“A Potpourri of Puzzling Neurologic Phenomena from Infants to Grandparents”
Accuracy in diagnosis leads to better treatment and happier patients. Using
monikers to label someone “epileptic” or “a hyperventilator” can sometimes add
grief to an already difficult situation. Always
carefully individualize evaluations to respect the unique character of every
dizzy.” There are four major
categories of dizziness (syncope, vertigo, ataxia, cognitive). It
should be noted that there can be significant overlap with these different types
or diminished cerebral blood flow.
orthostatic hypotension (low blood pressure while standing up).
orthostatic hypotension with iron deficiency, anemia, etc., playing a role.
phenomenon (common fainting), which can occur with striking “seizure-like
convulsions.” (5% of fainters in a blood donation center have outright tonic-clonic
activity as an accompaniment.)
spells (tantrums), which may or may not be associated with mitigating pulmonary
or cardiac disease.
causes, particularly reactive airways disease (asthma) without significant or
obvious wheezing (do not forget pulmonary embolism).
disease of a variety of types includingcongenital
valve disease, vascular anomalies, and even intrinsic myocardial conduction
or labyrinthine-like dizziness (a sensation of spinning or swaying, sometimes
or inner ear infection, inflammation, or even “just” otitic pressure (extremely
common in children and adults).
cochlear and labyrinthine dysfunction (such as after a motor vehicle accident).
or toxic vertigo such as a side effect of antibiotics, etc.
(ear “rocks”), especially in older patients.
causes, including cerebellopontine angle tumors (acoustic neuroma) and brainstem
*This is a presentation Dr. Adams has given to a
number of audiences of doctors, nurses, and other health care professionals for
leads us in to the third category of actual imbalanceorataxia
(difficulty walking and maneuvering with tendency towards falling). This
type of dizziness is associated with a wide-based stance, stumbling, plus
obvious impairment of leg and foot function.
(ischemic or hemorrhagic).
trauma with or without intracranial bleeding.
malformations (cavernous angiomas and aneurysms).
cord tumors or masses causing myelopathy (spinal cord dysfunction), spinal
stenosis, or herniated disks in the neck can cause ataxia.
neuropathy as from diabetes, toxicity from drugs, vitamin deficiency, and
vaguely described subjective or “cognitive” dizziness.
with panic attacks in the setting of anxiety is always the first to come to
mind; however, all the other causes of dizziness should be ruled out first. Patients
with marked anxiety and panic attacks often are afflicted by other types of
dizziness as well. The “hidden”
problems may be generating a secondary anxiety disorder.
encephalopathy (confusional state).
psychiatric disease. Children with
ADHD (attention deficit hyperactivity disorder) may feel their “mind is full” or
overloaded. A racing or ruminative
mind in obsessive-compulsive disorder and bipolar illness can put a child’s
brain in such a “buzz” that internal feelings and behavior can be very erratic
and suddenly unpredictable.
or postictal state withseizures(epilepsy),
which leads us in to the next part of our presentation.
seizure disorder is a paroxysmal involuntary electrical disturbance of brain
function. It may manifest as:
impairment or loss of consciousness.
is defined as recurrent seizures unrelated to
designation “seizure disorder” is sometimes more tactful, kind, and more easilyaccepted
than the label of “epilepsy.” I try
to reassure patients that seizure disorders or epilepsies are a very treatable
entity that can be effectively dealt with just as with diabetes or asthma. A
misdiagnosis of epilepsy versus complicated fainting can drastically harm and
change an individual’s future.
that a normal lifestyle is possible should be balanced with caution of long-term
or future issues. (To obtain or
license one has to be seizure free for three months before the application. Regulations
and restrictions vary considerably from state to state.)
from seizure activity or epilepsy is extremely rare. It
most often occurs in individuals more handicapped by congenital brain
abnormalities or acquired structural disease causing a more resistant electrical
cerebral irritability in an already compromised brain.
common sequelaecan include
risks of head and dental injuries, aspiration pneumonia, stress fractures, and
especially psychosocial hindrance in school and at work.
when taking a bath, swimming, operating dangerous machinery, or moving about in
precarious or high places is in order if seizures are not yet controlled.
is common not only due to inherent predisposition, but also to effects of medications. Interictal
or postictal state can be difficult to recognize and distinguish from depression. Head
trauma patients with a frontal lobe syndrome can show an odd affect of emotional
of seizures include:
trauma (history of a motor vehicle accident or of an assault).
