Dr. Greene’s Answer:

It’s worth checking with a neurologist about ongoing tingling like that. Migraines cause it in some kids, and sometimes it is the only symptom of the migraines. But it can also be caused by diabetes. Severe vitamin deficiencies could also do it, but a multivitamin should be enough to prevent that.

The tests to consider would be nerve conduction studies and a MRI. In adults, multiple sclerosis is now known to cause a glove and sock tingling (tingling of the hands and feet) that was once thought to be “in people’s heads” (not real). We now know that it is real.

MS is rare in children but it does occur. About 0.2 to 2 percent of all cases occur in kids. It is more common in girls than in boys, and it does run in families.

Dr. Greene`s Answer:

Seizure disorders do sometimes run in families. In general, epilepsy occurs in 0.5 to 1 percent of the population. In families with epilepsy, it can be much higher, but still each child will probably not have seizures.

Seizures can pass in different ways. Some are recessive and some are dominant while some are multifactorial. A neurologist or a geneticist could calculate the risk in a specific situation, but just because you have seizures doesn’t mean your child will.

Dr. Greene’s Answer:

Tics are common–more common than most people think. They are repeated, involuntary muscle movements and they often do increase during times of stress, including things like the strep or ear infections that you mentioned.

The time to consider medications to control the tic is if the tic is interfering too much with the child’s life, especially socially or academically. There are also other ways to treat tics than with medications. Biofeedback or Cognitive Behavioral Therapy, for example, really works for some kids. And of course, many kids with tics need no treatment at all and may very well outgrow the tics.

If medication is used, Clonidine is a good choice, but there are a number of good possibilities, such as penfluridol, topiramate, pimozide, and low-dose haloperidol. (American Family Physician, March 2008).

The main side effect to Clonidine involves drowsiness, and the decision to continue it (or adjust the dose) depends on whether, on balance, she seems better off. Sometimes a few weeks of therapy will break the cycle and tapering the dose may be a good choice. If you taper the Clonidine and the tics come back or increase again, you could gradually adjust the dose to the level that works the best.

Related concepts:

Gilles de la Tourette syndrome, tics, PANDA

Introduction to tourette syndrome:

The great English poet, author, and conversationalist, Dr. Samuel Johnson, is believed to have had Tourette syndrome, complete with both facial and vocal tics. His towering success in his day — before useful medicines had been developed – can be an inspiration and encouragement to today’s families living with Tourette.

What is tourette syndrome?

Tourette syndrome is named for Georges Gilles de la Tourette who first described the condition in 1885. Tics (involuntary, non-rhythmic movements) are the hallmark of the disease.
Brain scans and EEGs show differences in the brains of children with Tourette’s. These changes are influenced by genetics, neurobiology, and by what is going on in the child’s life.

Who gets tourette syndrome?

Gilles de la Tourette syndrome affects about one out of every 2,000 people. Tourette syndrome occurs worldwide, in all ethnic groups – though it is most common among whites. The condition usually runs in families. It is three to four times more common in boys than in girls, and usually begins before the age of seven.

What are the symptoms of tourette syndrome?

Tics are the classic symptom of this condition. In many children, these are so mild as to hardly be noticed. In others, the tics are severe and intrusive.
Motor tics are most people’s first symptoms. These may be simple, sudden, involuntary movements such as eye blinking, facial grimacing, lip licking, or fist clenching. They may also be more complex tasks such as chewing a shirt, hopping, or clapping. Whatever the movement, it is outside the child’s control – although this is often followed by a voluntary movement in an attempt to disguise the tic.
Vocal tics can also be a symptom of Tourette. They are also outside of a child’s control. Grunting, throat clearing, sniffling, hissing, clicking, whistling, and barking are simple vocal tics. Coprolalia, the involuntary speaking of obscenities, is a complex vocal tic, and the most famous symptom of Tourette syndrome. Echolalia (the compulsion to repeat words that are heard), and palilalia (the compulsion to repeat one’s own words) can also be Tourette’s symptoms.
Physical exertion, intense concentration, and sleep all suppress the symptoms of Tourette syndrome. I know a delightful man with Tourette who is very successful as a professional drummer (without medication).
Emotional stress, on the other hand, brings out the symptoms.
Children with Tourette also have a higher risk of other problems, such as obsessive-compulsive disorder.

