Showing posts with label Internal Medicine. Show all posts
Showing posts with label Internal Medicine. Show all posts
Medicos-Hub: Current Medical Diagnosis and Treatment 2011 (CMDT...
Medicos-Hub: Current Medical Diagnosis and Treatment 2011 (CMDT...: click here to download After downloading, unzip file with 7-zip file manager (for windows) or X-plorer/Zip manager (for mobile). Enjoy...!...
Current Medical Diagnosis and Treatment 2011 (CMDT)
click here to download After downloading, unzip file with 7-zip file manager (for windows) or X-plorer/Zip manager (for mobile).
Enjoy...!!!
Dont forget to post your comments. It helps me to serve you better.
Enjoy...!!!
Dont forget to post your comments. It helps me to serve you better.
MOTOR NEURON DISEASE
Aetiology is unknown. There is loss of motor neurons and glimpis in motor, motor nuclei of brainstem and anterior horn of spinal cord, with degeneration of costicospinal tract in spinal cord.
Classification
heriditary
1. Werdnig-Hoffmann disease(infantile spinal muscular atrophy).
2. Kugelberg- welander disease(adolescent spinal muscular atrophy)
sporadic
1.Amyotrophic lateral sclerosis
2. Progressive muscular atrophy.
3. Progressive bulbar palsy.
4. Primary lateral sclerosis.
Amyotrophic lateral sclerosis.
It is the most common form of progressive motor neuron disease. It is prime example of a neurodegenerative disease and is arguably the most devastating of the neurodegenerative disorder.
Pathology.
The pathologic hallmark of amyotropic lateral sclerosis is involvement of both upper and lower motor neurons.
The affected motor neurons undergo shrinkage wit accumulation of the pigment lipofuscin..Also there occurs proliferation of astroglia and microglia. In ALS, the motor neuron cytoskeleton is typically affected early in the illness..
The death of peripheral motor neurons leads to denervation and consequent atrophy of the corresponding muscle fibre. This is basis for the term 'amyotrophy'. Loss of costical motor neurons results in thinning of the costicospinal tract. This loss of fibres in tin lateral column and resulting fibrillary gliosis impart firmness. Hence the term lateral sclerosis.
The most remarkable feature is the selectivity of neuronck celll death. The entire sensory apparatus, the regulatory mechanism for the control and coordination of movement, and the components of the brain that are needed for cognitive processes, remain intact.
Thus motor neurons required for occular movili remains unaffected, as do the parasympathetic neurons in the sacral spinal cord that honesta the sphincters of bowel and bladder.
Clinical manifestations.
1. Weakness caused by denervation is associated with progressive wasting and atrophy of muscles, and particularly early in the illness, spontaneous twitching of motor units, or fasciculations.
2.In hands weakness is more in extensors as compared to flexors.
3. When initial involvement is that of bulbar neuron there is difficulty with chewing, swallowing, and movements of tongue and face.
4. With prominent costicospinal involvement, there is hyperactivity of the tendon reflexes and spartia resistance to passive movements of affected limbs..
5. Degeneration of corticobulbar projections honesta the brainstem results in dysarthria and exaggeration of the motor expressions of emotion. This results in involuntsy excess in weeping or laughing.
6. Even in the late stages of the illnesss, sensory, bowel and bladder, and cognitive functions are preserved.
Diagnostic guidelines forALS by committe of the World Federation of Neurology.
Motor neurons of
1 bulbar
2. Cervical
3. Thoraccic
4. Lumbosacral
can be involved.
The disorder is termed 'definite' when three or four of above are involved.
Probable when two sites are involved.
Possible when only one site is affected.
Essential for the diagnosis is simultaneous involvement of upper and lower motor neuron.
Investigations
1. Spine radiology
2. EMG
3. Nerve conduction velocity.
4. MRI of spine.
5. Thyroid function test.
6. Lumbar puncture.
Differential diagnosis.
1 tumors in the cervical region or at foramen magnum.
2. Cervical spondylosis.
3.multifocal motor neuropathy with conduction block.
Treatment
1. No treatment arrest the underlying pathologic process in ALS.
2. Riluzole 100mg/d produces a modest lengthning of survival.
It is a sodium channel blocker that inhibits glutamate release.