(ischemic or hemorrhagic infarction).
derangements, particularly hypoglycemia and hyponatremia.
effects of drugs and chemicals, particularly psychotropics, antidepressants,
stimulants, and even some antibiotics. Aspartame
or Nutrasweet can in some circumstances bring out seizures and migraines.
inflammatory processes including meningitis and encephalitis from bacterial or
viral causes, etc. (Focal infections
including chronic otitis media can sometimes cause focal inflammatory injury of
the brain resulting in a seizure disorder even without history of meningitis.)
or tumors of primary or metastatic character.
hereditary epilepsy and tendencies".
metabolic disturbances including inherited and mutated variety (tuberous
sclerosis and neurofibromatosis).
(no identifiable cause).
stratification of epilepsies include:
epilepsy as due to a brain lesion; for example, traumatic contusion, stroke or
or accessory epilepsy due to extracerebral disturbances as from toxic drug
effect, hyponatremia, or fever. With
regards to the latter, benign febrile seizures tend to be “benign,” except when
they are not. In particular, a
febrile seizure of focal nature carries a particularly high risk for subsequent
focal (partial) and partial complex seizures.
or idiopathic epilepsy.
of epileptic spells includes:
partial or focal seizures, which begin in one discrete area on one side of the
brain. There is minimal or limited impairment of consciousness.
or contraversive or “head turning” episodes.
mental phenomenon including déjà vu, jamais vu, and “dreamy states.”
forced emotional states such as unexplained instant combativeness.
phenomenon such as epigastric distress or chest heaviness with emotional
feelings that can range from mild anxiety to intense terror.
phenomenon even organisms can occur with seizures.
seizures secondarily generalized. Phenomena
can include Jacksonian spread and Todd’s or postictal paralysis. (With
the latter, a person may be “paralyzed” on one side for up to several minutes or
even several days after a seizure.)
seizures occur with bilateral brain electrical irritability and instability
without obvious focal onset.
absence seizures only with impairment only of consciousness.
absence seizures include other phenomenon on top of the alteration of
consciousness, such as:
jerks or myoclonus.
in postural tone.
types with loss of postoral tone.
as with lip smacking and blinking.
phenomenon including episodes associated with enuresis. (All
bedwetting is not due to delayed physical and social maturity. Nocturnal
seizures may present as bedwetting.)
epilepsy. (Not all myoclonic jerks
are epileptic.) Benign nocturnal
myoclonic jerks are, of course, common. Nonspecific
myoclonic jerks can occur due to spinal cord disease and other structural
injuries to the CNS.)
West syndrome is a triad of infantile spasms, mental deterioration, and a
characteristic EEG picture of hypsarrhythmia. This
syndrome usually develops between 3 and 12 months of age, and the spasms have
usually stopped before age 5, often transitions to other types of seizures. The
presentation is significant in that infantile spasms or “salaam attacks” can be
mistaken for colic. The spasms often
occur in clusters in the early morning or upon awakening from a nap with the
baby doubling up as if in pain and often crying afterwards.
mal or tonic-clonic seizures.
vii. Atonic seizures, particularly episodes that cause “drop attacks.”
or loss of movement, although without loss of muscle tone.
the other hand, to simplify classification of seizures, one could grossly
consider two major types of seizure disorders:
seizures tend to cause unresponsiveness and bilateral or non-focal motor
phenomenon. There is no obvious
or partial complex seizures originating on one side of the brain and often
suggesting discrete structural or identifiable etiologic injury to that side of
is often difficult to assess impairment of consciousness in neonates, infants,
and very young children. Neonatology
is, of course, a very delicate super specialization. The
clinical presentation of seizures in infants can be very subtle, nonspecific,
workup of a seizure should include:
clarification of familial predisposition as well as prior injuries or cerebral
insults, and in particular, head trauma. (Genetic
disorders such as tuberous sclerosis and neurofibromatosis are common.)
profile with blood sugar and sodium level, magnesium, T4, TSH,serum
more complex metabolic screens and genetic analysis dependingupon
the suggestive circumstances. A lead
level may be indicated. A drug
screen can be important, particularly to catch elicit drugs as with stimulant
for infection including blood cultures, urine tests, etc., may also be needed.
scanning with contrast is the “gold standard” for identifying hemorrhage and
clarifying traumatic injuries and as a tool to screen for aneurysms and vascular
resonance imaging (MRI) is more sensitive for posterior fossa or brainstem and
cerebellar disease and for “subtle lesions” in the temporal areas.mini
resonance angiography (MRA) has in many cases replaced the more tedious andinjuriouscatheterization
that was formerly needed for identification of aneurysms and vascular
emission tomography (PET) is used in evaluating focal metabolic function in
different areas of the brain.
