Central Nervous System (CNS)
- Brain:
- Cerebral Hemispheres:
- Two hemispheres (Right & Left).
- Each composed of lobes with centers and tracts (motor & sensory).
- Frontal Lobe:
- Personality, emotional control, social behavior.
- Micturition centers.
- Voluntary motor control.
- Parietal Lobe:
- Language (speech).
- Calculation, visual-spatial, constructional skills.
- Memory centers, optic tract, somatic sensation.
- Temporal Lobe:
- Auditory centers.
- Verbal memory.
- Smell centers.
- Occipital Lobe:
- Basal Ganglia:
- Function of the extrapyramidal system.
- Brain Stem:
- Composed of midbrain, pons, and medulla oblongata.
- Functions:
- Contains all sensory and motor pathways.
- Nuclei of cranial nerves (all except 1 & 2).
- Some centers (respiratory, conscious, sympathetic chain).
- Cerebellum:
- Composed of lateral hemispheres, vermis, and flocculonodular lobe.
- Function:
- Coordination (balance) of peripheral and axial body.
- Balance of eye and ear function.
- Spinal Cord:
- Composed of 31 segments.
- Ends at level L1 as conus medullaris & cauda equina.
- Transverse Section Components:
- Gray Matter:
- 2 anterior horn cells (motor nuclei).
- 2 posterior horn cells (sensory nuclei).
- Collections of cells for lower-order reflexes.
- White Matter:
- Afferent and efferent fibers of motor & sensory tracts.
- Stabilizes the spinal cord.
Peripheral Nervous System (PNS)
- Spinal nerves + Cranial nerves
- Anterior horn cell of spinal cord segment → peripheral spinal nerve → neuro-muscular junction → muscle
- Defect = Astereognosis + Agraphaesthesia
The Motor System
- Components:
- Pyramidal Tract (Voluntary Movements):
- CST (Corticospinal Tract).
- CBT (Corticobulbar Tract).
- Extra-Pyramidal Tract (Involuntary Movements):
- Function of Basal Ganglion.
- CST (UMN):
- Course:
- Motor cortex → Posterior limb of internal capsule until the anterior 2/3 → midbrain → pons → medulla oblongata (decussates in lower 1/3) → spinal cord → anterior horn cells → peripheral spinal nerves 'lower motor neurons'.
- AHC → Spinal segment → peripheral spinal nerve → neuro-muscular junction → muscle
- Muscle reflex arch
Motor System Lesions
- UMNL (Upper Motor Neuron Lesion) = CST lesion
- LMNL (Lower Motor Neuron Lesion) = PNS lesion
- UMNL vs. LMNL:
- Inspection:
- UMNL: Muscle bulk = normal (but mild atrophy due to disuse); no fasciculations.
- LMNL: Muscle bulk = marked atrophy; positive fasciculations.
- Tone:
- UMNL: Hypertonia = spasticity (clasp knife spasticity).
- LMNL: Hypotonia = flaccidity.
- Power:
- UMNL: Decreased.
- LMNL: Decreased.
- Reflexes:
- UMNL: Hyperreflexia, ankle clonus (+), Babinski (+), Abdominal (-), Cremasteric (-), Anal (-).
- LMNL: Hyporeflexia, ankle clonus (-), plantar reflex (-), Abdominal (-), Cremasteric (-), Anal (-).
- Differential Diagnosis (DD):
- UMNL:
- CVA (Cerebrovascular Accident).
- MS (Multiple Sclerosis).
- MND (Motor Neuron Disease).
- Space-occupying lesion.
- Spinal cord lesion.
- LMNL:
- AHC = Poliomyelitis.
- Spinal segment = radiculopathy.
- PSN = GBS (Guillain-Barré Syndrome).
- NMG = MG (Myasthenia Gravis).
- Muscle = congenital muscle disease.
- Note: Sudden onset of UMNL may lead to spinal shock, presenting with hypotonia and hyporeflexia.
