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New Treatments for Head Trauma and Cerebral Degenerations

Severe head trauma is uncommon in children less than two years of age. Trauma in less than one year of age accounts for about 86 per 1,000 children per year, and 50% of these injuries are to the skull and the head. Shaken baby syndrome is only one form of a specific child abuse and has a lot in common for pediatric neurologists because we see this all the time.

Paroxysmal non-epileptic spike-wave discharges that are mistaken sometimes for seizures. One is Sandifer’s syndrome with is a GE reflux syndrome occurring in children ages 4-14 months. They last from seconds to minutes. This can sometimes be mistaken for torticollis also because sometimes they just tilt their head one way or another. It does not occur at night.

Shuddering attack is something that happens fairly commonly. Usually seen in the first few years of life. They remit toward the end of the first decade. They occur during the daytime and last for a few seconds. What they tend to do is when they are angry or when they are aggravated, they’ll just shake. Obviously some parents would think they are having a tonic-clonic seizure probably,

Benign neonatal sleep myoclonus can occur within the first 15 days of life and usually if it occurs within the 15 days of life it can be either in the limb or a head jerk. As if falling asleep they just jerk their head. They disappear, usually within three to four months of age.

Benign paroxysmal vertigo, the onset is in the first five years of life. This is a common precursor of migraine. Consciousness is not impaired and the EEG is usually normal and you treat this with antihistaminics. What happens is they have a dramatic unprovoked and recurrent episodes of vertigo. What they will do is suddenly cling onto whoever is in front of them, or they will fall and lie down on the floor until the episode resolves and then start getting up.

Breath-holding spells are very variable. They usually remit by six years of age. They do not occur nocturnally. Usually precipitated by pain, trauma or vigorous crying. In some kids not even vigorous crying. There are two types: the pallid and the cyanotic one. The cyanotic one is more related to respiratory and the pallid is more related to cardiac. Treatment is really reassurance. The heart rate may fall down.

Sydenham’s chorea. It’s after a strep throat infection. The diagnosis is usually they have chorea, which typically presents as restlessness in school. It occurs in school-aged children, where all of a sudden the teacher tells you the kid is very restless, is not participating anymore. Sometimes misdiagnosed as having ADHD.

Lyme disease. The organism is Borrelia burgdorferi. Skin lesions are usually erythema chronica migrans. They can present with aseptic meningitis or encephalitis. They tend to have arthralgias. Diagnosis is by doing serum titers. If you are thinking of CSF Lyme, you’ve got to do CSF titers for Lyme disease as well. Treatment is ceftriaxone. You’ve got to ask about travel to endemic areas. If they give you a question about European Lyme disease.

Reye’s syndrome is normally associated with the use of aspirin in children less than 12 years of age and has also been seen after influenza virus infections. But can occur at any time also with other viral infections. Clinically they present with lethargy and signs of increased intracranial pressure and hepatomegaly. Laboratory diagnosis includes elevated ammonia and liver enzymes are elevated. Hypoglycemia. The definite diagnosis is obviously liver biopsy where you see fatty infiltrates of the liver. And EEG is really non-diagnostic but just shows diffuse slowing.

Some common causes of ataxia; acute ataxia or acute cerebellar ataxia are mainly seen in children following viral infections, especially varicellar infections. It’s seen in about 1% of those populations.  Intoxications, infections, trauma, metabolic disorders and hysteria.

A chronic non-progressive cerebellar hypoplasia, cerebral palsy, Dandy-Walker, Arnold-Chiari malformation. For the chronic progressive, the more common one is obviously is brain tumor or spinal cerebellar degeneration. Again this is more commonly a disorder of the adult as opposed to childhood, but can occur in children too.

Floppy infant. There are two ways of coming to this. One is obviously whether the child is sick or not sick. That’s your first thing that you need to differentiate. Because if the child is sick and floppy it may have nothing to do with any neurological disease but may have to do with a systemic disorder. If you have ruled out a systemic disorder, then your next thing is to say, "Are we dealing with a supratentorial neurological condition?