Is tourette syndrome contagious?

Tourette can be triggered by some infectious diseases, such as Lyme disease or strep. PANDA (Pediatric Autoimmune Neuropsychiatric Disorder) is the name given when antibodies to streptococcal infections trigger the symptoms.

How long does tourette syndrome last?

For most, Tourette is a lifelong condition. Thankfully, most have a significant decrease in their symptoms over time.

How is tourette syndrome diagnosed?

Tourette is often suspected based on the history and physical examination. Sometimes brain scans, EEGs, or neurotransmitter levels are used to clarify the diagnosis.
If there is any possibility of recent strep or Lyme disease, tests for them should be run, because specific treatment may be necessary both now and in the future.

How is tourette syndrome treated?

A number of medicines have been very successful in controlling the symptoms of Tourette.
These each have some side effects, and may not be used if the Tourette isn’t interfering much in everyday life. However, when the Tourette is very troublesome the medicines can be wonderful.
If strep or Lyme disease are identified, they must be treated aggressively.
Other types of support may also be important for the child with Tourette.

How can tourette syndrome be prevented?

Usually, Tourette syndrome is difficult to prevent. Lyme disease is generally preventable. Measures to prevent Lyme disease or strep may be helpful to reduce the risk of Tourette syndrome.
In the last few years, researchers have discovered some clues about the genetics of Tourette syndrome. In 2005, researchers from Yale University identified a genetic mutation present in some patients with Tourette syndrome. Clearly, more research is needed to determine the genetics of this condition and the potential for gene therapy.

Related A-to-Z Information:

Attention Deficit Hyperactivity Disorder (ADHD), Breath Holding, Encephalitis, Epilepsy, Head Banging, Hiccups, Lyme Disease, Night Terrors, Rabies, Scarlet Fever, Strep Throat, Streptococcus (Strep), Ticks

Dr. Greene’s Answer:

Both Joseph (John) Merrick, the Elephant Man, and Quasimodo, the Hunchback of Notre Dame, are dramatic examples that have shaped our perceptions of neurofibromatosis. NF, also known as von Recklinghausen disease, is one of humankind’s most common neurological genetic disorders, indeed it is one of the most common genetic disorders of any kind.

“Treat me as an equal!” cried Merrick, whose appearance caused him to be ridiculed and rejected by 19th-century England. This courageous, intelligent, sensitive poet could find employment only as a sideshow freak.

Merrick’s mother was once watching a parade when, in the crush of spectators, she was pushed under an Elephant’s feet, “which frightened her very much,” wrote Merrick. “This occurring during a time of pregnancy was the cause of my deformity.”

Some doctors didn’t agree.

Merrick was brought before the Pathological Society of London by Sir Frederick Treves. A diagnosis of NF was finally made in 1909. So firm was this diagnosis that NF came to be called “Elephant Man’s disease.” The photograph of Merrick’s notorious disfigurement became the indelible image of NF in the minds of those familiar with it.

Not until the 1980’s did we learn that Merrick didn’t have NF but an unrelated condition called proteus syndrome (British Medical Journal, 1986; 293:683–685). I didn’t know this in 1982 when I read Merrick’s poignant three-page autobiography — after my surgeon told me he thought I might have NF.

Neurofibromatosis is a genetic disorder of the nervous system that causes benign tumors to form on the nerves anywhere in the body at any time. At least eight varieties of NF exist and can range from very mild to quite severe (Clinical Pediatric Dermatology, WB Saunders,1993), but fortunately none are as disfiguring as the tragic case of the Elephant Man.

Café-au-lait spots are a classic feature of this disease. These flat birthmarks with distinct edges are a bit darker than the surrounding skin. In light-skinned individuals, they are the color of coffee with lots of cream. In those with darker skin, they can be the color of a rich, full-bodied, black coffee. The spots can increase in size, number, and darkness throughout childhood.

