Diseases information. Disorders. Treatment.

Disorders information
There are dermatologic lesions that assist in diagnosis for you clinically. A very prominent one is adenoma sebaceum, which are angiokeratomas. They can have hypopigmented macules, ash-leaf spots, shagreen patches. We saw this picture earlier, with the ash-leaf spot and several hypopigmented macules and there’s the shagreen patch. These can occur quite early in life. Later in life you may see angiofibromas or the adenoma sebaceum develop. Often you need to refer your patients to plastic surgery or dermatology for management of those. They can sometimes bleed and get messy. Periungual fibromas; all TS patients should have ultrasound of everything and ophthalmologic consult. Remember, they can also have retinal hamartomas, cardiac rhabdomyomas. Sometimes we know somebody has TS because they are having a problem in the neonatal nursery. They do a cardiac echo and find a huge intraventricular tumor. That occurs way before seizures. Interestingly, the cardiac tumors tend to regress but subsequently they can develop renal cysts and angiomyolipomas. That’s why it makes sense when I told you that this mutation seems to be in a tumor suppresser gene, they can also have problems in the liver, spleen, lungs. So usually they need extensive workup, if you saw a baby like this. Here is a picture of a retinal hamartoma. As you can see, here’s the disc with the optic cup and the vasculature, but this doesn’t belong. There is a dental enamel pit in an older child.
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Neurologic complications are: seizures, such as infantile spasms, mental retardation, autism develops very commonly. Twenty-five to thirty percent of patients with TS seem to evolve into the autistic spectrum disorders. Rarer complication could be hydrocephalus from one of the tumors obstructing the foramen of Monroe. They can rarely turn malignant. So it’s a very complex disease.

Cranial synostosis. So here we first want to show you what the major sutures are. As you can see, here is the metopic suture, there is the coronal one going across, sagittal and the lambdoid, and there’s the anterior fontanelle. So growth occurs at right angles to any suture. So any time you have synostosis where the suture fuses prematurely, you cannot grow orthogonal to it. You will grow tangential. I’ll show you, for instance, how brachycephaly develops. Here’s someone where there is coronal synostosis. Order provigil online online at health care pharmacy. See, the yellow line is thick. So the arrows show the growth that is orthogonal to this line, that is 90 degrees perpendicular, is limited and compensatory growth occurs tangential to the suture. What happens then is the shape of the head assumes that. Is that clear now? So what will happen if you have difficulty with a sagittal suture? You won’t be able to grow this way, you’ll grow that way. You’ll get a long boat-head. So it turns out that’s why we call it scaphocephaly. Boat-head. This is commonly encountered cranial synostosis, 40-50% of all cranial synostosis are sagittal. Coronal is brachycephaly, meaning short-head. Lambdoid will cause flat back, like plagiocephaly. When all the sutures are affected you get oxycephaly. I don’t have a good picture of it. What oxycephaly looks like, it looks like a tower-head. See, you can’t grow this way, you can’t grow this way, so you grow straight up. It’s not a very good-looking sight. You can get cranial synostoses in many congenital syndromes, like Cruzon’s syndrome, Apert’s syndrome, Carpenter syndrome, etc. A lot of these kids, again, you probably don’t see them in your continuity clinic in your program, however if you go to the craniofacial clinic, usually run by head and neck surgery and neurosurgery combined, you will see an amazing number of these things. Here are some examples of brachycephaly, scaphocephaly with elongation.

Vascular malformations. Vein of Galen’s you should know. The important associations you make with vein of Galen’s is, first of all I told you it is not an aneurysm. It’s an AVM. You may get this question, not for neurology. You may get it for Neonatology. It’s very very big in the differential for high output heart failure in the newborn. Auscultation of a cephalic bruit accompanies that. A lot of times that how people know somebody has this. You should know a word or two about berry aneurysms. Usually an adolescent gets into trouble, maybe 17-18-year-old, has the worst headache of their life, with a subarachnoid hemorrhage and severe neurologic morbidity. Then they realize after they do the angiography that they have a berry aneurysm. And these usually occur along the circle of Willis at the base of the head, and there is an association of berry aneurysms with aortic coarctation and polycystic renal disease. You just have to try to remember that type of strange associations.