3. Walking aids, respiratory support and physiotherapy are helpful
ACUTE RHEUMATIC FEVER (Harrison)
Acute Rheumatic Fever
· Multisystem disease resulting from an autoimmune reaction to infection with
group A streptococcus.
· Cardiac valvular damage [rheumatic heart disease (RHD)
.
Epidemiology
· children aged 5–14 years
· RHD more commonly affects females, sometimes up to twice as frequently as
males.
Pathogenesis
.
Cross-reactive epitopes are present in the streptococcal M protein and the Nacetylglucosamine
of group A streptococcal carbohydrate and are immunologically
similar to molecules in human myosin, tropomyosin, keratin, actin, laminin,
vimentin, and N-acetylglucosamine..
Clinical Features
· latent period - 3 weeks (1–5 weeks
· The most common clinical presentation of ARF is polyarthritis and fever.
Polyarthritis ---- 60–75% of cases and
carditis ----- 50–60%.
chorea -------- <2% to 30%.
Erythema marginatum and subcutaneous nodules <5% of cases.
Heart Involvement
Valvular damage is the hallmark of rheumatic carditis. mitral valve > aortic valve;
Early valvular damage leads to regurgitation. Over ensuing years, usually as a result of
recurrent episodes, leaflet thickening, scarring, calcification, and valvular stenosis may
develop Myocardial inflammation may affect electrical conduction pathways, leading
to P-R interval prolongation (first-degree AV block or rarely higher-level block) and
softening of the first heart sound.
2
Joint Involvement
· inflammation, with hot, swollen, red and/or tender joints,
· and involvement of more than one joint (i.e., polyarthritis)
· migratory,
· large joints—most commonly the knees, ankles, hips, and elbows
· —and is asymmetric.
· The pain is severe and usually disabling until anti-inflammatory medication is
commenced.
· highly responsive to salicylates and other nonsteroidal anti-inflammatory drugs
(NSAIDs).
Indeed, joint involvement that persists more than 1 or 2 days after starting salicylates is
unlikely to be due to ARF. Conversely, if salicylates are commenced early in the illness,
before fever and migratory polyarthritis have become manifest, it may be difficult to
make a diagnosis of ARF. For this reason, salicylates and other NSAIDs should be
withheld—and pain managed with acetaminophen or codeine—until the diagnosis is
confirmed.
Sydenham's Chorea
· mainly in females.
· The choreiform movements affect particularly the head (causing characteristic
darting movements of the tongue) and the upper limbs
· Chorea eventually resolves completely, usually within 6 weeks.
Skin Manifestations
· erythema marginatum, which begins as pink macules that clear centrally,
leaving a serpiginous, spreading edge. The rash is evanescent, appearing and
disappearing before the examiner's eyes. It occurs usually on the trunk,
sometimes on the limbs, but almost never on the face.
· Subcutaneous nodules occur as painless, small (0.5–2 cm), mobile lumps
beneath the skin overlying bony prominences, particularly of the hands, feet,
elbows, occiput, and occasionally the vertebrae. They are a delayed
manifestation, appearing 2–3 weeks after the onset of disease, last for just a few
days up to 3 weeks, and are commonly associated with carditis.
Other Features
1. Fever
2. . Although high-grade fever ( 39°C) is the rule, lower grade temperature
elevations are not uncommon.
3. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) are often
dramatically elevated. Occasionally the peripheral leukocyte count is mildly
3
elevated.
4. Evidence of a Preceding Group a Streptococcal Infection
5. preceding group A streptococcal infection is essential in making the diagnosis
of ARF. The most common serologic tests are the anti-streptolysin O (ASO)
and anti-DNase B (ADB) titers. Other Post-Streptococcal Syndromes that May
Be Confused with Rheumatic Fever
Post-streptococcal reactive arthritis (PSRA) is differentiated from ARF on the basis
of:
(1) small-joint involvement that is often symmetric;
(2) a short latent period following streptococcal infection (usually <1 week);
(3) occasional causation by nongroup A -hemolytic streptococcal infection;
(4) slower responsiveness to salicylates; and
(5) the absence of other features of ARF, particularly carditis.