Having one to three café-au-lait spots is quite common, present in as many as one in five healthy children. Each spot of significant size after the first three, though, is increasingly uncommon and increasingly likely to be associated with NF or with one of the other neurocutaneous syndromes, including neurofibromatosis type 2, segmental neurofibromatosis, tuberous sclerosis, McCune Albright syndrome, Fanconi anemia, Bloom syndrome and ataxia telangiectasia (CMAJ, Aug 2002; 167:3.).

Neurofibromatosis is diagnosed not with a test but by the presence of certain physical findings. People can receive a firm diagnosis of NF Type 1 if they have any two of the following symptoms (Textbook of Pediatrics, WB Saunders, 2000):

• At least six café-au-lait spots more than 5 mm in the greatest diameter (before puberty) or at least six café-au-lait spots of more than 15 mm (after puberty).
• Underarm or groin freckling (called axillary or inguinal freckling).
• Two or more Lisch nodules (small nodules in the iris of the eye).
• Two or more neurofibromas (rubbery lumps under the skin, often slightly purplish over the lump –these usually don’t show up before puberty) or one plexiform neurofriboma (thickened nerve trunks, often on the face – these are usually apparent at birth).
• A bone lesion consistent with NF, such as thinning of the long bones.
• Optic gliomas (small benign tumors of the optic nerve–most still have normal or near-normal vision).
• A close relative with NF (parent, sibling, or child).

Many of these findings do not show up in early childhood. Thus, any child with six or more café-au-lait spots that are more than 5 mm in diameter should be monitored and treated as having NF. This means a baseline examination by an ophthalmologist and audiologist (including vision and hearing tests) and a visit with a geneticist. It also means considering a baseline EEG and head CT or MRI, as well as a skeletal survey radiograph.

Each parent should have these same tests performed on him or her; siblings may be evaluated as well. NF is often so mild that a parent isn’t diagnosed until café-au-lait spots are found on his or her child. Often, a child’s course of disease follows that of his or her parents. Those with a presumptive diagnosis of NF need annual vision and hearing testing, as well as an annual neurologic and skeletal exam (in addition to a regular physical). Children with NF1 should have their growth followed on a special growth chart. They should be monitored for high blood pressure. Over the course of a lifetime, patients with NF1 need to be closely followed because of the higher risk of several rare types of cancers.

Very recent research shows that children with NF can have a higher frequency of social problems, attention problems, anxiety or depression, aggressive behavior, and a lower frequency of involvement in sports and other activities. They may have developmental delays. These problems are often mild enough to be missed unless looked for. Early screening and treatment for speech, motor, and cognitive problems and an increased effort to prevent and treat psychological problems can make a huge difference in the lives of these children (Journal of Pediatrics, 1999; 134:767–772).

The American Academy of Pediatrics recently published guidelines for doctors on how to better care for patients with NF1. The information was written for physicians, but can provide a useful information source for parents as well.

Although my condition turned out not to be NF, that period of uncertainty has left me feeling very close to my patients who have it. Joseph Merrick closed his autobiography with a portion of the poem that he passed out at his circus sideshow:

If I could reach from pole to pole
Or grasp the ocean with a span,
I would be measured by the soul;
The mind’s the standard of the man.
As it turns out, looks do matter.
Still

But progress in the treatment of disfiguring conditions is dramatic and accelerating every year. And progress in appreciating people for who they are is also being made, albeit at a slower pace.

Dr. Greene’s Answer:

Neurofibromatosis (a.k.a. von Recklinghausen’s disease) is a genetic disorder of the nervous system that causes benign tumors to form on the nerves anywhere in the body at any time. At least eight varieties of NF exist and can range from very mild to quite severe, but fortunately none are as disfiguring as the tragic case of the Elephant Man (who we now know did not have NF).

