Brain Cancers and NMJ Disorders

Question 1

What are the 3 main cardinal signs of brain tumours?

Answer 1

1) Headaches and Raised Intracranial Pressure
2) Epilepsy
3) Progressive Neurological deficit

Question 2

What are the main S/S of raised intracranial pressure?

Answer 2

1) +/- Vomiting
2) Headaches
3) Drowsiness

Question 3

You ask a patient with a brain tumour about any factors that aggravate their headache. What might they say?

Answer 3

1) Worst thing first in the morning

2) Coughing, straining or bending forward are felt the worst

Question 4

What is the main cardinal sign of ICP?

Answer 4

Papilloedema

- Venous obstruction return from retina

Question 5

In what organs do secondary tumours arise from?

Answer 5

1) Lung (NSCC)
2) Breast
3) Malignant melanoma
4) Kidney
5) Gut

Question 6

What is the main treatment for secondary tumours?

Answer 6

1) Surgery and adjuvant radiotherapy
2) Chemotherapy
3) Supportive Care

Question 7

From what cell do primary brain tumours originate?

Answer 7

Glial cells:

• Astrocytoma (90%).
• Oligodendroglioma (<5%).

Question 8

Which primary tumour are associated with defects in chromosome 1 and 19?

Answer 8

• Oligodendroglioma

Question 9

What are the 3 main features of oligodendroglioma?

Answer 9

1) Genetic defects in chromosomes 1 and 19
2) IDH1 mutation positive
3) Chemo sensitive
4) 10-15 median survival

Question 10

What is the WHO glioma tumour staging?

Answer 10

G1: Benign paediatric tumour
G2: Pre-Malignant Tumour
G3: Astrocytoma
G4: Glioblastoma Multiforme (GBM)

Question 11

What are the main features of a G2 glioma?

Answer 11

Ep: Young adults
Symptom: Seizures

Question 12

What are 3 causes of G2 Glioma deterioration?

Answer 12

1. Tumour transformation to a malignant phenotype.
2. Progressive mass effect due to slow growth.
3. Progressive neurological deficit from functional brain destruction.

Question 13

What are the main features of GBM?

Answer 13

PP: Initial genetic error of glucose glycolysis -> IDH 1 mutation -> excessive build up of 2-hydroxyglutarate -> genetic instability in glial cells triggered.
Good Prognosis: 1. <45 y/o.
2. Aggressive surgical therapy.
3. Good performance post surgery.
4. Secondary GBM.
5. MGMT ‘mutant’ - will respond well to chemo.

Poor Prognosis: 1. >50 y/o.

2. Poor neurological function after surgery.
3. Non-radical surgery treatment.
4. Primary GBM.
5. MGMT ‘wild type’ - no response to chemo.

Question 14

Describe the treatment for GBM.

Answer 14

1. Resective surgery.
2. Adjuvant chemotherapy with Temozolomide.
3. Palliative care.

Question 15

What disease is associated with NMJ damage?

Answer 15

Myasthenia Gravis

Question 16

What is the PP is behind Myasthenia Gravis?

Answer 16

Autoimmune disorder

• Autoantibodies IgG attach to Ach receptors and destroy them
• Fewer action potentials are able to fire and causes muscle fatigue and muscle weakness

Question 17

What are the main symptoms of Myasthenia Gravis?

Answer 17

• Muscle Fatigue

- Muscle weakness

Question 18

What are the main investigations of Myasthenia Gravis?

Answer 18

1. Serum anti-Ach receptor antibodies.

2. Ask the patient to count to 50.