Dr.

WC Foong Homework

1) What is narrow pulse pressure present in dengue shock syndrome?

● Pulse pressure less than ¼ of systolic blood pressure
● Let’s say
- SBP is 90mmHg
- DBP is 75mmHg
- ¼ of SBP= 22.5mmHg
- The pulse pressure is SBP(90) -DBP (75)= 15mmHg, hence the pulse pressure
is less than ¼ of SBP

2) Hypotonia ( the floppy child)

Peripheral nervous system Central nervous system

- Isolated motor delayed - GDD
- Normal reflexes/ diminished/ - Seizures
absent - Brisk reflexes
- Normal facies - Dysmorphism
- No muscles wasting, no
fasciculation

● Anatomical regions involved

- Anterior horn cells
- Peripheral nerve
- Neuromuscular junction
- myopathy
● Duchenne Muscular Dystrophy
● Dyskinesia cerebral palsy
- Wiggling
- Closer to tone & reflex
● Down syndrome
- Slanting palpebral fissures
- Epicanthal folds
- Midface hypoplasia
- Single transverse palmar
creases
- Sandal gap between the
toes
● Prader-willi syndrome
- Global developmental
delay
- Short stature
- Genital hypoplasia
- Failure to thrive due to
poor suckling

exception
# quadriplegic and diplegic cerebral palsy is hypertonia
# fixed flexion and extension deformity - spasticity
Increased in tone
 3) Differentiate between symmetrical and asymmetrical muscles weakness
- Explain the clinical features of each and compare and contrast

Symmetrical muscle weakness Asymmetrical muscle weakness

Guillain-barre syndrome (a.k.a acute postinfectious Poliomyelitis (Acute Flaccid Paralysis)

polyneuropathy)
Clinical presentation
Clinical presentation ● Pure motor deficit
● Preceded by URTI or AGE (caused by
campylobacter) Investigation
● 2-3 weeks post infection, there is an ascending, ● Need to send sample of stool for poliovirus
progressive symmetrical muscle weakness to confirm the diagnosis, this also act as a
● Bilateral facial weakness screening & surveillance test
● Impair bulbar muscles leads to difficulty with chewing
and swallowing Pathophysiology
● Anterior horn cells destruction
Physical sign
● Autonomic dysfunction
- tachycardia/ bradycardia
- Arrhythmias
- Hypertension
- Orthostatic hypotension
- Urinary retention
- Ileus
- Loss of sweating
● Loss of deep tendon reflex
● Loss of sensation over the distal limbs or trunk

Anatomical pathology
● Peripheral neuropathies
● Antibodies against myelin in the peripheral nervous
system

Investigation
● MRI to rule out spinal cord lesion
● Nerve conduction studies

Management
● Supportive

Myasthenia gravis

Clinical presentation
● Increased fatigability towards the end of the day
(diurnal worsening)
● Types
- Juvenile myasthenia
● Present after 10 years old
 ● Difficulty chewing
● Fatigability improve with rest or
anticholinergic drugs
- Congenital
● Respiratory problems due to
respiratory muscle weakness
● Multiple joints contractures
(arthrogryposis)
● May not responds to
anticholinesterase inhibitor

Physical sign
● Ptosis
● Ophthalmoplegia
● Loss of facial expression

Anatomical pathology
● Neuromuscular junction: neuromuscular transmission
disorder

Charcot- marie-tooth disease

Clinical manifestation
● Symmetrical, progressive distal muscle wasting

Pathophysiology
● Peripheral neuropathies
● Myelin gene mutations
● Inherited by autosomal dominant

Spinal muscular atrophy

Clinical manifestation
● Progressive, Symmetrical muscle weakness

Pathophysiology
● Anterior horn cells degeneration due to SMN 1 gene
mutations
● Autosomal recessive

4) Differentiate between proximal and distal muscle weakness

- Explain the clinical features of each and compare and contrast

Proximal muscle weakness (MYOPATHIES) Distal muscle weakness (NEUROPATHY)

Duchenne muscular dystrophy Guillain-barre syndrome

Clinical presentation Clinical presentation
● Delayed walking ● Ascending, progressive symmetrical
● Slower and clumsy weakness post URTI or AGE.
● Wheel-chair bound by 10- 14 years old onwards ● Affect the distal muscles first
● Difficulty getting up from sitting position
● Difficulty in climbing
● Tendency to falling
● Learning difficulties

