Department of Pediatrics
Soochow University Affiliated Children’s Hospital
Neuromuscular Disease
神经系统疾病
Main contents: four divisions
1.
2.
3.
4.
Introduce the general examination of neurological system.
Introduce the examination of the cranial nerves.
Emphasize the motor examination.
Introduce the sensory examination.
Neuromuscular Disease
神经系统疾病
Topic 1. General examination
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
microcephaly. hydrocephalus.
cephalus quadratus.
malformation of CNS
a bulging fontanelle is indicative of raised intracranial pressure.
Neuromuscular Disease
神经系统疾病
Topic 1. General examination
Neuromuscular Disease
神经系统疾病
a bulging fontanelle is 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
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
first cranial nerve (olfactory nerve).
second nerve (optical nerve).
third nerve (oculomotor nerve).
fourth nerve (trochlear nerve).
fifth nerve (trigeminal nerve).
sixth nerve (abducent nerve).
seventh nerve (facial nerve). eighth nerve (vestibulocochlear nerve). ninth nerve (glossopharyngeal nerve).
tenth nerve (vagus nerve). eleventh nerve (accessory nerve). 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
□ 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 nasolabial folds and drawing of the face to the normal side.
□the child shouldn’t be able to blow out the cheeks and close the mouth tightly.
Neuromuscular Disease
神经系统疾病
Topic 2. The cranial nerves
□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 shoulders and 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
Ⅱ not tested. blinks to bright light; appears to fix/follow(3+months).
Ⅲ Ⅵ assess highlights on pupils for squint; abnormal position of globe.
Ⅴ
Ⅻ corneal reflexes; sucking movements.
Ⅶ
Ⅺ facial asymmetry especially while crying.
Ⅷ startles to sound; quietens with soothing.
Ⅸ Ⅹ gag reflex.
not tested.
tongue movement.
Neuromuscular Disease
神经系统疾病
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
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
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
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
Neuromuscular Disease
神经系统疾病
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
1.1 The microbiological cause
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
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
□ 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
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
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
□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
□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 and 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
□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
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
□CT or MRI scanning the diagnosis is confirmed.
□A lumbar puncture is avoided because of the significant risk of cerebellar herniation.
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
□What important features whould you cheak on the general examination of neurological system ?
□What are complications of bacterial meningitis?
□What are clinical features of viral meningitis?
Neuromuscular Disease
神经系统疾病
神经系统疾病