Café-au-lait spots are a classic feature of this disease. These flat birthmarks with distinct edges are a bit darker than the surrounding skin. In light-skinned individuals, they are the color of coffee with lots of cream. In those with darker skin, they can be the color of a rich, full-bodied, black coffee. The spots can increase in size, number, and darkness throughout childhood.

Having one to three café-au-lait spots is quite common, present in as many as one in five healthy children. Each spot of significant size after the first three, though, is increasingly uncommon and increasingly likely to be associated with NF or with one of the other neurocutaneous syndromes.

Any child with six or more café-au-lait spots that are more than 5 mm in diameter should be monitored and treated as having NF. This means a baseline examination by an ophthalmologist and audiologist (including vision and hearing tests). It also means a baseline EEG and head CT or MRI, as well as a skeletal survey radiograph and a visit with a geneticist.

Children with NF may also have other findings, including freckling in the armpit and groin areas, nodules seen on eye exam, and certain bone findings seen on xrays.

Each parent should have these same tests performed on him or her. NF is often so mild that a parent isn’t diagnosed until café-au-lait spots are found on his or her child. Often, a child’s course of disease follows that of his or her parents. Approximately 50% of cases are inherited; the other half are due to spontaneous mutations.

Those with a presumptive diagnosis of NF need annual vision and hearing testing, as well as an annual neurologic exam (in addition to a regular physical). They also benefit from preventive support of several kinds.

Dr. Greene`s Answer:

These could be seizures. Normally our brains coordinate the intricate affairs of our bodies with an orchestra of electrical impulses. Occasionally this harmonious energy is interrupted by the clash of many brain cells sounding off at once in a disorganized storm of electro-chemical noise. This burst of simultaneous, contradictory signals is called a seizure or convulsion. The seizure impulses override the normal functioning of the brain. Over 3 percent of children will have a seizure before the age of fifteen.

When most people think of seizures, they picture rhythmic jerking movements of the arms and legs. Seizures can look like this image, but can also have many different appearances depending on which brain cells are affected. Often the actual appearance of a seizure is so different from what people expect that the diagnosis of seizure is missed.

Seizures are classified as generalized or partial depending on how much of the brain is involved (International Classification of Epileptic Seizures, 1981). Seizure activity seizes control of normal brain function. Some partial seizures affect areas of the brain that direct movement; others stimulate areas of the brain that perceive reality or that regulate the body’s internal functions.

Partial Seizures

Uncontrolled muscle activity is the most common form of partial seizure. This can either be tonic (increased muscle tone) or clonic (rhythmic muscle contractions). Usually this involves the face, neck, or limbs. One classic appearance is the forceful turning of the head and eyes to one side. Sometimes complex movements such as lip-smacking, sucking, or swallowing can occur. These might be confused with the tics of Tourette’s syndrome. Sometimes partial seizures can appear somewhat intentional, as when a seizure makes someone pull at clothing or caress an object. A partial seizure in the part of the brain that directs the “flight” response might even send someone running aimlessly.

Sensory seizures are more difficult to detect. An explosion of activity in the auditory centers of the brain might cause someone to suddenly hear things that aren’t there. If the seizure is in the back of the head, all manner of visual disturbance might occur, from temporary blindness, to flashes of light, to complex visual hallucinations. Auditory or visual hallucinations might even be misdiagnosed as schizophrenia or some other mental illness.Olfactory seizures, in which people smell aromas that are not present, may be either pleasant or revolting. Gustatory seizures, with bursts of taste sensation, are rare.

Suddenly the world starts to spin out of control, and you are falling. Paroxysmal vertigo is another type of partial seizure. Terror!!! Seizures in primitive reaches of the brain can produce states of absolute fear. Or states of vague anxiety can be partial seizures. Hopelessness :^( Seizures in the limbic system of the brain can produce spasms of depression — or the sensation of total well-being.

Seizures can thrust vivid memories into consciousness, or distorted, mangled memories. They can create dream-like states, sudden nightmares, and states of unreality or detachment. Partial seizures can slow time to a syrupy pace, or make it seem to rush by in fast-action fury.