- Note: Muscle wasting + fasciculation + hyperreflexia + hypertonia = Mixed lesion.
Cerebellum Clinical Lesions
- Types:
- Cerebellar ataxia
- Sensory ataxia
- Vestibular ataxia
- Causes:
- Congenital:
- Friedreich's ataxia
- Spinocerebellar ataxia
- Structural:
- Toxic:
- Drugs (lithium, phenytoin, amiodarone, toluene, 5-fluorouracil, cytosine arabinoside)
- Alcohol
- Heavy metals/chemicals (mercury, lead, thallium)
- Infection/Post-infectious:
- HIV
- Varicella zoster
- Whipple’s disease
- Degenerative:
- Multiple system atrophy
- Creutzfeldt–Jakob disease
- Inflammatory/Immune-mediated:
- Multiple sclerosis
- Gluten ataxia
- Paraneoplastic ataxia
- Hashimoto encephalopathy
- Metabolic:
- Vitamin B1 or E deficiency
- Hypothyroidism
- Hypoparathyroidism
- Vascular:
- Stroke
- Vascular malformations
- Superficial siderosis
- Clinical Abnormality:
- Face:
- Head nodding & titubation
- Dysarthria = Staccato, scanning, explosive speech
- Horizontal nystagmus
- Upper Limb:
- Finger-nose test = intention tremor & dysmetria
- Rapid alternating movements = dysdiadochokinesia
- Rebound test = overshooting
- +/- Hypotonia & pendular reflex
- Lower Limb:
- Heel-shin test = impaired
- Gait = ataxic gait
- Romberg sign = positive
The sensory system
- Speech abnormalities
- DD of dysphasia :
- Brocs dysphasia
- Wernickes dysphasia
- DD of dysarthria:
- Staccato speech = ataxia, MS
- Slurred speech = CN 5,7,12 palsy (MND, MG)
- Monotones speech = parkinsonism
- DD of Dysphonia:
- RLN injury
- Vocal cord lesion
- Posterior(dorsal) columnsensory tract
- Spino talamic (anterior lateral) sensory tract
Site of Lesion and Motor/Sensory System Effects
- Cerebral Hemisphere:
- Motor: Contralateral hemiplegia or paresis (UMN in UL & LL)
- Sensory: Contralateral sensory loss of face and body
- Brain Stem:
- Motor: Contralateral hemiplegia or paresis (UMN in UL & LL)
- Sensory: Ipsilateral sensory loss of face, contralateral sensory loss of body
- Hemicut above C5:
- Motor: Ipsilateral hemiplegia or paresis (UMN in UL & LL)
- Sensory: Complete cut above C5 results in DEAD
- Hemicut C5-T1:
- Motor: Ipsilateral hemiplegia or paresis (LMNL in UL, UMNL in LL)
- Sensory: Complete cut C5-T1 results in quadriplegia or paresis (LMNL in UL, UMNL in LL)
- Hemicut T1-L1:
- Motor: Ipsilateral monoplegia or paresis (UMNL in LL)
- Sensory: Complete cut T1-L1 results in paraplegia or paresis (UMNL in LL)
- Hemicut Below L1:
- Motor: Ipsilateral monoplegia or paresis (LMNL in LL)
- Sensory: Complete cut below L1 results in paraplegia or paresis (LMNL in LL)
- If Hemi cut : ipsilateral PCS lost & contralateral STT lost (brown squared syndrome) below site of lesion
- If complete cut : complete sensory lose below site of lesion
Spinal Cord Lesions
- Causes:
- Spinal compression.
- Postural trauma.
- Inflammation (transverse myelitis).
- Neoplasm.
- Artery (ASA thrombosis).
- Lesions of degeneration (Friedreich's ataxia, Vit B12 deficiency, myelopathy, syringomyelia).
Cerebrovascular Accident (CVA)
- Anatomy:
- Brain supplied by two main systems:
- Carotid system:
- ICA → ACA & MCA → supply cerebral hemisphere.
