CASE CNS DEVELOPMENT & MALFORMATION

Block              :  Neuroscience

Week              :  1

Theme            :  Central Nervous System Development & Malformation

Week Chair    :  dr. Astradea Simanungkalit, SpS

Case Title       :  Hydrocephalus and Cerebellum Hypoplasia

Week Objectives:

1.    Describe the development of the nervous system.

2.    List the component of central nervous system (hemisphere, cerebellum, brain stem,
spinal cord).

3.    Describes the major parts of the brain and cerebellum. State functions for each
structure.

4.    Describe the embryology and anatomy of the brain, cerebellum, and brain ventricles

5.    Describe the production, circulation and absorption of cerebrospinal fluid

6.    Describe the congenital anomalies of the central nervous system

Case Objectives:

1. Define the term of macrocephaly, hydrocephalus, and cerebellum hypoplasia

2. Describe etiology of congenital anomalies that cause cerebellum hypoplasia and
hydrocephalus
3. Describe the patophysiology of CSF circulation disorder
4. Describe the clinical manifestations and complications of hydrocephalus and
cerebellum hypoplasia
5. Describe the treatment for hydrocephalus and seizure
6. Describe the prognosis of congenital anomalies involving cerebellum hypoplasia
Page 1

A 20-year-old woman lived in Karawaci was admitted to the ER at 9 months of

pregnancy due to uterine contraction. She was unmarried and had an unplanned
pregnancy. She did not realize she was pregnant until 12th weeks of pregnancy and
during that time she had a habit of alcohol consumption. She only had checked her
pregnancy that one time and did not go for a follow up.

1. What are the problems and risk factors of this pregnancy? 

2. What should be checked and evaluated in the ante-natal care? 

3. What are the possible problems in the baby according to the risk factors in
this case?

Page 2

             Ten months later, the mother brought the baby boy to a pediatrician in Siloam
Hospital. There was no prior visit for monthly check up and immunization prior to this
visit. She started noticing recently that the child has enlarged head, mainly on the back
of his head. He could roll over, but had not started to crawl, only able to sit with
support, and started babbling just recently.

            On physical examination, head circumference was 49 cm, body length 70 cm,
body weight 8200 kg. Vital sign was normal and the other physical examination results
were unremarkable. The baby was alert, can roll, but had difficulties to sit by his own. On
motor examination, there was mild spasticity on both arms and legs.

1. Currently, is there any problem with this baby? 

2. What can cause the problems found in this baby? 

3. What information does the mother needs to know about the baby? 

4. What radiologic examination would you sugest to assess the baby

problems?