Sometimes partial sensory seizures are as subtle as periods of numbness, discomfort, or pain in parts of the body. Sometimes, rather than creating new sensations, they merely distort those that are already there.

Sometimes only the body’s internal regulatory parts of the brain are affected by a partial seizure — there might be a sudden increase in the amount of saliva, or in the speed of stool through the intestines.

Many of the symptoms that can be produced by partial seizures are common. Only when they are triggered outside the normal pathways of brain function are they seizures. The potential for each of them is, after all, present in each of our brains.

Generalized Seizures

Generalized seizures are disruptions of both sides of the brain at once, and as a result, usually include an impairment or loss of consciousness. The classic tonic-clonic or grand mal seizure would be difficult not to notice. The individual suddenly loses consciousness, sometimes emitting a piercing cry or haunting moan. The eyes roll back in the head; muscles throughout the face, trunk, and limbs become rigid in a tonic contraction. The person falls, unprotected, to the ground. Often the tongue is bitten. More frighteningly, breathing stops and the person begins to turn blue. Sphincter control is lost; urine and sometimes stool are passed. This tonic phase usually lasts for tens of seconds. As the clonic phase begins, the muscles relax and then contract again with rhythmic jerks. It is not uncommon for this phase to last for 30 minutes or more. When the generalized tonic-clonic seizure is finally over, the entire body relaxes, and the person is unconscious.

Absence or petit mal seizures, by contrast, are often nothing more than brief, motionless, blank staring into space. The head may fall forward slightly and the eyelids may flicker, but the effect is undramatic. After absence spells the people immediately resume whatever they were doing or saying just before the seizure interrupted them. They don’t even notice the pause.

The story of your nephew’s 2-year-old reminds me of one of the other types of generalized seizures — atonic seizures. Here the affected person usually has a sudden increase and then loss of muscle tone, leading to a complete or partial collapse. These are often called “drop attacks.” The unprotected falls can easily result in injury in the head or body.

Although you suggested that falls begin her episodes, it is possible that seizures begin each event. People might not notice the seizures until she topples.

What Causes Seizures

The most common cause of seizures in childhood is the rapid onset of a high fever. Febrile seizures, while very frightening to parents, are not dangerous.

Head trauma is another common cause of seizures. Head trauma may have been the cause of seizures in this particular situation. On the other hand, head trauma may also have resulted from the seizures, created a collection of blood in the skull, and produced the limp.

Infections of various types (especially meningitis) are the other common known cause of seizures. Other less common causes include drugs, toxins, brain tumors, strokes, neurologic syndromes, altered blood sugar levels (too low or too high), or abnormalities of blood levels of sodium, calcium, or magnesium.

Epilepsy is defined as recurrent seizures unrelated to fever or identifiable acute damage to the brain. Somewhere between 0.4 and 1 percent of people have epilepsy, making it about as common as diabetes (Pediatric Epilepsy, Demos Publishers, 1993).

How Do You Tell If It Is Seizures?

Often the story is compelling enough to clinch the diagnosis of seizure. When this is not the case, the hallmark test is the electroencephalogram or EEG. This painless, non-invasive test simply records brain waves to look for abnormalities. A positive test can make the diagnosis. A negative test though, unless it happens during the event, can’t completely rule out a seizure. Sometimes the situation that triggers the event is reproduced to try to capture it on video and/or EEG.

From what I’ve heard it sounds to me that this girl’s pediatrician might need to consider an EEG, as well as either a head CT or an MRI to look inside this little girl’s brain. You are wise to suspect that the cause of these falls is not in the bones. And, whatever turns out to be going on, an unexplained limp in a child should be investigated thoroughly.

Dr. Greene`s Answer:

A burst of simultaneous, contradictory signals from brain cells is called a seizure or convulsion. The most common cause of seizures in childhood is the rapid onset of a high fever. Febrile seizures, while very frightening to parents, are not dangerous.