Tuberous sclerosis occurs in about 1:30,000 births, dominant inheritance but many are spontaneous mutations. So if you see a baby with TS you should examine the skin of the patients family and inquire about seizure history in parents. It is certainly a dominant one but a large number of spontaneous mutations. There are two chromosomes that have been identified. One is tuberous sclerosis complex number 1 on chromosome 9 with the first linkage TSE-2, tuberous sclerosis complex 2 on chromosome 16. Interestingly this gene was cloned first, called tuberin and more recently we know this is due to hamartin. So patients may have a mutation in either one of them and they seem to be tumor suppresser genes so when you have a mutation strange things grow all over in your body. Even though TS is very important in neurology it’s really a multi-system disease.
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So the cerebral lesions are what I am going to talk about first. They are cortical tubers, or hamartomas and this is where epilepsy comes from. Epilepsy comes from the cortex, and it’s the tubers that cause that problem. There are subependymal glial nodules that I don’t think are that big a problem, except maybe they will appear on the Board with the radiologic questions. Because they make a big issue about these things sticking into the ventricle, giving it a verrucous irregular appearance. You all remember what I am talking about? Okay, so radiologic identification of those and then they can have giant cell astrocytomas in some of these patients. These are malignant. So here’s what I’m talking about in terms of radiologic. This is just a CT scan. You can see down here, this is the parenchyma of the brain. Here’s the ventricular border. It’s a little fuzzy here but you can see that there are these little intense things sticking into the ventricle. You may see that on the Board. That’s unquestionably tuberous sclerosis. If you saw them on an MRI you won’t see them that well because calcified things don’t have a signal on the MRI but in the MRI you may see hamartomas, such as these. Hamartomas are best seen in T-2 weighted images.

Medical articles at canadian pharmacy blog. Microcephaly can have a variety of etiologies, depending on when you discover it. What I mean by that is some people are born microcephalic because they may have a chromosomal syndrome, or Cornelia de Lange or fetal alcohol, or one of the STORCH, meaning syphilis, toxo, rubella, and all that type of syndromes. On the other hand, it can become an acquired process if you have perinatal asphyxia. So someone is born with the proper head circumference but let’s say they have severe meconium aspiration, get very sick, the neonatal course is very stormy, and then you find that at nine-months-of-age they have not gained 10 centimeters because they acquired the damage at that time. So that’s a different situation.
Macrocephaly is kids with big heads. Here we have again a big differential. It can be metabolic disease, like Sturge, Tay-Sachs, Hurler’s, some of those. Leukodystrophies like Alexander and Canavan are known to create progressively large head. You can see it sometimes in neurocutaneous syndromes, or bone disease. There is a syndrome called Sotos syndrome which is truly macrencephaly. There is no hydrocephalus. The brain looks normal although it’s rather large. Kids present with a big head, mildly hypotonic, and mental acuity varies and a lot of them tend to be kind of dull. They are not only cerebrally large, they are just big kids. They are kind of macrosomic. You will notice that when I talked about macrocephaly I didn’t really say anything about hydrocephalus, because that will come under CSF circulation and that’s not really big encephalus, but hydrocephalus.
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Obstructive hydrocephalus can be due to a congenital problem, like aqueductal stenosis. We are referring to the aqueduct of Sylvius that connects the third ventricle to the fourth. It can also be acquired due to midline brain tumors that compress the aqueduct. It may be a congenital anomaly, such as Dandy-Walker syndrome which has atresia of the foramina of Magendie and Luschka with compensatory dilatation of the fourth ventricle and cerebellar hypoplasia. Later when we talk about ataxia I’m going to show you a picture of Dandy-Walker. Another reason kids sometimes present with obstructive hydrocephalus is they have a vein of Galen’s aneurysm. It’s often called an aneurysm but that’s a misnomer. It’s not an aneurysm, it’s really an AVM. The difference being, an aneurysm is an abnormal swelling due to weakness of the wall of an artery. AVM is really anomalous arteriovenous channels. It’s a high conductance, low resistance channel. There are inferior fossa hematoma, like after trauma you could develop obstructive hydrocephalus. The key features of obstructive hydrocephalus, you will notice, that we are focusing on obstruction in the vicinity either due to a mass lesion or due to a congenital lesion, in such a manner that the flow from third to fourth ventricle is affected. That’s the key. What we call communicating hydrocephalus, where there still may be a problem with obstruction but it is not proximal to the fourth ventricle. This could happen partly because you have a problem with arachnoid granulations on the convexities. This could happen because the child had meningitis as a neonate and there was a lot of pus. Because the meninges got fibrosed and the absorptive surfaces are damaged. It could be post CMV or toxo, maybe a sequelae of large subarachnoid hemorrhages again. Rarely you can get communicating hydrocephalus due to excessive production of CSF, such as a choroid plexus papilloma. These tend to occur quite frequently between the second and third ventricles, near the foramen of Monroe. Then there can be obstruction downstream from the fourth ventricle. We already saw the anatomy of the Chiari and I did indicate to you then that sometimes they will gradually develop headaches and hydrocephalus, so that could be another etiology.