EEG or electroencephalogram, which helps in clarifying:
or absence of electrical irritability (a “normal” EEG does not absolutely rule
out a seizure disorder). Recurrent,
unexplained stereotyped events may merit an empiric trial on anticonvulsants. A
positive response to treatment, however, does not necessarily confirm a
diagnosis of epilepsy. In
particular, migraine phenomenon, panic attacks, and labile psychiatric changes
can also respond positively to some of the newer “broad spectrum”
anticonvulsants. Some of the newer
agents, for instance lamotrigine, can be very useful psychiatrically as a mood
frequency of electrical discharge and to help in assessment of the threshold
(tendency) for further seizures.
help to judge the therapeutic benefit to a medication; however, do not chase the
brain wave testjust to try
to normalize it. The seizure patient
should be treated clinically, hoping to improve but not necessarily normalize
to one side of the EEG to suggest focal structural disease.
definitive diagnosis in some cases from the EEG alone
mapping can be helpful in localizing a locus of structural disease that might
merit surgical intervention.
tests to clarify manifestations of systemic disease:
echocardiogram to identify congenital vascular abnormalities and valvular
disease in the heart, which could be a source of emboli.
spinal tap if meningitis is suspected.
or treatment of seizure disorders. I
think it would be mostusefuto
start from the most easy and common sense suggestions and progress to the most
drastic and heroic treatments:
forget to counsel with regards:
fasting or skipping meals.
regular sleeping hours, particularly not enough sleep but also considering
issues of disturbed circadian rhythms and excessive sleeping.
of alcohol, particularly alcoholic binges.
with regards to flickering stimuli as from video games.
not overestimate or “push” the issue of stress as a reason for the medical
problem. Never forget that all
medical problems cause significant stress and anxiety even as apart from
pre-existing psychiatric tendencies and conditions.
of modifiable derangements including
therapy (to be further discussed).
diet or liquid fat supplement (besides being distasteful, long-term vascular
disease is a concern).
resection of not only obvious neoplastic processes but also of resistant
irritable electrical scar foci.
or “class action” side effects are:
including ataxia and vertigo.
slowing, including problems with word finding. (This
is an important issue in children who are developing their thought patterns and
speed of communication.)
change. (Depression, new onset
aggressive tendencies, and even simple loss of enthusiasm. Suicidal
ideation is not common without other psychiatric factors involved.)
occurs within several days to up toweeks after
exposure but not always.
sure the drug is the culprit. Do not
be fooled by:
to other substances including foods, skin care products such as lotions and
and other infections.
f.Photosensitivity, although without “true” rash.
rash on the trunk probably promotes more suspicion for skin reaction to the
of an anticonvulsant rash or sensitivity is critical to not “burn bridges” with
a therapeutic approach that may be desperately needed in the future.
an antiepileptic drug (AED) is a suspected cause of a rash, always stop the drug
due to possible relentless progression to Stevens-Johnson syndrome or toxic
with the effects of oral contraceptive drugs.
of weight regulation (changes in appetite).
or alteration of seizures can occur. Take
the family seriously if they report increased seizures or other new phenomena
are associated with the institution ofthedrug.
dyscrasias including aplastic anemia as from felbamate (Felbatol).
failure or dysfunction as from felbamate (Felbatol) and valproic acid (Depakote
ER). It should be clarified that
phenytoin (Dilantin) very commonly causes a “reactive” elevation of liver
enzymes that can be “followed” and is not necessarily harmful or consequential. Valproic
acid can cause troublesome hyperammonemia even without obvious liver enzyme
hyperplasia as from phenytoin (Dilantin).
from topiramate (Topamax) and zonisamide (Zonegran).