- Vertebral basilar system
- Each artery supplies a part of the brain:
- Anterior Cerebral Artery (ACA): Supplies the frontal lobe.
- Middle Cerebral Artery (MCA): Supplies the temporal and parietal lobe.
- Posterior Cerebral Artery (PCA): Supplies the visual centers.
- Basilar Artery: Supplies the brain stem and cerebellum.
- Note:
- Motor involvement:
- ACA → L.L > U/L & face.
- MCA → U/L & face > L.Limb.
- PCA → face & U/L > L.limb.
- Sensory involvement: Contralateral hemisensory loss.
- Definition: Acute Focal neurological defect due to a vascular cause.
- Types:
- Ischemic CVA (85%):
- Embolism is the most common cause (heart = A.Fib).
- Thrombosis.
- Hypotension.
- Arteriosclerosis.
- Vasculitis.
- Space-occupying lesion.
- Risk factors:
- Hypercholesterolemia.
- Uncontrolled HTN & D.M.
- Obesity = high LDL.
- Smoking.
- Lack of exercise.
- Alcohol.
- OCP.
- Homocysteinemia
- Hemorrhagic CVA (15%):
- Epidemiology:
- 3rd most common cause of death in developed countries.
- Common in Black populations.
- Age > 40.
- Male > Female.
- Duration of Symptoms:
- Transient Ischemic Attack (TIA): Acute focal neurological defect due to a vascular cause lasting < 24 hours.
- Stroke: Acute focal neurological defect due to a vascular cause lasting > 24 hours.
- Clinical Presentation:
- ACA Occlusion:
- Contralateral hemiplegia or paresis.
- Contralateral hemisensory loss (L.L > U/L & face).
- Emotional changes.
- Behavioral & personality changes.
- Urine incontinence.
- MCA Occlusion:
- Contralateral hemiparesis or plagia.
- Contralateral hemisensory loss (UL & face > LL).
- Auditory & smell disturbance.
- Aphasia.
- Impaired memory: Especially visual spatial memory, calculation, constructional skills.
- Contralateral homonymous hemianopia.
- PCA/Basilar Occlusion (Brain Stem Infarction):
- Contralateral hemiparesis & hemiplegia.
- Contralateral sensory loss of (UL > L.L) of body.
- Ipsilateral sensory of face.
- Ataxia (cerebellum lesion).
- Cranial Nerve palsy (7, 9, 10, 11, 12).
- Visual hallucination & visual loss.
- Horner syndrome.
- Respiratory failure type II.
- Locked-in syndrome (loss of consciousness, can move eyelid).
- Stroke Variants:
- Minor Stroke: Symptoms > 24 hours but resolve without significant defect.
- Progressive Stroke (Stroke in Evolution): Symptoms worsen due to increasing size of infarction.
- Complete Stroke: Rapid, maximum, persistent neurological defect within a few hours due to multiple emboli.
- Source of embolism is the carotid artery, affecting MCA & retinal artery.
- TIA Evaluation: Any patient with TIA should undergo Doppler-Duplex U/S.
- Amaurosis fugax
- Auscultation (Carotid Bruit not always present).
- Lateral Medullary Syndrome:
- Ipsilateral ataxia.
- Ipsilateral Horner's syndrome.
- Ipsilateral CN 5, 9, 10, 11 involvement.
- Contralateral STT sensory loss.
- Differential Diagnosis of CVA:
- Hypoglycemia.
- Space-occupying lesion.
- Encephalitis.
- Epilepsy (Todd’s palsy).
- Peripheral neuropathy.
- Horner's Syndrome:
- Partial ptosis
- Miosis
- Anhydrosis
- Enophthalmos
CVA Investigations
- To Confirm Diagnosis (According to Site):
- CT without contrast: Sensitive in detecting strokes due to ACA & MCA occlusion; aim is to exclude hemorrhagic CVA, as changes of infarction need 48 hours to appear. HRCT scan can show changes in 12-36 hours.
