☝️
🔺 In NEUROMUSCULAR DISEASES
Number 12 (the correct D) .

Immunotherapy in Neurological diseases:

-Immunotherapy is the treatment of disease by activating or suppressing the immune system.
-Two forms:
1)Activation immunotherapies:
Elicit or amplify an immune response.
E.g. chemotherapy in treatment of some brain tumors.
2)Suppression immunotherapies:
Reduce or suppress immune response.
E.g.Immunosuppressive drugs such as Glucocorticoids.

-Immunomodulators: are the active agents of immunotherapy.

Examples of different modalities used in the management of neurological diseases:

1)Glucocorticoid treatment:
E.g.Prednisone, methylprednisolone.
1-In acute attack of Multiple sclerosis.
2-Long treatment in myasthenia gravis.
3-Bell's palsy.
4-Temporal arteritis.
5-Cluster headache.
6-SLE, dermatomyositis,polymyositis.
7-Some epilepsy syndromes,such as West syndrome" ACTH".
8-Becker muscular dystrophy.

2)Pharmacological immunosuppression treatment:
Eg.Azathioprine , mycophenolate mofetil.
Used in:
1-Myasthenia gravis.
2-Neuromyelitis optica (Devic's disease).
3-SLE.
4-Dermatomyositis,polymyositis.
5-Behçet's disease.


3)Intravenous immunoglobulin(IVIG):
-It is the use of a mixture of antibodies (immunoglobulins) to treat a number of health conditions.
Conditions in which used:
1-Guillain-Barré Syndrome.
2-Chronic inflammatory demyelinating polyneuropathy.
3-Multifocal motor neuropathy.
4-Stiff person syndrome,
5-Multiple sclerosis
6-Acute exacerbation of myasthenia gravis"short term".
7-Dermatomyositis and polymyositis.

4)Plasmapheresis:
-It's a broad range of procedures in which extracorporeal separation of blood components results in infiltrated plasma product.
Indications:
1-GBS.
2-Myasthenia gravis.
3-Chronic inflammatory demyelinating polyneuropathy.
4-Wilson's disease.
5-Lambert–Eaton myasthenic syndrome .
6-Multiple sclerosis.
7-Neuromyelitis optica.
8-Transverse myelitis.
9-HIV-related neuropathy.
10-Acute disseminated encephalomyelitis (ADEM).
11-Antiphospholipid antibody syndrome (APS or APLS).


5)Cytokine "interferon-beta-1":
-In treatment of Multiple sclerosis.

6)Monoclonal antibodies:
E.g Ocrelizumab, Alemtuzumab,etc
-In Treatment of Multiple sclerosis.

🔳=Class
◼️=Group
▪️=Drug

Management Strategies of Guillain-Barré Syndrome:


a.Immediate management of patients with AIDP"Acute inflammatory demyelinating polyneuropathy" focuses on close monitoring and supportive care:
1) Serial maximal respiratory pressures and forced vital capacities (FVC)
2) Mechanical ventilation and intensive care for precipitous decrease in any measure or low respiratory values: (FVC <20 mL/kg, maximal inspiratory pressure <30 mm Hg, or maximal expiratory pressure <40 mmHg).
3) Management of autonomic instability:
-Labile blood pressure, cardiac arrhythmias (cardiac monitoring required), urine retention, and ileus.
4) Management of pain:
-Very common symptom in acute and plateau phases of care.
-Refractory pain may be treated with antiepileptic medications; short-term narcotics may be necessary.

b. Management of disease course: Immunomodulation
1-Intravenous immunoglobulin:
-(IVIG; 0.4 g/kg/day for 5 days)
-IVIG is often the initial therapy chosen because of its ease of administration and good safety record.
-IVIG may be preferable to plasma exchange (PE).