glaucoma as from topiramate (Topamax).
from carbamazepine (Tegretol, Carbatrol) and to a lesser extent oxcarbazepine (Trileptal).
particularly neural tube defects in the setting of valproic acid or Depakote. This
adverse effect has serious future implicationsin
a young girl who may at some point in the future become a prospective mother.
diagnostic testing at 14-20 weeks with alpha-fetoprotein levels and structural
regards to birth defects in animals and in early clinical studies, the newer
anticonvulsants seemed to have a favorable profile and most are Class C. (Possible
choices include Keppra, Vimpat, Lamictal, Lyrica, Neurontin, etc.)
drugs (the order of this list does not suggest importance or efficacy):
effects (avoid if possible).
(Dilantin, Phenytek, andIVCerebyx)
effective in certain seizure types such as focal seizures and secondary
generalized tonic-clonic seizures, excluding absence or myoclonic epilepsy.
is not helpful and can even exacerbate absence or myoclonic epilepsy.
has the advantage of a “therapeutic window,” when tends to be very protective in
the range of 10-20. However, ataxia
and lethargy can occur over that level and is a common problem with that drug.
can often be given in a once-daily dosage as compared to most of the other
anticonvulsants needing a scheduled multidose regimen.
advise parents to vigorously shake Dilantin and other suspensions due to
is very effective for a variety of seizure types, particularly absence,
myoclonic, generalized tonic-clonic convulsions, and helpful but somewhat less
useful for strict partial or focal seizures.
very useful, it may not have quite lived up to its original “billing” as the
“all purpose anticonvulsant.”
extended-release formulation sometimes lessens side effects of limiting nausea.
gain is often a big issue.
toxicity with high ammonia or even hepatic failure can occur.
forget the issue of teratogenicitydysraphism
young girls or females that may some day become mothers).
in focal or partial complex seizures or in secondary generalized seizures, it
can exacerbate absence or myoclonic epilepsy.
the “gold standard” used for controlled comparison in studies of benefit of
lethargy, and disturbed gait can be quite striking when carbamazepine is
initiated. Slow titrations are
high” blood levels can be associated with _____________ ________________.
favorable behavioral effects such as decreased anxiety, improved mood, and
as a mood stabilizer for bipolar illness.
has been the standard pregnancy drug, althoughthe newer agents are
all primarily Class C.
abnormalities can include low white blood cell counts (usually of benign
character) and hyponatremia of uncertain significance, both of which need
monitoring with blood work.
FDA indications include:
disorders in patient greater than age 18.
treatment for partial seizures in pediatric patients greater than and equal to 2
years of age.
is optimism for significant help in other conditions including new-onset typical
and atypical absence seizures, generalized tonic-clonic seizures, atonic and
myoclonic seizures, and the difficult to treat Lennox-Gastaut syndrome.
can occur in up to 10% of patients as it does with other drugs, particularly
carbamazepine. The drug should
absolutely be stopped if a rash occurs and will probably not be an option in the
to “antigen-loading phenomenon” titration should be slowespecially
when added to valproic acid. Depakote
greatly increases the half life of lamotrigine.
combination of lamotrigine and valproic acid has been very effective in very
difficult, resistant epilepsies, even those who have sometimes failed ablative
in partial complex and mixed-seizures disorders as with secondary convulsions is
comparable to carbamazepine.
has a 95-100% renal excretion and no appreciable interactions with other drugs.
has an idiosyncrasy of a slower absorption with greater increasing doses due to
saturation kinetics. (Higher doses
sometimes do not seem to provide much more added benefit.)
as an antineuralgic agent in palliating intractable pain and in treating
patients can be susceptible to its sedative and depressant effects.
has a renal excretion and limited interactions with other drugs.
is effective in partial complex and mixed-type seizures with convulsions.
mental slowing, and ataxia can occur, particularly in the elderly.
is very helpful in treating neuralgic pain and intractable headaches in
conditions such as fibromyalgia.
opposed to the other anticonvulsants listed, it is a “scheduled” drug as some
individuals have used it illicit fashion to “get high.”
has a broad range of benefits in a variety of seizure disorders.
has renal excretion and limited interactions with other drugs.
