Upload
ami-banks
View
225
Download
3
Embed Size (px)
Citation preview
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
Neuromuscular Disease
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.
Neuromuscular Disease
神经系统疾病
Topic 1. General examination
1.1 level of consciousness consciousness
unconsciousness:
□sleepy (drowsy).
□stupor (lethargy).
□coma.
complicating abnormal mental condition
□confusion.
□delirium.
Neuromuscular Disease
神经系统疾病
Topic 1. General examination
1.2 head □head shape, head circumference microcephaly.
hydrocephalus. cephalus quadratus.
malformation of CNS. □fontanelle
a bulging fontanelle is indicative of raised intracranial pressure.
Neuromuscular Disease
神经系统疾病
Topic 1. General examination
hydrocephal
us
Microcephaly
Neuromuscular Disease
神经系统疾病
Topic 1. General examination
a bulging fontanelleis indicative of raised intracranial pressure.
Neuromuscular Disease
神经系统疾病
Topic 1. General examination
1.3 Skin: neurocutaneous stigmata
neurofibromatosis
tuberous sclerosis
Neuromuscular Disease
神经系统疾病
Topic 1. General examination
The general examination of neurological system
emphasize:
□level of consciousness.
□head.
□skin.
Neuromuscular Disease
神经系统疾病
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).
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
base of skull
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
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.
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
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.
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
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’.
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
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.
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
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.
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
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.
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
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.
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
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.
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
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’.
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
The eleventh nerve (accessory nerve)eleventh nerve lesion the child doesn’t raise his shouldersand push his head against resistance.
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
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.
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
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.
Neuromuscular Disease
神经系统疾病
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.
Neuromuscular Disease
神经系统疾病
Topic 3. Motor examination
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.
Neuromuscular Disease
神经系统疾病
Topic 3. Motor examination
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.
Neuromuscular Disease
神经系统疾病
inborn reflex
Moro reflex
Grasp reflex
Sucking reflex
Rooting reflex
Neuromuscular Disease
神经系统疾病
Topic 3. Motor examination
3.4 Mobility and gait (e.g. cerebral palsy)
□writhing actions (athetosis).
□abnormal movements (scissors gait).
Neuromuscular Disease
神经系统疾病
Topic 3. Motor examination
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.
Neuromuscular Disease
神经系统疾病
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.
Neuromuscular Disease
神经系统疾病
Section 2.Infections of the CNS (p128)
Neuromuscular Disease
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.
Neuromuscular Disease
神经系统疾病
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
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
1.2 Risk factor
□infant.
□the immunosuppressed.
□cranial or spinal defects.
□CSF leak.
□penetrating trauma.
□ventricular shunt.
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
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.
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
1.3 clinical features: signs
Meningeal irritation sign:
1) neck stiffness.
2) Kernig’s sign.
3) Brudzinki’s sign.
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
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).
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
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.
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
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.
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
Subdural effusions normal
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
ependymitis normal
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
Hydrocephalus normal
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
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.
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
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.
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
1.5 The diagnosis
Other methods help to identify of pathogens
□several blood cultures.
□nasopharyngeal swabs cultures.
□purpuric rash skin scrapings.
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
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.
Neuromuscular Disease
神经系统疾病
Topic 1. Bacterial meningitis
1.7 Long-term sequelae
□deafness (up to 30%).
□reduced vision.
□epilepsy.
□neurodevelopmental delay.
□mental retardation.
Neuromuscular Disease
神经系统疾病
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.
Neuromuscular Disease
神经系统疾病
Topic 2. Encephalitis
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.
Neuromuscular Disease
神经系统疾病
Topic 2. Encephalitis
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.
Neuromuscular Disease
神经系统疾病
Topic 2. Encephalitis
EEG: normal abnormal
Neuromuscular Disease
神经系统疾病
Topic 2. Encephalitis
MRI: viral encephalitis normal
Neuromuscular Disease
神经系统疾病
Topic 2. Encephalitis
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.
Neuromuscular Disease
神经系统疾病
Topic 3. Viral meningitis
3.1 Causes of Viral meningitis
□enteroviruses (80%).
□arboviruses.
□herpesviruses.
□mumps.
Neuromuscular Disease
神经系统疾病
Topic 3. Viral meningitis
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).
Neuromuscular Disease
神经系统疾病
Topic 3. Viral meningitis
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.
Neuromuscular Disease
神经系统疾病
Topic 4. Brain abscess
4.1 Causative agents
□Staphylococcus aureus.
□Streptococci.
□Gram-negative bacilli.
□Anaerobes.
□Fungi (occasionally).
Neuromuscular Disease
神经系统疾病
Topic 4. Brain abscess
4.3 clinical features
1) fever.
2) headaches.
3) vomiting.
4) seizure.
5) focal neurological signs.
6) lethargy. coma.
Neuromuscular Disease
神经系统疾病
Topic 4. Brain abscess
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.
Neuromuscular Disease
神经系统疾病
Topic 4. Brain abscess
CT MRI
Neuromuscular Disease
神经系统疾病
Topic 4. Brain abscess
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.
Neuromuscular Disease
神经系统疾病
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?
Neuromuscular Disease
神经系统疾病
Thank
youThank
you