Pediatric autoimmune neuropsychiatric disorders associated with streptococcal
infection (PANDAS) is a term that links a range of tic disorders and obsessivecompulsive
symptoms with group A streptococcal infections. People with PANDAS are
said not to be at risk of carditis, unlike patients with Sydenham's chorea. The diagnoses
of PANDAS and PSRA should rarely be made in populations with a high incidence of
ARF.
Confirming the Diagnosis
Diagnostic Categories Criteria
Primary episode of rheumatic fevera · Two major plus evidence of preceding
group A streptococcal infection
· or one major and two minor
manifestations plus evidence of
preceding group A streptococcal
infection
Recurrent attack of rheumatic fever
in a patient without established
rheumatic heart disease
· Two major plus evidence of preceding
group A streptococcal infection
· or one major and two minor
manifestations plus evidence of
preceding group A streptococcal
infection
Major manifestations Joints (polyarthritis)
Obvious Carditis
Erythema marginatum
Nodules (Subcutaneous )
Sydneham’s Chorea
4
Minor manifestations Prolonged P-R interval
Elevated erythrocyte sedimentation rate or
leukocyte counte
Arthralgia(poly)
CRP Raised
Elevated temperature
Supporting evidence of a preceding
streptococcal infection within the last
45 days
Elevated or rising anti-streptolysin O or other
streptococcal antibody, or
A positive throat culture, or
Rapid antigen test for group A streptococcus,
or
Recent scarlet fevere
Treatment: Acute Rheumatic Fever
Table 322-2 Recommended Tests in Cases of Possible Acute Rheumatic Fever
Recommended for all cases
· White blood cell count
· Erythrocyte sedimentation rate
· C-reactive protein
· Blood cultures if febrile
· Electrocardiogram (repeat in 2 weeks and 2 months if prolonged P-R interval
or other rhythm abnormality)
· Chest x-ray if clinical or echocardiographic evidence of carditis
· Echocardiogram (consider repeating after 1 month if negative)
· Throat swab (preferably before giving antibiotics)–culture for group A
streptococcus
· Anti-streptococcal serology: both anti-streptolysin O and anti-DNase B titres,
if available (repeat 10–14 days later if 1st test not confirmatory)
Tests for alternative diagnoses, depending on clinical features
5
· Repeated blood cultures if possible endocarditis
· Joint aspirate (microscopy and culture) for possible septic arthritis
· Copper, ceruloplasmin, anti-nuclear antibody, drug screen for choreiform
movements
· Serology and auto-immune markers for arboviral, auto-immune or reactive
arthritis
treatment of ARF is symptomatic.
1. Antibiotics
· Penicillin is the drug of choice and can be given orally
· [as phenoxymethyl penicillin, 500 mg (250 mg for children 27 kg) PO twice
daily,
· or amoxicillin 50 mg/kg (max 1 g) daily, for 10 days]
· or as a single dose of 1.2 million units (600,000 units for children 27 kg) IM
benzathine penicillin G.
2. Salicylates and NSAIDs
for the treatment of arthritis, arthralgia, and fever, once the diagnosis is confirmed.
They are of no proven value in the treatment of carditis or chorea.
Aspirin is the drug of choice.
An initial dose of 80–100 mg/kg per day in children (4–8 g/d in adults) in 4–5 divided
doses is often needed for the first few days up to 2 weeks. the dose can be reduced to
60–70 mg/kg per day for a further 2–4 weeks. Although less well studied, naproxen at a
dose of 10–20 mg/kg per day has been reported to lead to good symptomatic response.
3. Congestive Heart Failure
Glucocorticoids
If used, prednisone or prednisolone are recommended at doses of 1–2 mg/kg per day
(maximum, 80 mg). Glucocorticoids are often only required for a few days or up to a
maximum of 3 weeks.
4. Management of Heart Failure
See Chap. 234.
6
5. Bed Rest
When arthritis and arthralgia are present, and for patients with heart failure..
6. Chorea
· Carbamazepine
· or sodium valproate
7. Intravenous Immunoglobulin (Ivig)
may lead to more rapid resolution of chorea
not recommended except in cases of severe chorea refractory to other treatments.