- MRI: Sensitive in detecting strokes of the brain stem & cerebellum due to occlusion of basilar & PCA; also used to exclude hemorrhagic CVA.
- To Detect the Cause:
- Labs:
- Blood sugar
- CBC: Polycythemia & thrombocytosis
- WBC, ESR, & CRP: Due to clot or vasculitis & SLE
- Antinuclear Ab: When suspecting vasculitis due to SLE
- RBS
- Lipid profile
- Imaging:
- Chest X-ray: For aspiration pneumonia.
- ECG.
- Doppler U/S of carotid artery: Both after stabilization of patient.
CVA Complications
- Chest infection → Most Common Cause of death.
- Epilepsy & dementia.
- DVT & Pulmonary embolism (2nd cause of death).
- UTI & Constipation.
- Depression.
- Painful shoulder.
CVA Management
- Initial Steps:
- DO MRI or CT scan
- Hypo dense (Infarction)
- Hyper Dense (White lesion)
- Neurosurgeon Consultant (
- Admit the patient
- Primary Survey (A, B, C):
- A: Patent airway.
- B: Breathing, give patient 60% of O2 for the brain.
- C: Circulation, insert 2 large cannulas and send blood for investigations.
- Measure BP: If hypotension, give IV fluids (5% dextrose saline), but don’t over-hydrate (cerebral edema).
- NGT insertion: For gastric lavage & prevent aspiration pneumonia.
- Insertion of urinary catheter.
- Prevent Further Stroke:
- Aspirin 75mg + Dipyridamole 200mg.
- Control DM: Soluble insulin by Sliding Scale.
- Control BP: Transient HTN seen after stroke usually does not require Rx, but if BP > 230/100 mmHg or end-organ damage, gradually lower BP to 160/90. Anticoagulant is C/I.
- Lipid-lowering agent (statin or omega-3).
- Control Risk Factors.
- Physiotherapy + Rehabilitation: Decrease complications & improve prognosis.
- Thrombolytics:
- Given with caution & only if no contraindications.
- Not given > 4 hours because higher doses are needed, which may lead to rupture → Hemorrhagic CVA.
- Note: Ischemia may lead to fever, which in turn will lead to increase infarction size.
- Indications for Anticoagulant in Stroke:
- Atrial Fibrillation
- Stroke in evolution
- Recurrent TIA
- Blood sugar monitoring is important to exclude DM.
- Before considering management of ischemia, you must exclude hemorrhage.
Management of TIA (Recurrent 30%)
1. Aspirin 75mg for life.
2. Treat the risk factors.
3. Regular Duplex U/S of carotid vessels; if >70% occlusion, stent is required.
Hemorrhagic CVA
- Differential Diagnosis:
- Sunstroke
- Subarachnoid Hemorrhage
- Hypoglycemia
- Drug addict
- Types & Causes:
- Subarachnoid Hemorrhage:
- Rupture of saccular aneurysm
- Berry aneurysm
- A-V malformation
- Idiopathic = hereditary hemorrhagic telangiectasia / PKD
- Intracranial Hemorrhage:
- Rupture of micro B.V.
- Buchard Charcot B/c HTN.
- Clinical Presentation:
- Subarachnoid Hemorrhage :
- Vomiting.
- Blurred vision due to papilledema.
- Irregular respiration.
- Neck stiffness & occipital Headache.
- Coma
- Intracranial Hemorrhage :
- Vomiting.
- Blurred vision due to papilledema.
- Irregular respiration.
- Neck stiffness & Frontal Headache.
- Coma
- Diagnosis:
- CT or MRI (sensitivity 75%)
- Lumbar puncture:
- Xanthochromia after 12 hours (yellowish pinkish CSF) & lasts for 2 weeks.
- ECG: ST-segment elevation
- Treatment:
- Bed Rest.
- Control BP gradually.
- Dexamethasone & Mannitol.