Physical sign
● Waddling gait
● Gower sign where the patient turn prone to rise, this is
not normal after 3 years old
● Brachioradialis atrophy
● Pseudohypertrophy - falsely calf muscle enlarged
● Hypotonia, depressed reflex
● Scoliosis
● Respiratory system
- Respiratory failure
● Cvs system
- cardiomyopathy

Pathophysiology
● X-linked recessive
● Dystrophin gene mutation

Becker muscular dystrophy

Clinical presentation
● Age of onset: 11 years old
● Similar but Milder than Duchenne muscular dystrophy,
slower in progression

Pathophysiology
● Some degree of functional dystrophin

Spinal muscular atrophy Charcot-marie tooth disease (a.k.a hereditary

motor sensory neuropathies)
Clinical presentation Clinical presentation
● Proximal muscle weakness ● Progressive and symmetrical
● Earlier age of onset in type I (usually before 6 months ● Lower limb more affected than upper
of age) limb
● Type II around 15-18 months ● Difficulty walking & frequent tripping due
● Type III after 1 years old to foot and distal leg weakness
● Frequent ankle sprains

Physical sign Physical sign

 ● Proximal muscle wasting ● Hammertoes (Foot deformities) & High
arch (pes cavus)
● Inverted Champagne bottle like-
indicates distal muscle wasting
● Bilateral foot drop
● Loss of sensation
● Loss of knee reflexes

Congenital Muscular Dystrophy

Clinical features
● Muscle weakness at birth or early infancy
● Contractures
● Learning difficulties
● Some are static course
● AR

Congenital myopathy
Clinical features
● Muscle weakness at birth or in infancy
● Static or slowly progressive

Inflammatory polymyositis/ dermatomyositis

Myasthenia gravis
Clinical features
● Generalized weakness
● Affected facial muscles > proximal muscle > distal
muscle

5) Differential diagnosis of upper motor neuron lesion and lower motor neuron lesion

UMN LMN
● Hypertonia ● May have fasciculation
● Muscle weakness ● Muscle wasting (loss of muscle
● Brisk hyperreflexia bulk)
● Corticospinal tract disorder ● Hypotonia
- Hip abducted (frog- leg
sign)
● Poor antigravity movement
● hypo/ absent of deep tendon
reflexes

Cerebral palsy Guillain-barre syndrome

Traumatic brain injury Spinal muscular atrophy

Spina bifida
 ● Depends on the level of spinal
cord lesion
● Severe form Usually affect the L3

Clinical presentation
● Lower limb weakness
● Urinary and fecal incontinence

Physical sign
● Sacral pit/ dimple/ tuft of hair
● myelomeningocele/ meningocele
● Talipes equinovarus
● Dislocated hip (hip shortened,
adducted, internal rotation)

6) Compare and contrast Peripheral neuropathy vs Myopathy

Peripheral neuropathy Myopathy

- Tend to affect the distal muscles - Tend to lead to PROXIMAL
first muscle weakness
- EXCEPT Myasthenia gravis that
affect the proximal muscles more
than distal muscles

Anterior horn cells ● duchenne/ becker/ congenital

● SMA dystrophy
● Poliomyelitis ● Benign acute myositis
● polymyositis/ dermatomyositis
● Dystrophia myotonica
● Metabolic myopathies
● Congenital myopathies

Peripheral nerve
● Charcot marie tooth disease
● GBS

Neuromuscular junctions
● Myasthenia gravis
7) How does Oral rehydration salt work?
- Oral rehydration salt consists of sodium and glucose
- It works by increased absorption of water and solutes to keep patient
hydrated
- Active pumping of sodium out of the epithelial cells to the intestinal lumen
via na+- K+ ATPase
- This in turn allow facilitated diffusion of glucose via sodium glucose
transporter from the intestinal lumen to the epithelial cells and finally to
the interstitial space and the blood
- Glucose acts as solute to drag water from the intestinal lumen and finally
to the blood