Prognosis
Untreated, ARF lasts on average 12 weeks. With treatment, patients are usually
discharged from hospital within 1–2 weeks. Inflammatory markers should be monitored
every 1–2 weeks until they have normalized (usually within 4–6 weeks), and an
echocardiogram should be performed after 1 month to determine if there has been
progression of carditis. Cases with more severe carditis need close clinical and
echocardiographic monitoring in the longer term.
Secondary Prevention
· The best antibiotic for secondary prophylaxis is benzathine penicillin G (1.2
million units, or 600,000 units if 27 kg) delivered every 4 weeks. It can be
given every 3 weeks, or even every 2 weeks, to persons considered to be at
particularly high risk, although in settings where good compliance with 4-
weekly dosing can be achieved, more frequent dosing is rarely needed.
· Oral penicillin V (250 mg) can be given twice-daily instead but is somewhat
less effective than benzathine penicillin G.
· Penicillin allergic patients can receive erythromycin (250 mg) twice daily.
Category of Patient Duration of Prophylaxis
Rheumatic fever without carditis For 5 years after the last attack or 21 years
of age (whichever is longer)
Rheumatic fever with carditis but no
residual valvular disease
For 10 years after the last attack, or 21
years of age (whichever is longer)
Rheumatic fever with persistent valvular
disease, evident clinically or on
echocardiography
For 10 years after the last attack, or 40
years of age (whichever is longer).
Sometimes lifelong prophylaxis.
7
Copyright ©
· The McGraw-Hill Companies.
· Dr.Shami Bhagat (SKIMS)
· All rights reserved.
· Multisystem disease resulting from an autoimmune reaction to infection with
group A streptococcus.
· Cardiac valvular damage [rheumatic heart disease (RHD)
.
Epidemiology
· children aged 5–14 years
· RHD more commonly affects females, sometimes up to twice as frequently as
males.
Pathogenesis
.
Cross-reactive epitopes are present in the streptococcal M protein and the Nacetylglucosamine
of group A streptococcal carbohydrate and are immunologically
similar to molecules in human myosin, tropomyosin, keratin, actin, laminin,
vimentin, and N-acetylglucosamine..
Clinical Features
· latent period - 3 weeks (1–5 weeks
· The most common clinical presentation of ARF is polyarthritis and fever.
Polyarthritis ---- 60–75% of cases and
carditis ----- 50–60%.
chorea -------- <2% to 30%.
Erythema marginatum and subcutaneous nodules <5% of cases.
Heart Involvement
Valvular damage is the hallmark of rheumatic carditis. mitral valve > aortic valve;
Early valvular damage leads to regurgitation. Over ensuing years, usually as a result of
recurrent episodes, leaflet thickening, scarring, calcification, and valvular stenosis may
develop Myocardial inflammation may affect electrical conduction pathways, leading
to P-R interval prolongation (first-degree AV block or rarely higher-level block) and
softening of the first heart sound.
2
Joint Involvement
· inflammation, with hot, swollen, red and/or tender joints,
· and involvement of more than one joint (i.e., polyarthritis)
· migratory,
· large joints—most commonly the knees, ankles, hips, and elbows
· —and is asymmetric.
· The pain is severe and usually disabling until anti-inflammatory medication is
commenced.
· highly responsive to salicylates and other nonsteroidal anti-inflammatory drugs
(NSAIDs).
Indeed, joint involvement that persists more than 1 or 2 days after starting salicylates is
unlikely to be due to ARF. Conversely, if salicylates are commenced early in the illness,
before fever and migratory polyarthritis have become manifest, it may be difficult to
make a diagnosis of ARF. For this reason, salicylates and other NSAIDs should be
withheld—and pain managed with acetaminophen or codeine—until the diagnosis is
confirmed.
Sydenham's Chorea
· mainly in females.
· The choreiform movements affect particularly the head (causing characteristic
darting movements of the tongue) and the upper limbs
· Chorea eventually resolves completely, usually within 6 weeks.
Skin Manifestations
· erythema marginatum, which begins as pink macules that clear centrally,
leaving a serpiginous, spreading edge. The rash is evanescent, appearing and
disappearing before the examiner's eyes. It occurs usually on the trunk,
sometimes on the limbs, but almost never on the face.