can be helpful in very resistant seizure disorders and in patients who are on
is approved as being very effective for partial and mixed seizures in ages 4 and
over. Studies suggest tolerance and
possible safety even used in children down to less than one year of age.
with carbamazepine, Trileptal has been helpful in treating chronic and
can occur as with carbamazepine, but probably less often.
with careful titration also tends to show a positive trend in some patients as
compared to carbamazepine.
with Neurontin, a primary renal excretion limits interactions with other drugs.
less sleepiness or fatigueNeurontin (gabapentin)
or Lyrica (pregabalin).
systemic adverse effects.
worsening of mood and behavior can be seen even.(This most
commonly occurs in patients with handicaps such as cerebral palsy.)
tocause less cognitive
impairment, lethargy, and sleepiness than some other anticonvulsants.
dose can be a therapeutic dose (250-500 mg p.o. b.i.d. in an average adult).
help in control of partial seizures and generalized tonic-clonic events, benefit
in the Lennox-Gastaut syndrome and with infantile spasms has been shown.
drug has been extremely helpful in prophylaxis of very intractable migraine.events,lowersuch
acidosis with altered sodium bicarbonate levels can occur, although usually
without clinical symptoms.Hypohidrosismore
often in children.
side effects can include problems with “word finding,” which would be
distressing with a developing young mind.
tends to be “black or white.” If it induces dysphoria, it has to be discontinued.
or dysesthesias of the arms and legs can sometimes be a limiting side effect,
probably prompting discontinuation of the drug.
tends to curve the appetite, although will not necessarily cause weight loss.
is a sulfonamide with consideration of allergic sensitivity to sulfa.
with topiramate, cognitive slowing or dullness can occur.
tends to curve the appetite, although will not necessarily cause weight loss.
for partial seizures as well as treatment of migraine and neuropathic pain,
especially with spasticity.
be useful as a nighttime hypnotic for insomnia.
in the control of absence seizures. (In
fact, it is the “gold standard.”)
adjunct in the management of “breakthrough” seizures, particularly in the
setting of marked anxiety, nervousness, and sporadic myoclonic jerks.
to stop a cycle of “breakthrough” seizures, preventing a visit to the emergency
for partial seizures with or without generalization and Lennox-Gastaut
risk of potentially fatal aplastic anemia and liver failure requiring blood
for Lennox-Gastaut syndrome (adults and children over 4 years old).
in resistant partial complex and secondary generalized seizures and with
infantile spasms or West syndrome. Warning
can cause irreversible atrophy of the optic nerve with visual loss or
useful in focal, partial complex, and seizures secondarily generalizing from a
for generalized seizure types such as absence, tonic, tonic clonic, myoclonic,
(Zarontin, for simple absence seizures only).
opposed to the monitoring required with phenytoin, valproic acid, and
carbamazepine, the “newer” anticonvulsant agents are more empirically
need for tedious andobsessivedrug-level
risk of cognitive side effects:
(Lyrica), renal excretion.
(Vimpat), renal excretion.
promote weight loss.
(Lamictal) tends to be neutral with regards to weight gain.
regards to efficacy in partial and mixed seizure disorders with or without
is comparable to carbamazepine. The
latter has been the standard with which to compare the new anticonvulsants.
is comparable to phenytoin and carbamazepine.
is effective as carbamazepine but possibly less rash, hyponatremia, and adverse
CNS side effects, particularly with careful titration.
lacosamide, and zonisamide have been helpful in resistant cases.
10-year-old “princess” turned “devilish.”
Resolution: A truly angelic straight-A student was turned into an obnoxious
biting monster by “innocent” use of Dimetapp by her parents. A switch to a
nonsedating sinus medication allowed her to revert back to her normal
5-month-old girl with spells of “shuddering.”
Resolution: Multiple EEG studies in
an infant with clusters of shuddering spells over a several-week period failed
to reveal any answer. Eventual
treatment of urinary tract abnormalities leading to recurrent infection stopped
the vasovagal phenomenon (fainting) that was associated with that irritation.
10-year-old handicapped girl with a history of multiple shunt revisions for
hydrocephalus having spells of intermittent jerking with lethargy not responding
to triple anticonvulsant therapy.