-
 8) Nephrotic syndrome
- Lab values of importance
● Nephrotic-range proteinuria is defined as the urinary loss of 3 grams or
more of proteins per 24 hours or, on a single spot urine sample, the
presence of 2 g of protein per gram of urinary creatinine.
- Triad of post-glomerulonephritis
● Acute nephritic syndrome
● Low C3 level
● Recent streptococcus infection
Age group, presentation, cause of edema, features, management

Age presentation Cause of edema management

group

2- 5 Nephrotic syndrome ● High dose corticosteroid

● Periorbital edema (worse in the ● Hypoalbuminemia (prednisolone) from
morning) 60mg/m2, reduce to
● Improved at the end of the day 40mg/m2, taper slowly to
● Generalized swelling Primary wean off
- Scroll and vulva ● idiopathic ● IV serum albumin
- Ankle edema - Minimal replacement
- Sacral edema change ● Antibiotics (for prophylaxis)
- Abdominal distension disease
● Frothy urine
Secondary
● Henoch-schonlein
Complication purpura
● Pleural effusion ● SLE
● Abdominal pain. TRO ● Malaria
Spontaneous bacterial peritonitis ● Allergens (bee sting)
● Hypercoagulability due to
intravascular dehydration

Congestive cardiac failure

● Fluid overload
● Pulmonary edema

Malnutrition
● kwashiorkor

myxedema hypothyroidism

Clinical features Glomerulonephritis ● Maintain water & electrolyte

● Tea-colored urine ● Post-streptococcal balance
● Skin rash infection ● Monitor renal function test
 - Non-blanching purpura on ● Henoch-schonlein
extensor surface purpura
especially at buttock, ● SLE
arms, legs and ankles
- Butterfly rash on the face
● Joint pain
● Abdominal pain

Physical signs
● Decreased urine output
● Hypertension
● Hematuria
- > 10 RBC per high power
field
● Mild Proteinuria

jaundice Liver cirrhosis

2- 10 Henoch-scholein purpura ● Complications: Nephritis

● Non-blanching syndrome
purpuric rash on ● No specific treatment,
extensor surface supportive therapy for rash,
● Arthralgia arthralgia, fever and malaise

Lab investigation - what is normal and abnormal level of albumin

● Nephrotic syndrome
● Urine sample
- Urine analysis
- UFME
- Urine protein to creatinine ratio (measure from early
morning urine sample)
● Normal is < 20mg/mmol
● Blood albumin level
- Normal 40 mg/dL
- Nephrotic < 20-25mg/dL
Intravascular volume depletion (fluid management)?
● Treat the nephrotic syndrome
- Steroid like prednisolone
● Minimal change nephrotic syndrome
● Should respond to prednisolone
● If not responding- inform the specialist
- Atypical nephrotic syndrome- IV albumin
AP. SC Foong Homework
Differential diagnosis of Facial nerve palsy
- Bell’s palsy
● Peripheral neuropathies
● Isolated LMN paresis of CN VII
● Affect the whole one sided face including the eyebrow
- Ramsay hunt syndrome/ herpes zoster oticus
- triad of ipsilateral facial paralysis, otalgia, and vesicles near the ear
and auditory canal.
● Inflammation of geniculate ganglion of cranial nerve VII
 ●

●
- Forcep delivery injury
● damaged just before or at the time of delivery
● Generally mild injury, self- limited within a few months, with
favorable prognosis
● involves the lower motor neuron
● So ipsilateral face affected from the forehead to the chin
- Otitis media
- Facial nerve palsies from acute and chronic otitis media have a
more gradual onset, with accompanying ear pain and fever.
- Alterations in the middle ear microenvironments, such as elevated
pressure, osteitis, or acute inflammation, wherein facial nerve
physiology may be directly affected (swelling or direct pressure on
the nerve)
- It is possibly a result of the inflammatory response within the
fallopian canal to the acute or chronic otitis media
- Tympanic segment is the most common site to be involved
- Treatment :
- Acute otitis media and acute mastoiditis (cortical
mastoidectomy +ventilation tube)
- chronic otitis media with cholesteatoma ( mastoidectomy ±
facial nerve decompression )
Facial nerve palsy upper or lower motor neuron lesion

UMN LMN

Central supranuclear lesion Bell’s palsy

Forcep delivery injury

- Ramsay hunt syndrome/

herpes zoster oticus