MS & ALS

Question 1

DF , Etiology . pathogenesis of MS

Answer 1

-DF :It is an INFLAMMATORY disease of the CNS (Brain & Spinal cord). It affects mainly the WHITE MATTER in the form of patchy DEMYELINATION
– Etiology : —“-UN-K-N-O-W-N=, -probably AUTOIMMUNE
-Pathogenesis : The immune system attacks its own CNS, leading to DEMYELINATION
* ANTIBODIES occur against proteins in the myelin sheath surrounding the nerves.
* This causes inflammation and injury’s to the sheath and ultimately to the nerves.
* The result may be multiple areas of scarring (SCLEROSIS).
* The damage slows down the nerve signals leading to impairment of the function

Question 2

RISK FACTORS OF MS

Answer 2

–RISK FACTORS
1. INHERITANCE: Genetic factors play a role.
2. INFECTIONS: Viral or Bacterial infections may trigger the disease
3. STRESS: Physical & Emotional.
4. SURGERY & TRAUMA.
5. Pregnancy & Labour.
6. LOW VITAMIN D LEVELS.

Question 3

CLINICAL PICTURE of MS

Answer 3

-TYPE OF THE PATIENT :
Age: 15 - 45 years. Sex: more common in FEMALES
-BEHAVIOUR OF THE DISEASE:
Onset: usually acute. Course: commonly REMISSIONS & EXACERBATIONS
—FEATURES : any of the following manifestations:
1. Mentality dysfunction
- Depression or Euphoria or Emotional !ability - Cognitive impairment
2. Speech dysfunction (Dysarthria)
- SSS: Staccato, Slurred, Scanning.
3. Cranial nerved sfunction 2, 3, 7, 8
* Optic: 2 Optic neuritis (VISUAL LOSS), visual field defects.
* Oculomotor:3 Ophthalmoplegia, Diplopia,
* Facial:7 UMNL (commonly), /LMNL (rarely).
* Cochleo-vestibular: 8 Vertigo (common).
4. MOTOR dysfunction :(UMNL)
- PARAPARESIS, Monoparesis, hemiparesis, quadriparesis, - Pseudo-bulbar palsy.
5. SENSORY dysfunction (Most common initial feature of MS)
5.1Initially: Parasthesias, Late: sensory loss (S or D) & sensory ataxia
5.2 Lhermitte sign: Electric-like sensation felt in the back & limbs on flexing the neck.It is due to posterior column affection in the cervical region.
6. CEREBELLAR dysfunction
- Features of cerebellar ataxia (common).
7. AUTONOMIC dysfunction
- Bladder dysfunction: Urgency (most common bladder feature), Incomplete emptying.
- Bowel dysfunction: Constipation, poor evacuation, incontinence.
- Sexual dysfunction: Impotence.
8. EPILEPSY
- Occurs in 2 to 3 % of patients.
9. HEAT SENSITIVITY (Uhthoff phenomenon)
- Small increases in body temp. can temporarily worsen current or preexisting features.
-Uhthoff phenomenon is presumably the result of: conduction block developing in central pathways as the body temperature increases

Question 4

CLINICAL PATTERNS ( Types OF MS )

Answer 4

1. RelaP-sing remitting (RRMS): 85%
   1.1 Attacks which leave permanent deficits, followed by periods of remission, or less commonly:
   1.2 Attacks which do not leave permanent deficits, followed by periods of remission (Benign MS).
2. Progressive: 10%
   2.1 Primaryprogressive: {PPMS)
   From the onset, continuous deterioration occurs with no periods of remission.
   2.2 Secondary progressive: {SPMS)
   Initial RRMS that changes to continuous deterioration occurs with no periods of remission.
3. Progressive relapsing (PRMS) 5%
   3.1 Steady deterioration since onset with superimposed attacks.

Question 5

OTHER ENTITIES

Answer 5

1. Clinically Isolated Syndrome (CIS)
   -It is the first attack of a disease compatible with MS (eg, optic neuritis) that exhibits characteristics of inflammatory demyelination but has yet to fulfill MS diagnostic criteria (sometimes labeled possible MS).
2. Radiologically Isolated Syndrome (RIS)
   - Incidental brain MRI findings highly suggestive of MS in the absence of: signs or symptoms of the disease.

Question 6

INVESTIGATIONS

Answer 6

1. Fundus examination:
   * Pallor of the optic disc.
2. CSF examination: “When MRI is unavailable or nondiagnostic”
   * Cells: mainly T lymphocytes
   * Proteins: mainly IgG.
   -The most important findings in CSF
   * INCREACE GAMMA GLOBULINS especially IgG.
   * OLIGOCLONAL BANDS on protein electrophoresis in 95 % of cases.
3. Cortical Evoked Responses: “CER”
   3.1-Nomrnlly: Stimulation of any sensory receptor (visual, auditory, or somatosensory) evokes an electrical signal in the corresponding region of the cerebral cortex “CER”.
   3.2- In MS: Stimulation of any sensory receptor (visual, audito1y, or somatosensory) evokes a slow or abnormal CER due to loss of myelin (t nerve impulse conduction).
   –Therefore: Recording of CER may help in detection of demyelinated lesions in MS e.g. Abnormal Visual Evoked Potential (VEP) = lesion in the visual pathway.
4. IMAGING: (MRI)
   A. Confirms the diagnosis: (Most important investigation)
   Patchy multiple areas in the white matter (Plaques = areas of demyelination).
   B. Differentiates new lesions from old lesions:
   In new lesions of MS, recent inflammation leads to increased vascular permeability; this is detected by leakage of IV contrast agent Gadoliniwn into the brain on MRI.
   .

Question 7

Treatment of MS

Answer 7

1. ImmunoModulatory Therapy
   -VALUE
   * They reduce: the frequency & severity of the relapses.
   * They delay: the progression to disability.
   –MEDICATIONS
   Disease-Modifying Agents for MS (DMAMS) currently approved
   for use by the FDA include the following:
   * Interferon beta-1 a.
   * Interferon beta-1 b.
   * Glatiramer acetate.
   * Mitoxantrone.
   * Alemtuzumab.
   * Daclizumab.
   * Natalizumab.
2. ImmunoSuppressive Therapy “MAC”
   * Methotrexate.
   * Azathioprine.
   * C: Cyclophosphamide, Cyclosporine, corticosteroids :
   Methylprednisolone: .