neuromuscular disease 神经系统疾病 neuromuscular disease(1) neurological examination...

Neuromuscular Disease

神经系统疾病

Neuromuscular Disease(1)

Neurological Examination Infections of the CNS

Department of Pediatrics

Soochow University Affiliated Children’s Hospital


神经系统疾病

Section 1. Neurological Examination (P123)

Main contents: four divisions

1. Introduce the general examination of

neurological system.

2. Introduce the examination of the cranial

nerves.

3. Emphasize the motor examination.

4. Introduce the sensory examination.


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Topic 1. General examination

1.1 level of consciousness consciousness

unconsciousness:

□sleepy (drowsy).

□stupor (lethargy).

□coma.

complicating abnormal mental condition

□confusion.

□delirium.


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1.2 head □head shape, head circumference microcephaly.

hydrocephalus. cephalus quadratus.

malformation of CNS. □fontanelle

a bulging fontanelle is indicative of raised intracranial pressure.


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hydrocephal

us

Microcephaly


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a bulging fontanelleis indicative of raised intracranial pressure.


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1.3 Skin: neurocutaneous stigmata

neurofibromatosis

tuberous sclerosis


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The general examination of neurological system

emphasize:

□level of consciousness.

□head.

□skin.


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Topic 2. The cranial nerves

1. first cranial nerve (olfactory nerve).

2. second nerve (optical nerve).

3. third nerve (oculomotor nerve).

4. fourth nerve (trochlear nerve).

5. fifth nerve (trigeminal nerve).

6. sixth nerve (abducent nerve).

7. seventh nerve (facial nerve).

8. eighth nerve (vestibulocochlear nerve).

9. ninth nerve (glossopharyngeal nerve).

10. tenth nerve (vagus nerve).

11. eleventh nerve (accessory nerve).

12. twelfth nerve (hypoglossal nerve).


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base of skull


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The first cranial nerve (olfactory nerve)

□ rarely examined in children.

□ assessed by using smelling agents (e.g.

cigarette, soap).

□ possible by asking direct questions about the

child’s sense of smell.


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The second nerve (optical nerve) □vision:

testing by covering one eye and asking the older child to read picture cards.

□visual field:

using a small toy or finger with the child focusing on your nose.

□fundus of eye:

testing by funduscopy.


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third nerve (oculomotor nerve)

fourth nerve (trochlear nerve)

sixth nerve (abducent nerve)

□testing three pair of nerves together.

□steadying the child’s head

while asking him to follow

with his eyes a bright object or finger

traversing a large ‘H’.


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The fifth nerve (trigeminal nerve)

□motor component:

requires the child to

push his jaw against your hand.

□motor component lesion:

drawing of the jaw to

the abnormal side on gaping.


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The fifth nerve (trigeminal nerve)

□sensory component:

asking the child to close his

eyes and say ‘yes’ every time

you touch the three divisions

(ophthalmic, maxillary and mandibular).

□the corneal reflex test.


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The seventh nerve (facial nerve)

seventh-nerve palsy

□facial asymmetry with loss of nasolabi

al folds and drawing of the face to t

he normal side.

□the child shouldn’t be able to blow out

the cheeks and close the mouth tigh

tly.


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The seventh nerve(facial nerve)seventh-nerve palsy

□lower motor neuron lesion: loss of the forehead creases; the child doesn’t close the eye tightly.□upper motor neuron lesion: preservation of the forehead creases; the child should be able to close the eye tightly.


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The eighth nerve

(vestibulocochlear nerve)

□checked by direct questions

relating to hearing.

□around 3~4 months of age:

the child should be able to

turn head to sound source,

and smile with sweet sounding.


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ninth nerve (glossopharyngeal nerve)

tenth nerve (vagus nerve)

□functioning normally:

the uvula rises symmetrically

when the child says ‘aah’.

□the nerves lesion:

the uvula rises asymmetrically

when the child says ‘aah’.


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The eleventh nerve (accessory nerve)eleventh nerve lesion the child doesn’t raise his shouldersand push his head against resistance.


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The twelfth nerve (hypoglossal nerve)

□asking the child to protrude

and waggle the tongue from side to side.

□the nerves lesion:

drawing of the tongue to

the abnormal side on protruding.


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Nerve test (Cranial nerve assessment in infants)

Ⅰ not tested.