Head trauma is another common cause of seizures. Infections of various types (especially meningitis) are the other common known cause of seizures. Other less common causes include drugs, toxins, brain tumors, strokes, neurologic syndromes, altered blood sugar levels (too low or too high), or abnormalities of blood levels of sodium, calcium, or magnesium.

Epilepsy is defined as recurrent seizures unrelated to fever or identifiable acute damage to the brain. Somewhere between 0.4 and 1 percent of people have epilepsy, making it about as common as diabetes (Pediatric Epilepsy, Demos Publishers, 1993).

Dr. Greene’s Answer:

Uncontrolled muscle activity is the most common form of partial seizure. This can either be tonic (increased muscle tone) or clonic (rhythmic muscle contractions). Usually this involves the face, neck, or limbs. One classic appearance is the forceful turning of the head and eyes to one side. Sometimes complex movements such as lip-smacking, sucking, or swallowing can occur. These might be confused with the tics of Tourette’s syndrome. Sometimes partial seizures can appear somewhat intentional, as when a seizure makes someone pull at clothing or caress an object. A partial seizure in the part of the brain that directs the “flight” response might even send someone running aimlessly.

Sensory seizures are more difficult to detect. An explosion of activity in the auditory centers of the brain might cause someone to suddenly hear things that aren’t there. If the seizure is in the back of the head, all manner of visual disturbance might occur, from temporary blindness, to flashes of light, to complex visual hallucinations. Auditory or visual hallucinations might even be misdiagnosed as schizophrenia or some other mental illness. Olfactory seizures, in which people smell aromas that are not present, may be either pleasant or revolting. Gustatory seizures, with bursts of taste sensation, are rare.

Suddenly the world starts to spin out of control, and you are falling. Paroxysmal vertigo is another type of partial seizure. Terror!!! Seizures in primitive reaches of the brain can produce states of absolute fear. Or states of vague anxiety can be partial seizures. Seizures in the limbic system of the brain can produce spasms of depression — or the sensation of total well-being.

Seizures can thrust vivid memories into consciousness, or distorted, mangled memories. They can create dream-like states, sudden nightmares, and states of unreality or detachment. Partial seizures can slow time to a syrupy pace, or make it seem to rush by in fast-action fury.

Sometimes partial sensory seizures are as subtle as periods of numbness, discomfort, or pain in parts of the body. Sometimes, rather than creating new sensations, they merely distort those that are already there.

Sometimes only the body’s internal regulatory parts of the brain are affected by a partial seizure — there might be a sudden increase in the amount of saliva, or in the speed of stool through the intestines.

Many of the symptoms that can be produced by partial seizures are common. Only when they are triggered outside the normal pathways of brain function are they seizures. The potential for each of them is, after all, present in each of our brains.

Dr. Greene’s Answer:

Generalized seizures are disruptions of both sides of the brain at once, and as a result, usually include an impairment or loss of consciousness. The classic tonic-clonic or grand mal seizure would be difficult not to notice. The individual suddenly loses consciousness, sometimes emitting a piercing cry or haunting moan. The eyes roll back in the head; muscles throughout the face, trunk, and limbs become rigid in a tonic contraction. The person falls, unprotected, to the ground. Often the tongue is bitten. More frighteningly, breathing stops and the person begins to turn blue. Sphincter control is lost; urine and sometimes stool are passed. This tonic phase usually lasts for tens of seconds. As the clonic phase begins, the muscles relax and then contract again with rhythmic jerks. It is not uncommon for this phase to last for 30 minutes or more. When the generalized tonic-clonic seizure is finally over, the entire body relaxes, and the person is unconscious.

Absence or petit mal seizures, by contrast, are often nothing more than brief, motionless, blank staring into space. The head may fall forward slightly and the eyelids may flicker, but the effect is undramatic. After absence spells the people immediately resume whatever they were doing or saying just before the seizure interrupted them. They don’t even notice the pause.

One of the other types of generalized seizures is called atonic seizures. Here, the affected person usually has a sudden increase and then loss of muscle tone, leading to a complete or partial collapse. These are often called “drop attacks.” The unprotected falls can easily result in injury in the head or body.