· Subcutaneous nodules occur as painless, small (0.5–2 cm), mobile lumps
beneath the skin overlying bony prominences, particularly of the hands, feet,
elbows, occiput, and occasionally the vertebrae. They are a delayed
manifestation, appearing 2–3 weeks after the onset of disease, last for just a few
days up to 3 weeks, and are commonly associated with carditis.
Other Features
1. Fever
2. . Although high-grade fever ( 39°C) is the rule, lower grade temperature
elevations are not uncommon.
3. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) are often
dramatically elevated. Occasionally the peripheral leukocyte count is mildly
3
elevated.
4. Evidence of a Preceding Group a Streptococcal Infection
5. preceding group A streptococcal infection is essential in making the diagnosis
of ARF. The most common serologic tests are the anti-streptolysin O (ASO)
and anti-DNase B (ADB) titers. Other Post-Streptococcal Syndromes that May
Be Confused with Rheumatic Fever
Post-streptococcal reactive arthritis (PSRA) is differentiated from ARF on the basis
of:
(1) small-joint involvement that is often symmetric;
(2) a short latent period following streptococcal infection (usually <1 week);
(3) occasional causation by nongroup A -hemolytic streptococcal infection;
(4) slower responsiveness to salicylates; and
(5) the absence of other features of ARF, particularly carditis.
Pediatric autoimmune neuropsychiatric disorders associated with streptococcal
infection (PANDAS) is a term that links a range of tic disorders and obsessivecompulsive
symptoms with group A streptococcal infections. People with PANDAS are
said not to be at risk of carditis, unlike patients with Sydenham's chorea. The diagnoses
of PANDAS and PSRA should rarely be made in populations with a high incidence of
ARF.
Confirming the Diagnosis
Diagnostic Categories Criteria
Primary episode of rheumatic fevera · Two major plus evidence of preceding
group A streptococcal infection
· or one major and two minor
manifestations plus evidence of
preceding group A streptococcal
infection
Recurrent attack of rheumatic fever
in a patient without established
rheumatic heart disease
· Two major plus evidence of preceding
group A streptococcal infection
· or one major and two minor
manifestations plus evidence of
preceding group A streptococcal
infection
Major manifestations Joints (polyarthritis)
Obvious Carditis
Erythema marginatum
Nodules (Subcutaneous )
Sydneham’s Chorea
4
Minor manifestations Prolonged P-R interval
Elevated erythrocyte sedimentation rate or
leukocyte counte
Arthralgia(poly)
CRP Raised
Elevated temperature
Supporting evidence of a preceding
streptococcal infection within the last
45 days
Elevated or rising anti-streptolysin O or other
streptococcal antibody, or
A positive throat culture, or
Rapid antigen test for group A streptococcus,
or
Recent scarlet fevere
Treatment: Acute Rheumatic Fever
Table 322-2 Recommended Tests in Cases of Possible Acute Rheumatic Fever
Recommended for all cases
· White blood cell count
· Erythrocyte sedimentation rate
· C-reactive protein
· Blood cultures if febrile
· Electrocardiogram (repeat in 2 weeks and 2 months if prolonged P-R interval
or other rhythm abnormality)
· Chest x-ray if clinical or echocardiographic evidence of carditis
· Echocardiogram (consider repeating after 1 month if negative)
· Throat swab (preferably before giving antibiotics)–culture for group A
streptococcus
· Anti-streptococcal serology: both anti-streptolysin O and anti-DNase B titres,
if available (repeat 10–14 days later if 1st test not confirmatory)
Tests for alternative diagnoses, depending on clinical features
5
· Repeated blood cultures if possible endocarditis
· Joint aspirate (microscopy and culture) for possible septic arthritis
· Copper, ceruloplasmin, anti-nuclear antibody, drug screen for choreiform
movements
· Serology and auto-immune markers for arboviral, auto-immune or reactive
arthritis
treatment of ARF is symptomatic.
1. Antibiotics
· Penicillin is the drug of choice and can be given orally
· [as phenoxymethyl penicillin, 500 mg (250 mg for children 27 kg) PO twice
daily,
· or amoxicillin 50 mg/kg (max 1 g) daily, for 10 days]
· or as a single dose of 1.2 million units (600,000 units for children 27 kg) IM
benzathine penicillin G.
2. Salicylates and NSAIDs
for the treatment of arthritis, arthralgia, and fever, once the diagnosis is confirmed.