- Urgent neurosurgery consultant call
Intracranial Mass Lesions
- Causes:
- Hematoma
- Infective
- Cerebral abscess (pyogenic, Toxoplasma)
- Tuberculoma
- Hydatid cysts
- Granuloma
- Neoplastic
- Clinical Features:
- Local effects on adjacent brain tissue
- Raised intracranial pressure:
- Headache(Frontal ,early morning, increased by staring, coughing,bending ,wailking)
- Impairment of conscious level
- Papilledema
- Vomiting, bradycardia, arterial hypertension
- False localizing signs 6th cranial nerve lesion (unilateral or bilateral)
- Bilateral extensor plantar responses
- Investigations:
* If there is no CT or MRI fundoscopy papilledema.
* cranial nerve palsies, particularly CN VI (lateral rectus because it has a long intracranial root
- CT ( 1st to be done) or MRI
- CBC, ESR, CRP may be normal
- NB: Lumbar puncture is CI because there is high risk of herniation ( conning)
- Differential Diagnosis of neck stiffness:
- Subarachnoid hemorrhage.
- Meningitis.
- Disc prolapse or subluxation.
- Posterior fossa tumor.
- Migraine.
- Severe upper lobar pneumonia.
- Treatment:
Decrease intracranial pressure by giving manitol & dexamethasone & Rx cause
Benign Intracranial Hypertension (Pseudo tumor cerebri)
* This condition usually occurs in obese young women. without a space-occupying lesion (Exclusion Dx)
* The condition can be precipitated by drugs, including tetracycline, and vitamin A and OCP.
* **CP:**
1. Headache, sometimes accompanied by
2. Transient diplopia & visual disturbance
3. Papilledema
4. 6th cranial nerve palsy may also be present (False localizing sign).
* **Investigations**
1. Lumbar puncture which shows raised CSF pressure,
2. CT OR MRI = normal-sized or small ventricles.
3. Visual perimeter is indicated to monitor the progression of disease.
* **Rx:**
1. Rx any precipitating condition(Decrease weight)
2. Acetazolamide(Diuretics)3- Repeated LP
Multiple Sclerosis
- Definition: Multiple neurological sclerosing defect separated by space & Time (R&R 80%).
- Causes: Demyelination of white matter of CNS.
- T-lymphocyte mediated autoimmune response
- Female > Male (20-40 yrs).
- +ve F/H (Caucasians).
- HLA Dr2.
- Ab in CSF oligoclonal band IgG 80%
- Post Viral infection (Measles), Ag-Ab 2ry Ab in CSF.
- Hot Climate → C/P exacerbation during hot bath (Utthoff's symptoms).
- Clinical Picture:
- Optic Nerve (Most common site):
- Painful unilateral optic neuritis (Intraocular Ophthalmoplegia), start unilateral then bilateral (increased by eye movement).
- Diplopia: Due to optic neuritis → decreasing in propagation of single image less than other eye.
- Impaired color vision (Rods & Cons).
- D/D: 1- M.S 2- Vit B12 Def 3- Transverse Myelitis 4- sub-acute sclerosing pan encephalitis.
- CI if evidence of high ICP
- Brain:
a/ Motor: UMNL Mono progress to- Spastic Hemiplegia or paresis contralateral.
If frontal lobes are affected bilateral pts may have paraplegia progress to quadri
b/ Sensory: PCS Deep & Fine.
Parasthesia and tingling sensation, radiating on arms back legs on flexion of pt. neck due to dorsal column Nuclei involvement. BARBER’S CHAIR OR LERMITTS SIGN.
c/ Cerebellum: dystaxia and other cerebellar sign. Charcot triade (Nystagmus , tremor & Scanning speech)
d/ Frontal: Euphoria or depression (emotional labiality)
e/ brain stem: CN palsy especially CN3+4+6,(Diplopia)CN 5(trigeminal neuralgia), CN7, CN 12 (dysarthria) - Spinal Cord:
a/ Sub-acute degeneration of spinal cord b/ Urinary Dysfunction: Sphincter disturbance incontinise (Common in male)
- Investigations:
- Visual Evoked potential: (delay) used for Subclinical Dx.
