some degree of tinnitus or hyperacusis.
Either may be associated with at least mild hearing loss and can become severe enough
to be debilitating.
No specific cause of tinnitus has been identified. Most likely the symptoms of tinnitus
reflect disturbance in both the auditory and nonauditory structures within the central
nervous system. Other causes may include a disturbance in the limbic nervous system
as well as a sensitivity of the brain to serotonin fluctuation. Emotional symptoms
such as anxiety and depression may also aggravate the condition.
A multidisciplinary approach to treatment is the most effective method for managing
tinnitus and hyperacusis, including an evaluation by an audiologist. The audiologist
can objectively measure the degree of hearing loss and recommend hearing aids and
proper audiologic treatment.
There is no standardized treatment for tinnitus at this point. Many classes of medication—e.g.,
antiseizure or antidepressant—have been tried with varying success. Medical management
may also include reducing any medications or treatments that may aggravate the condition,
such as aspirin or nonsteroidal anti-inflammatory agents.
Most tinnitus sufferers find that constant background noise may help symptoms that
tend to worsen in a silent or more quiet setting. External noise, such as white
noise generators, indoor waterfalls, fans, heaters, or fish tanks, may help reduce
the irritating aspect of symptoms. Special masking devices can also be inserted
at tinnitus specialty clinics.
If tinnitus is associated with vertigo or dizziness, comes on abruptly, or is accompanied
by other symptoms, prompt evaluation is needed. Certain serious clinical conditions
can cause these symptoms.
Back to Top | Back to FAQs
Tongue cramping
Many people experience simple tremors in their tongues. Often the cause is not known.
Occasionally it can occur from conditions which affect nerve transmission to the
tongue and may or may not involve other motor nerves. A condition referred to as
oral mandibular dystonia, or OMD, may also cause these symptoms. This is a localized
disturbance of muscular movement causing involuntary contractions which are often
repetitive and may involve the mouth, jaw, and/or tongue. It may occur either from
disturbances in the brain or from injury to certain nerves.
Sometimes the condition can arise from taking certain medications, such as tranquilizers
or other drugs. Generally, the condition is without serious implications, but sometimes
a progressive disorder is present.
After the diagnosis is made, treatment ranges from medications to correcting metabolic
disturbances, if present, to injecting various medications to reduce the spasms
or cramping. This might include botulinum toxin.
Back to Top |
Back to FAQs
|