They are of no proven value in the treatment of carditis or chorea.
Aspirin is the drug of choice.
An initial dose of 80–100 mg/kg per day in children (4–8 g/d in adults) in 4–5 divided
doses is often needed for the first few days up to 2 weeks. the dose can be reduced to
60–70 mg/kg per day for a further 2–4 weeks. Although less well studied, naproxen at a
dose of 10–20 mg/kg per day has been reported to lead to good symptomatic response.
3. Congestive Heart Failure
Glucocorticoids
If used, prednisone or prednisolone are recommended at doses of 1–2 mg/kg per day
(maximum, 80 mg). Glucocorticoids are often only required for a few days or up to a
maximum of 3 weeks.
4. Management of Heart Failure
See Chap. 234.
6
5. Bed Rest
When arthritis and arthralgia are present, and for patients with heart failure..
6. Chorea
· Carbamazepine
· or sodium valproate
7. Intravenous Immunoglobulin (Ivig)
may lead to more rapid resolution of chorea
not recommended except in cases of severe chorea refractory to other treatments.
Prognosis
Untreated, ARF lasts on average 12 weeks. With treatment, patients are usually
discharged from hospital within 1–2 weeks. Inflammatory markers should be monitored
every 1–2 weeks until they have normalized (usually within 4–6 weeks), and an
echocardiogram should be performed after 1 month to determine if there has been
progression of carditis. Cases with more severe carditis need close clinical and
echocardiographic monitoring in the longer term.
Secondary Prevention
· The best antibiotic for secondary prophylaxis is benzathine penicillin G (1.2
million units, or 600,000 units if 27 kg) delivered every 4 weeks. It can be
given every 3 weeks, or even every 2 weeks, to persons considered to be at
particularly high risk, although in settings where good compliance with 4-
weekly dosing can be achieved, more frequent dosing is rarely needed.
· Oral penicillin V (250 mg) can be given twice-daily instead but is somewhat
less effective than benzathine penicillin G.
· Penicillin allergic patients can receive erythromycin (250 mg) twice daily.
Category of Patient Duration of Prophylaxis
Rheumatic fever without carditis For 5 years after the last attack or 21 years
of age (whichever is longer)
Rheumatic fever with carditis but no
residual valvular disease
For 10 years after the last attack, or 21
years of age (whichever is longer)
Rheumatic fever with persistent valvular
disease, evident clinically or on
echocardiography
For 10 years after the last attack, or 40
years of age (whichever is longer).
Sometimes lifelong prophylaxis.
7
Copyright ©
· The McGraw-Hill Companies.
· Dr.Shami Bhagat (SKIMS)
· All rights reserved.
GULLIAN BAIRRE SYNDROME
Gbs is d comonest acute polyneuropathy presumed to b autoimune.,afect people at any age,folows viral inf,after surgery or rabies immunization
pathology
monophasic mediatd inflamatory f peripheral n.Multifocal demylination result n conductn block,secondary axonal deg.
C/f
acute disorder,progres for few days,motor neuropathy,sensory findings minimum,distal paraesthesia,motor paralysis,absent reflexes,bifacial weaknes,resp paralysis,suden fluctuatn in bp n heart rate,blader inv rare
investigation
csf become abn aftr 1wk raised proteins,normal sugar,cell count upto 50 cells/mmc
nerve conductn velocity slow
acute intermitent porphyria
Rx
maintaine a b c
ventilate
monitor pulse bp cardiac activity
steroids
plasmapheresis
gama globulin 0.4g/kg/d for 5days
pathology
monophasic mediatd inflamatory f peripheral n.Multifocal demylination result n conductn block,secondary axonal deg.
C/f
acute disorder,progres for few days,motor neuropathy,sensory findings minimum,distal paraesthesia,motor paralysis,absent reflexes,bifacial weaknes,resp paralysis,suden fluctuatn in bp n heart rate,blader inv rare
investigation
csf become abn aftr 1wk raised proteins,normal sugar,cell count upto 50 cells/mmc
nerve conductn velocity slow
acute intermitent porphyria
Rx
maintaine a b c
ventilate
monitor pulse bp cardiac activity
steroids
plasmapheresis
gama globulin 0.4g/kg/d for 5days
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