- MRI: 1st and most sensitive (White abnormal patches).
- Lumbar Puncture: mild T – Lymphocyte (Pleocytosis) . Protein. Oligoclonal band: IgG 80%
- Acute Relapsing:
1/Steroid ((methyl prednisolone)) 1mg-IV x 3days. 2/Rx Complication.
3/Vit B12. - For prevention of relapsing:
1/interferon beta (DOC)
2/Azathioprine: decrease progression not cure
- Common causes of optic disc swelling
Raised intracranial pressure (papilloedem) 1-Cerebral mass lesion (tumour, abscess) 2-Obstructive hydrocephalus 3-Idiopathic intracranial hypertension Obstruction of ocular venous drainage - 1-Central retinal vein occlusion
2-Cavernous sinus thrombosis Systemic disorders affecting retinal vessels 1-Hypertension 2-Vasculitis 3-Hypercapnia
Optic nerve damage
1-Demyelination (optic neuritis) 2-Leber’s hereditary optic neuropathy 3- Anterior ischaemic optic neuropathy 4-Toxins e.g. methanol, Hypocalcemia 5-Infiltration of optic disc 6-Sarcoidosis 7-Glioma 8-Lymphoma - Prognosis:
- good30% = 10yrs50% = 15yrs.
Poor prognostic factors: *Progressive type. *Frequent relapse. *Male. *Age>40 or <20.*Motor and cerebellar presentation - Optic atrophy
Motor Neuron Disease (Lateral Amylotrophic Sclerosis)
- Definition: Progressive disorder of unknown etiology which results in degeneration of Cranial Nerve Nuclei, AHC, & pyramidal tract.
- Causes: Unknown etiology but theories may be:
- Autoimmune: rare Disease common in male, > 40 yrs not ass’ with other autoimmune
- Trauma: Blunt or electrical.
- Toxins
- Post-viral infections
- Clinical Presentation:
- S&S of UMNL & LMNL:
Cranial Nerve Nuclei & AHC.
Pyramidal tract + Betz Cells. -LMNL. -Msc wasting & fasciculation.
-UMNL. - Reflexes - Cranial nerve involvement (s&s of bulbar & pseudobulbar) difficult in
Speech (Dysarthria). - Swallowing (Dysphagia) Aspiration pneumonia (MCC of death)
- Rarely urinary dysfunction.
- Common Presentation of MS: 1-Optic Neuritis & 6th CN palsy (False Localizing Sign) 2-Spinal cord lesions.
3-Brain stem lesions. 4-R&R Sensory lesions. D/D 1-Cervical cord compression (tumor, disc) 2-Late in Syringomylia 3-Sub-acute degeneration of spinal cord
- if the patient complained of UMNL & LMNL at same limb it’s called ((Mixed Lesion)).
- DD of mixed lesion:
- Motor Neuron Disease.
- Spinocerebellar Ataxia.
- Syringomyelia.
- Cervical myelopathy.
- Vit B deficiency
- No exact Inv but CT & MRI to exclude other D/D
- Treatment:
1/Rilazale (Glutamate antagonist) which improve symptoms. 2/Rx complication. Prognosis: poor prognosis Fatal in 5-7yrs
Gullain Barre Syndrome
- Def: Acute inflammatory ascending poly neuropathy Post infections 1-4 weeks ( CMV+EBV URTIs= Dyspnea) (Campylobacter Gastro enteritis= Diarrhea)
- C/PPolyneuropathy (motor, CN involvement & Sensory)
1/Motor Affect Muscles (LMNL). Lower Limb > Upper limb (Distal to proximal).
Pts complain of fatigability difficulty from raising from sitting position or climbing stairs S&S of LMNL (Loss of Reflexes).
2/Cranial Nerves Involvement: 7th (Bilateral), 3th, 4th, 6th & also bulbar Complaining of (Diplopia, Drooling saliva, regurgitation& dysphagia).
