4d. NPH & CJD (subcortical dementia)

Question 1

What happens in NPH?

Answer 1

Normal Pressure Hydrocephalus

There is a change in the cerebrospinal fluid of the brain leading to enlargement of the ventricles and this leads to brain damage for a part reversible (the only dementia that is for a part treatable).

It’s called idiopathic because we don’t know the exact cause.

Question 2

What are 3 symptoms of NPH?

Answer 2

1. Cognitive deficits: psychomotor slowing, impaired attention, executive and visuospatial dysfunction.
2. Gait disturbances: walking apraxia = difficulties with starting walking, magnetic walk = feet are stuck to the ground like magnetics.
3. Urinary incontinence

Question 3

What are 2 things that are important to remember in NPH?

Answer 3

1. To remember the symptoms of this disease you have to remember “wet, wacky and wobbly”.
2. Patients are pretty much aware of their cognitive deficits.

Question 4

What do you see on EEG in NPH patients?

Answer 4

Comparable with other dementias > slowing of brain waves.

Question 5

What do you see on MRI in NPH patients? (3x)

Answer 5

1. Proportional large ventricles leading to crowding of the brain tissue.
2. Damage due to the pressure > white around the ventricles
3. Loss of curves in the cortex and the corpus callosum getting thinner

Question 6

What are 2 treatment options in NPH?

Answer 6

1. Lumbar punction: get some fluid out of the back > then the pressure gets lower > you can see whether there is an improvement of the symptoms.
2. Shunt: from the brain to the stomach to get the CSF into the stomach > symptoms may improve.

Question 7

What especially improves after treatment in NPH?

And hardly any effect on?

Answer 7

Improvement: especially the gait improves, and also the sphincter control (=the urinary control)

Hardly any effect on: cognition

Question 8

What is CJD?

Answer 8

Creutzfeld Jacob’s Disease

This is the most rare disease of the lectures. It’s a prion disease leading to holes in the brain filled with water and eventually spreading around the whole brain leading to a rapid process of dementia and deterioration.

Most of the time it’s sporadic CJD which is not related to infected meats.

Question 9

What are 3 features of diagnosis of Classic CJD?

2nd one > 2 out of 4

Answer 9

1. Fast progressive dementia (duration +- 4/5 months)
2. 2 out of 4:
   o Myoclonus = movement of the muscles
   o Visual or cerebellar disorders = coordination problems
   o (Extra)pyramidal signs
   o Akinetic mutism = they can hardly speak at the end of the disease
3. Specific EEG abnormalities (see notes)

Question 10

What is cortical ribboning? And in what disease is it present?

Answer 10

The ventricles are enlarged (in CJD).

Question 11

What are features of diagnosis of Variant CJD?

1. The following 3…
2. 4 out of 5…

Answer 11

1. The following 3:
   a. Progressive neuropsychiatric symptoms
   b. Duration > 6 months
   c. Routine care – no other explanation, no positive family history for spongiform encephalopathy
2. AND 4 out of 5:
   a. Early psychiatric symptoms
   b. Persistent painful sensibility disorders
   c. Ataxia
   d. Myoclonus, chorea or dystonia
   e. Dementia

Question 12

What do you see on a MRI in case of the variant form of CJD?

Answer 12

You can see pulvinar signs, which look like a hockey stick in the thalamus.

Question 13

What are the 3 main differences between classic and variant CJD?

Answer 13

1. Duration
2. Psychiatry
3. EEG: in variant form no typical EEG