Ⅱ blinks to bright light;

appears to fix/follow(3+months).

Ⅲ- assess highlights on pupils for squint;Ⅵ abnormal position of globe.

Ⅴ corneal reflexes; sucking movements.

Ⅶ facial asymmetry especially while crying.

Ⅷ startles to sound; quietens with soothing.

Ⅸ- gag reflex.Ⅹ Ⅺ not tested.

Ⅻ tongue movement.


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Topic 3. Motor examination

3.1 Muscle tone

□assessed by observing osture and moving joints.

□Hypotonicity: a frog-leg; floppy posture.

□Spasticity: increased tone

that relaxes suddenly (clasp-knife).

□Rigidity: constant resistance

to passive movement.


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3.2 Muscle power: assessed by inviting the child to perform some functions against resistance.Power Observation (Grading of muscle power from 0 to 5)

0 No power.

1 Barely perceptible power

without gravity removed horizontally.

2 Movement with gravity removed horizontally

doesn’t overcome gravity (e.g. lift off the bed) .

3 Movement just overcomes gravity.

4 Movement against gravity and some resistance.

5 Normal power.


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3.3 Reflexes

□assessed by using a finger in neonates;

a small hammer in older hildren

(knee reflex test).

□Plantar reflexes are not downgoing until the child is walking.

□Pathologic reflexe: Babinski sign.


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inborn reflex

Moro reflex

Grasp reflex

Sucking reflex

Rooting reflex


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3.4 Mobility and gait (e.g. cerebral palsy)

□writhing actions (athetosis).

□abnormal movements (scissors gait).


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3.5 Speech □speech sometimes affected

in motor disorders.

□cerebellar abnormalities:

1) poor coordination.

2) intention tremor.

3) dysdiadochokinesia.

4) pendular nystagmus.

5) hypotonia.

6) staccato speech.


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Topic 4. Sensory examination

Sensory assessment often proves

the most difficult part of the neurological examination.

4.1 superficial sensation □pain sensation: tested with a needle to assess.

□touch sensation: tested with a piece of cotton.

□temperature sensation: using warm and cold tubes.

by asking the child to identify sensation of the skin.

4.2 deep sensation □vibration sense: tested with a tuning fork.

□position sense: by asking the child to identify positions

of the thumbs and big toes with the eyes closed.


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Section 2.Infections of the CNS (p128)


神经系统疾病

Section 2.Infections of the CNS (p128)

Main contents: four divisions 1.bacterial meningitis the causes, clinical features, complication, diagnosis and management.2.encephalitis the causes and clinical features.3.viral meningitis the clinical features.4.brain abscess the clinical features.


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Topic 1. Bacterial meningitis

Neonatal period or less than

2 months

□Group B streptococci

□Gram-negative bacilli

□Staphylococci

□Listeria monocytogenes

□Haemophilus influenzae

Children (exceed 2 months)

□Neisseria meningitides

□Haemophilus influenzae

□Streptococcus pneumoniae

if immunosuppressed

□Gram-negative bacilli

□Staphylococci

1.1 The microbiological cause


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1.2 Risk factor

□infant.

□the immunosuppressed.

□cranial or spinal defects.

□CSF leak.

□penetrating trauma.

□ventricular shunt.


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1.3 Clinical features : symptoms □ there may be a preceding URI (upper respiratory infection)

for a few days.

□ a fever.

□ poor feeding.

□ photophobia.

□ vomiting.

□ convulsion (up to 25% of these patients).

□ irritability.

□ drowsiness.


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1.3 clinical features: signs

Meningeal irritation sign:

1) neck stiffness.

2) Kernig’s sign.

3) Brudzinki’s sign.


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1.3 clinical features: signs

□Meningeal irritation sign

(neck stiffness, Kernig’s sign, Brudzinki’s sign)

□A bulging fontanelle

is indicative of raised intracranial pressure.

(meningeal irritation sign are often absent in infants).

□Focal neurological signs

(palsy, aphasia).


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1.3 clinical features: meningococcaemia

□often rapidly progressive over a few hours.

□fulminating septicaemia, shock and DIC

(disseminated intravascular coagulation).

□a purpuric rash in a toxic child.


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1.4 Complication

□subdural effusions

(due to resistant organisms or inadequate treatment).

□ependymitis.