3/Sensory: Parasthesia & Numbness (Distal to proximal) LLUL.
4/Spinal Cord: Urinary symptoms (Retention). 5/Dyspnea: Late in course suggestive of diaphragmatic & intercostal Muscle Weakness RFII Death. may occur within hours of presentation - Inv: 1/Nerve conduction studies: Abnormal (Confirm Dx). 2/Electromyogram (EMG): Normal.
3/CSF: Albumin-Cytology Dissociation ( High Protein & normal cells +Sugar) 4/Identify the Underlying cause: Stool Analysis & Bronchoalveolar lavage.
5/Spirometer & ABG to moniter lung function - No 1-Sensory Loss. 2Ocular impairment. 3Cerebellar or extrapyramidal sign. 4Disturbance of level of consciousness. - Rx: 1/High dose IV gamma globulin during acute phase to reduce symptoms Plasma phoresis or exchange (Useful in Rx 1st week). 3/Ventilator support if respiratory Muscle is affected. 4/physiotherapy & occupational. 5/Vit B12 N.B: steroid has no role b/c no further formation of Abs
Polyneuropathy
- Causes of polyneuropathy
* 1-Genetic:Charcot–Marie–Tooth disease, Familial amyloid polyneuropathy, neuralgic amyotrophy
* 2-Drugs:Amiodarone, Antibiotics (dapsone, isoniazid, metronidazole( AntiretroviralsChemotherapy(cisplatin, vincristine(, Phenytoin
* 3-Toxins:Alcohol,Nitrous oxide Rarely: lead, arsenic, mercury, organophosphates, solvents
* 4-Vitamin deficiencies:Thiamin, Pyridoxine, Vitamin B12, Vitamin E
* 5-Infections: HIV, Leprosy, Brucellosis
* 6-Inflammatory:Guillain–Barré syndrome, Vasculitis (polyarteritis nodosa, Wegener’s granulomatosis), rheumatoid arthritis, systemic lupus erythematous) Paraneoplastic
* 7-Systemic medical conditions:Diabetes, Renal failure, Sarcoidosis
* 8-Malignant disease: Infiltration
* 9-Others:Paraproteinaemias, Amyloidosis
Myasthenia Graves
- Def: Its progressive neuromuscular junction disorder due to ( effect on receptor). Autoimmune: IgG 80% 1Female>Male.
*(20-40yrs) mainly affect two age group 15- 50yrs & >60yrs 2+ve F/h/ HLAB8 B/Thymic Abnormality: ASMA 15% Prognosis: Recovery in 3-6 months - Thymoma- Thymic hyperplasia.
- Drug Induced 5% (Transient): D-pencilamine C/P: Muscle weakness characteristic: at end of day.After repeated ms work exercise R & R Course. Use of some drugs (Aminoglycosides, Ciprofloxacin, Propranolol & Quinine). Muscle affects: 1/Extralocular ms + Levetor palpebral superiors ms Diplopia & Ptosis. 2/Bulbar ms Dysphagia. *(Aspiration Pneumonia) 3/Shoulder Gridle proximal ms weakness of UL (weakness of combing hair). 4/Pelvic Gridle weakness on climbing stairs & arising from sitting position.
- Trunk. 6/Respiratory ms RFII N.B: there is no sensory loss & complaining of Ms pain. Inv: 1/Edrophonium test: (Tension test) short acting. Improve symptoms within 30sec (best test). 2/EMG: decremental response. 3/Ab: Anticholine receptor Ab (80%) =Screening for other autoimmune.
Treatment: 1/Pyridostigmine (Long Acting Anticholinesterase) DOC. 2 Thymoma +ve CT or CXR ve Ab Autoimmune Resection =Thymectomy.(60% improvement) Steroid. 3/Immunoglobulin. 4/Plasmaphresis or exchange. life threating due to respiratory failure type II, sudden Rapid progressive weakness especially respiratory ms. Severe dyspnea Treated by Emergency Intubation & Mechanical ventilation + plasma