Resolution: Her antiepileptic drugs were associated with marked sedation and
personality change but did not help in decreasing spells of sudden altered
consciousness with variable intermittent drop attacks. After
nine months of repeated CT scans and pleadings with the neurosurgeon, another
shunt revision was done showing not only blockage but also shunt infection. The
spells and drop attacks were eliminated with much improvement in her
personality. This allowed the
tapering and elimination of her anticonvulsants that were causing serious
sedative side effects.
14-year-old female basketball player with paroxysmal exertional dizziness
stopping her play.
Resolution: Seizure disorder, cardiac disease, and common teenage girl problems
of orthostatic hypotension and iron deficiency were ruled out. Treatment
of “silent” asthma (marked exertional-related reactive airways disease without
wheezing) was made with Singulair and beta-adrenergic inhalers. She was then
able to achieve her high-athletic potential without spells.
7-year-old with recurrent salivation and drooling in the setting of persistent
and chronic rhinitis and postnasal drip.
Resolution: Antihistamines and
decongestants in multiple trials failed to provide any benefit. Benign
childhood epilepsy or Rolandic epilepsy was present with the presentation of
postnasal drip and drooling as focal seizure activity. After
EEG identification, there was complete elimination of episodes with low-dose
9-year-old baseball player with sleep onset jerks and twitches.
Resolution: Nocturnal disturbances of jerks and twitches turned out to be focal
seizures caused by the glancing blow of a baseball as it slipped out of the
young outfielder’s hand, hitting his head one year prior. Gabapentin
(Neurontin) halted the nighttime problems without slowing the cognitive function
in this straight-A student. Levetiracetam
(Keppra) had to be added later due to marked seizure activity on his EEG.
9-year-old boy being considered for diagnosis of Tourette’s syndrome due to
tics, twitching, and multiple “restless” behaviors.
Resolution: Caution should be made
in jumping to treatment with antipsychotic tranquilizers for tics. The
following discoveries were made:
with facial twitching and grimacing was eliminated by prescription glasses for
treatment stopped the recurrent throat clearing that was a subject to ridicule
for this boy in class.
odd barking cough resolved with treatment of his reactive airways disease with
restlessness with limited ability to sit still was essentially eliminated by
treatment of his chronic constipation with his infrequent “once a week bowel
movements.” The end result of this was elimination of his distracting behavior
with no need for tranquilizers over a three-year followup.
moral of the story is that all tics are not Tourette’s.
and Pregnancy: Minimizing the
Risks. Martha J. Morrell, M.D.,Contemporary
Profile of Levetiracetam: Towards
Ideal Characteristics. P.N. Pat
Therapeutics, 85(2000) 77-85.
Treatment of Epilepsy: Principles
and Practice. Editor Elaine Willie, M.D. Text
published by Lea and Fbiger.
Agents: 2002. John
R. Gates, M.D. Minnesota Epilepsy
Group, P.A. Web site: www.mnepilepsy.org
Acid Supplementation to Prevent Neural Tube Defects.The
Medical Letter,Volume 46 (Issue
Safety of Oxcarbazepine in Very Young Children with Partial Epilepsy. Ralph
S. Northam, M.D.,Neurology
Reviews, June 2004.
Importance of Monotherapy in Pregnancy. Paige
B. Pennell, M.D. Neurology 60 (supplement 4), June 2003.
Effect Profiles and Behavioral Consequences of Antiepileptic Medications. John
R. Gates, M.D. Epilepsy and
Behavior 1, 153-159 (2000).
to Minimize Drug Induced Rash in Epilepsy Patients. Barry
al,Practical Neurology, Page
and Tolerability of the New Antiepileptic Drugs I: Treatment
of New Onset Epilepsy. J. A.
French, M.D., et al. Neurology 62,
Effect of Tiagabine on Spasticity in Children with Intractable Epilepsy: New
Pilot Study. Kenton R. Holden, M.D.,
et al. Pediatric Neurology,
Volume 21, Number 4, October 1999.
and Tolerability of the New Antiepileptic Drugs II: Treatment
of Refractory Epilepsy. J. A.
French, M.D., et al. Neurology 62,
Principles, Clinical Applications, and Related Fields. Ernst
Neidermeyer, et al. Published by
Urban and Schwartzenberg.
Robert Adams, M.D., is a board-certified neurologist who graduated fromKansasUniversityMedicalCenterand
who has been in clinical practice for over 20 years.