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A parent brought her child to a pediatric neurologist because the boy exhibited a number of serious neurological signs. These included lack of coordination, especially around the region of the trunk, vomiting, lack of ability to develop new motor skills, and headaches. An MRI further revealed an enlarged cranium, a large cyst which appeared to replace much
of the cerebellum and reduction in the size of the corpus callosum. Which of the following is the most likely diagnosis?

a. Pyramidal tract syndrome
b. Spina bifida
c. Anencephaly
d. Dandy-Walker syndrome
e. Meningomyelocele

:mad:
 

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D) Dandy-Walker syndrome

My ans is D) Dandy-Walker syndrome

Explanation is coming in a while.....

Dandy-Walker syndrome is a congenital brain malformation involving the cerebellum and the fluid filled spaces around it. A key feature of this syndrome is the partial or even complete absence of the part of the brain located between the two cerebellar hemispheres (cerebellar vermis).

The key features of this syndrome are an enlargement of the fourth ventricle; a partial or complete absence of the cerebellar vermis, the posterior midline area of cerebellar cortex responsible for coordination of the axial musculature; and cyst formation near the internal base of the skull. An increase in the size of the fluid spaces surrounding the brain as well as an increase in pressure may also be present.

This often involves hydrocephaly and complications due to associated genetic conditions, such as Spina Bifida (choice B)
 

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D.Dandy-walker:)
The Dandy-Walker malformation consists of a large posterior fossa cyst that is continuous with the fourth ventricle and defective development of the cerebellum, including partial or complete absence of the vermis. Hydrocephalus develops in 70 to 90 percent of patients with Dandy-Walker malformation, and is caused by atresia of the foramina of Luschka and Magendie. Dandy-Walker malformation is a heterogeneous disorder. Some patients have a syndromic form with associated congenital anomalies including dysgenesis of the corpus callosum, orofacial deformities, congenital abnormalities of the heart, genitourinary and gastrointestinal systems .
There is a wide range in neurodevelopmental outcome, which depends on the effectiveness of management of hydrocephalus as well as the associated central nervous system abnormalities.
 

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Elimination

A parent brought her child to a pediatric neurologist because the boy exhibited a number of serious neurological signs. These included lack of coordination, especially around the region of the trunk, vomiting, lack of ability to develop new motor skills, and headaches. An MRI further revealed an enlarged cranium, a large cyst which appeared to replace much
of the cerebellum and reduction in the size of the corpus callosum. Which of the following is the most likely diagnosis?

a. Pyramidal tract syndrome
b. Spina bifida
c. Anencephaly
d. Dandy-Walker syndrome
e. Meningomyelocele
So no shock but my answer would also be D.
One way to help you answer questions not sure of, especially in FRED and UWorld programmes, is to cross out the answers that you know are wrong. Let's do that here

a. Pyramidal tract syndrome
Lesions of the pyramidal tract have Parkinsonian symptoms, so festinant gait, fine resting tremor, etc. Think of a diagnosis of PD, another Parkinson plus syndrome such as Multi-System Atrophy etc, or as a side effect of drugs, particularly typical anti-psychotics, such as haloperidol. Needless to say, vanishingly rare for a pediatric neurologists patient cohort.

b. Spina Bifida
This is one of the diseases on the neural tube defect (NTD) spectrum. The diseases are typified as the name suggests by incomplete closure of the neural tube in the early stages of embryogenesis. The symptoms listed here do not fit with this diagnosis.

c. Anencephaly
This is the most severe of the neural tube defects and is incompatible with life (the translation is 'no brain'). These fetuses are still born. Therefore this has to be wrong.

d. Dandy Walker Syndrome
Even if you know absolutely nothing about this condition, it is the only one that is not struck out by simple reasoning. Therefore logic determines that this must be the correct option.

e. Meningomyelocele
This is yet another disease on the NTD spectrum. Here the defect is usually in the cervical (or occaisionally lumbar) spine, with failure of closure of the dorsal aspect of the vertebrae, and herniation of all the meninges leaving a fluid (CSF) filled sac covered in skin herniating from the back of the child.

There are some questions where you unequivocally know the answer. These are great, but most other people probably do too. Therefore you need a system to get the trickier questions that will improve your score above the mean.
One method is the strikeout method. This will at least narrow the choices to one or two, or very occasionally three with resulant greater odds of correctly guessing a 1:2 or 1:3.
 
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