Neurodegenerative Disorders - Demyelinating Disorders + ALS

Question 1

Demyelinating Disorders - Definition

Answer 1

Destruction of myelin sheath, not affecting directly other elements of nervous system. Neuron membrane not able to change enough ions to cause depolarization -> action potential.

Question 2

Demyelinating Disorders - Multiple Sclerosis - Definition

Answer 2

Progressive idiopathic autoimmune disease. Myelin damaged, decreasing/impairing transmission of neural electrical conduction, leading to sensorimotor, cognitive and psychiatric symptoms.

Question 3

Multiple Sclerosis - Supratentorial (neocortex) Symptoms (5)

Answer 3

Motor plan
Executive functions
Aphasia
UMNS
Sensory deficits

Question 4

Multiple Sclerosis - Infratentorial (subcortical structures) Symptoms (5)

Answer 4

Automatic reaction deficit
Coordination deficit
Speech disorders
Dystonia
Attention disorders

Question 5

Multiple Sclerosis - Spinal Cord Related Symptoms (2)

Answer 5

LMNS
Strength

Question 6

Plaques - Multiple Sclerosis

Answer 6

White, rounded areas of neuron apoptosis, dysfunction in the area.
Not seen in the first 30-40 days after an acute relapse.

Question 7

MS Disease Types - Clinical Isolated Syndrome (CIS)

Answer 7

Happens only once and never again.
Episode lasting 24h of demyelination, cause focal symptoms. Not able to get MS diagnosis. Enhance chances of developing MS significantly.

Question 8

MS Disease Types - Relapsing remitting MS (RRMS)

Answer 8

85% of all MS.
Clearly defined relapses, followed by partial or complete recovery, no apparent progression.

Question 9

MS Disease Types - Primary progressive MS (PPMS)

Answer 9

15% of all MS. Worsening of neurological functions, without relapse or remission periods.

Question 10

MS Disease Types - Secondary progressive MS (SPMS)

Answer 10

Progressive type, developed by a few CIS patients. Starts as RRMS and develops into SPMS.

Question 11

Progressive Multifocal Leukoencephalopathy - Definition

Answer 11

Progressive demyelinating disease due to reactivation of John Cunningham virus. Observed in patients with compromised immune system or undergoing pharmacological immunosuppressive therapy: AIDS, RRMS (meds), organ transplant…

Question 12

Progressive Multifocal Leukoencephalopathy - Symptoms (6)

Answer 12

Generalized muscle weakness
Impairment of balance and gait
Dysarthrophonia
Executive functions (mood, personality)
Visual deficit
Coordination deficit

Question 13

Amyotrophic Lateral Sclerosis - Definition

Answer 13

Idiopathic. Fast-progressive neurodegenerative disease. Apoptosis of 1st and 2nd motoneuron. Progressive muscle weakness. Prognosis: 7 years due to respiratory complications.

Question 14

Amyotrophic Lateral Sclerosis - Diagnosis

Answer 14

Clinical evaluation, confirmed by MRI
- Lesion in first MN
- Lesion in second MN
- Other neurodegenerative diseases excluded.
- MRI findings compatible with symptoms

Question 15

Amyotrophic Lateral Sclerosis -
Symptoms and Progression (3)

Answer 15

3 Main phases:
- muscle weakness, dysphagia, fasciculation(contraction-relaxation in high frequency)
- Hypotrophy, hypertonia, dysarthrophonia (difficult to control muscles in throat and abs, difficult to speak)
- Hypomobility, pain, deformation, respiratory complications, death.

Question 16

Amyotrophic Lateral Sclerosis - Treatment Possibilities (3)

Answer 16

Lithium and riluzone.
Ventilation with high levels of O2
Physiotherapy: improvement of functionality and reduction of complications