□hydrocephalus.

□SIADH (inappropriate ADH secretion):

Hyponatraemia and a hypo-osmolar state.

□cranial nerve lesion.


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Subdural effusions normal


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ependymitis normal


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Hydrocephalus normal


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1.5 The diagnosis Examination of the CSF (cerebrospinal fluid)

□an increase

in WBC、 neutrophil and protein.

□a decrease

in CSF glucose.

□a Gram stain:

help to identify of pathogens.

□rapid bacterial antigen detection:

help to identify the organism.


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1.5 The diagnosis The lumbar puncture is avoided:

□severe raised intracranial pressure.

□severe cardiorespiratory compromise.

□fulminant meningococcaemia

with a typical rash and

rapidly progressive course.


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1.5 The diagnosis

Other methods help to identify of pathogens

□several blood cultures.

□nasopharyngeal swabs cultures.

□purpuric rash skin scrapings.


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1.6 management□before identification of the organism:

employing thirdgeneration cephalosporins

such as cefotaxime or ceftriaxone.

□after identification of the organism:

antibiotics have been given according to

the causative organism.

□other management:

mannitol and Dex. for brain edema.

supportive methods.


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1.7 Long-term sequelae

□deafness (up to 30%).

□reduced vision.

□epilepsy.

□neurodevelopmental delay.

□mental retardation.


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Topic 2. Encephalitis

a diffuse infection of the brain parenchyma.

2.1 Causes of encephalitis

□enteroviruses

□arboviruses

□herpes (simplex, varicella)

□measles

□mumps

□rubella

□HIV (Human Immunodeficiency Viruses).

□sometimes with toxoplasma,

mycobacteria and mycoplasma.


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2.2 clinical features□The disease:

1)mild and self-limiting (enteroviruses).

2)severe and rapidly progressive (herpes, mycoplasma).

□symptoms:

fever. headache. vomiting.

sometimes seizures.

hallucination. photophobia.

a nonspecific erythematous rash.

□signs:

altered consciousness (drowsy, stupor, coma).

neck stiffness.


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2.3 The diagnosis

□The CSF shows :

1) normal or increase in white cells.

2) normal or mild increase in protein.

3) normal in CSF glucose.

□EEG (electroencephalogram).

□CT or MRI scanning.


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EEG: normal abnormal


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MRI: viral encephalitis normal


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2.4 management

□specific antimicrobial drugs:

1) aciclovir is the drug of choice with herpes infection.

2) treatment mycoplasma.

□control complications:

1) control raised intracranial pressure (mannitol an

d Dex. for brain edema).

2) control seizures.

□supportive methods.


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Topic 3. Viral meningitis

3.1 Causes of Viral meningitis

□enteroviruses (80%).

□arboviruses.

□herpesviruses.

□mumps.


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3.2 clinical features□symptoms:

1) a nonspecific febrile illness.

2) headache.

3) vomiting.

4) seizure.

□signs:

1) altered consciousness (drowsy, stupor, coma).

2) neck stiffness.

3) focal neurological signs (palsy, aphasia).


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3.3 The diagnosis

The CSF shows :

□normal or increase in white cells.

□normal or mild increase in protein.

□normal in CSF glucose.

□viruses can sometimes be isolated from the CSF.


神经系统疾病

Topic 4. Brain abscess

4.1 Causative agents

□Staphylococcus aureus.

□Streptococci.

□Gram-negative bacilli.

□Anaerobes.

□Fungi (occasionally).


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4.3 clinical features

1) fever.

2) headaches.

3) vomiting.

4) seizure.

5) focal neurological signs.

6) lethargy. coma.


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4.4 The diagnosis

□CT or MRI scanning the diagnosis is confirmed.

□A lumbar puncture is avoided

because of the significant risk of cerebellar h

erniation.


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CT MRI


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4.5 management

□Treatment involves:

combination antibiotics in high dose.

□Surgical aspiration indication:

1) multilocular abscess.

2) fungal abscess.

3) gas formation.

4) significantly raised intracranial pressure.


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Question

□What important features whould you cheak on the general ex

amination of neurological system ?

□What are complications of bacterial meningitis?

□What are clinical features of viral meningitis?


神经系统疾病

Thank

youThank

you

neuromuscular disease 神经系统疾病 neuromuscular disease(1) neurological examination...

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