2-Plasmapheresis:
-(Five to six exchanges over1–2 weeks)
-Intravenous immunoglobulin and plasmapheresis are likely equally effective in mitigating disease severity and should be instituted early and appear equally effective when given within the first 2 weeks after onset.
-Combination therapy consisting of both does not provide additional benefit.
-Plasmapheresis may be precluded in hemodynamically unstable patients.
-These measures generally increase the pace of recovery, although their effects on the severity of the disease, the risk of respiratory and autonomic dysfunction, and ultimate disability are less clear.
-Do not give IVIG then PE,because PE will wash IG.

3-Corticosteroids:
-Have not been found to be effective in GBS.

نفس هذه.

بالنسبة للـ"Aplastic anemia "؛ الدكتور ماشرحها، لكن هي صفحة.

Common manifestations of MS:
From most to least common.

1-Sensory disturbance:
The most common presenting
manifestations of MS are paresthesias: tingling, pins and needles, dysesthesias (burning, gritty, sandy, electrical, or wet sensations), or hypoesthesia.

2-Motor symptoms:
Motor symptoms are the second most common initial manifestation of MS and include limb weakness, loss of dexterity, and gait disturbance.

3-Optic neuritis:
The third most common presenting manifestation of MS is optic neuritis, which is characterized by loss of vision affecting usually one eye evolving over hours or days.Loss of vision can be
complete or partial. May be Scotoma.It is often associated with periorbital pain during eye movements. Reduced color vision.

4-Ataxia and tremor:
Discoordinated movements of the limbs or gait are common in MS and can be caused by plaques affecting the cerebellar afferent or efferent pathways.

5-Diplopia:
Double vision is common in MS and is caused by disconjugate eye movements.
The most common abnormal eye movement observed in MS is internuclear ophthalmoplegia (INO). Due to Demyelinating plaques that affect the medial longitudinal fasciculus, a tract that yokes the sixth cranial nerve nucleus.

6-Vertigo:
It can be the initial manifestation of MS or can occur at any time during the course of the disease.

7-Fatigue:
Over the course of the disease, fatigue is one of the most common MS symptoms and contends with cognitive impairment as the major cause of loss of vocation.

8-Facial pain:
Trigeminal neuralgia.
To distinguish it from its idiopathic counterpart are bilateral occurrence, constant duration, involvement of the first branch of the 5CN.

9-Bladder and bowel dysfunction:
Patients often complain of urinary urgency, frequency, hesitancy, and incontinence.

10-Facial weakness:
Lower motor neuron facial weakness, similar to Bell's palsy.

11-Flexor spasms:
One of the paroxysmal symptoms of MS, and a hallmark of the disease, is flexor spasms.

12-Neuropsychiatric dysfunction:
Difficulties with short-term memory, attention, information processing, problem solving, multitasking, and language function.

13-Dysarthria
14-Dysphagia:
Particularly later in the course of the disease.

15-Facial myokymia:
Chronic flickering contractions of the orbicularis occuli or other muscles of facial expression.

Others: Headache, Hearing loss, Cramps, Loss of consciousness, Loss of taste,Tinnitus.

Investigation

* CBC to exclude other dx .
* work up of peripheral neuropathy
Thyroid panel
Rheumatology profiles
Vitamin B-12
Folic acid
Hemoglobin A1C
(ESR)
Rapid protein reagent
Immunoelectrophoresis of serum protein
Tests for heavy metals .
* biochemical
- LFT
- electrolyte level
- CPK level
- stool culture for C.jejeni
* serologic tests for virus
* serum autoantibodies
Ab of GM1 , antiGM1
* Pulmonary function tests
* ABG ..O2 saturation
* CSF unlysis
albuminocytologic dissociation, which is an elevation in CSF protein (>0.55 g/L) without an elevation in white blood cells.
May be normal in the 1st days
* nerve conduction studies
helpful in the diagnostic workup and prognostic evaluation of patients with suspected GBS.
*Others
EMG
Imaging
Muscle biopsy .