Palsy's

Question 1

What is cerebral palsy (CP)?

Answer 1

Cerebral palsy (CP) is the name given to the permanent neurological problems resulting from damage to the brain around the time of birth. It is not a progressive condition, however the nature of the symptoms and problems may change over time during growth and development

Question 2

What is there a huge variation of in CP?

Answer 2

severity and type of symptoms, ranging from completely wheelchair bound and dependent on others for all activities of daily living, to para-olympic athletes with only subtle problems with coordination or mobility.

Question 3

What are the different types of causes of CP?

Answer 3

Antenatal
Perinatal
Postnatal

Question 4

Antenatal causes of CP

Answer 4

Maternal infections
Trauma during pregnancy

Question 5

Perinatal causes of CP?

Answer 5

Birth asphyxia
Pre-term birth

Question 6

Postnatal causes of CP?

Answer 6

Meningitis
Severe neonatal jaundice
Head injury

Question 7

What are the different types of CP?

Answer 7

Spastic: hypertonia (increased tone) and reduced function resulting from damage to upper motor neurones
Dyskinetic: problems controlling muscle tone, with hypertonia and hypotonia, causing athetoid movements and oro-motor problems. This is the result of damage to the basal ganglia.
Ataxic: problems with coordinated movement resulting from damage to the cerebellum
Mixed: a mix of spastic, dyskinetic and/or ataxic features

Question 8

What is spastic CP also known as?

Answer 8

Spastic CP is also known as pyramidal CP. Dyskinetic CP is also known as athetoid CP and extrapyramidal CP.

Question 9

What are the patters of Spastic CP?

Answer 9

Monoplegia: one limb affected
Hemiplegia: one side of the body affected
Diplegia: four limbs are affects, but mostly the legs
Quadriplegia: four limbs are affected more severely, often with seizures, speech disturbance and other impairments

Question 10

What are the S + S of someone with CP?

Answer 10

more evident during development:

• Failure to meet milestones
• Increased or decreased tone, generally or in specific limbs
• Hand preference below 18 months is a key sign to remember for exams
• Problems with coordination, speech or walking
• Feeding or swallowing problems
• Learning difficulties

Question 11

What are the types of gait that can be found in a child with CP on neurological examination?

Answer 11

• Hemiplegic / diplegic gait: indicates an upper motor neurone lesion
• Broad based gait / ataxic gait: indicates a cerebellar lesion
• High stepping gait: indicates foot drop or a lower motor neurone lesion
• Waddling gait: indicates pelvic muscle weakness due to myopathy
• Antalgic gait (limp): indicates localised pain

Question 12

On neurological examination what will you find in an UMN lesion for a child with CP?

Answer 12

Inspection: Muscle bulk preserved
Tone: Hypertonia
Power: Slightly reduced
Reflexes: Brisk

Question 13

On neurological examination what will you find in an LMN lesion for a child with CP?

Answer 13

Inspection: Reduced muscle bulk with fasciculations
Tone: Hypotonia
Power: Dramatically reduced
Reflexes: Reduced

Question 14

What is a hemiplegic and diplegic gait?

Answer 14

Gait is caused by increased muscle tone and spasticity in the legs. The leg will be extended with plantar flexion of the feet and toes. This means they have to swing the leg around in a large semicircle when moving their leg from behind them to in front. There is not enough space to swing the extended leg in a straight line below them.

Question 15

What will patients with hemiplegic or diplegic gait have signs of?

Answer 15

They will have signs of an upper motor neurone lesion, with good muscle bulk, increased tone, brisk reflexes and slightly reduced power. Power may be normal. Look for athetoid movements that indicate extrapyramidal (basal ganglia) involvement. Test for coordination to look for cerebellar involvement.

Question 16

Complications and Associated Conditions of CP

Answer 16

• Learning disability
• Epilepsy
• Kyphoscoliosis
• Muscle contractures
• Hearing and visual impairment
• Gastro-oesophageal reflux

Question 17

Management of CP

Answer 17

Incurable
When asked how you would manage complex conditions such as cerebral palsy the answer should always start with “management will involve a multi-disciplinary team approach”.
Physiotherapy is used to stretch and strengthen muscles, maximise function and prevent muscle contractures.

Occupational therapy is used to help patients manage their everyday activities, such as getting dressed and using the bathroom. That can involve techniques to perform tasks despite disability. They can also make adaptations and supply equipment, such as rails for assistance or fitting a hoist for a patient who is entirely wheelchair bound.

Speech and language therapy can help with speech and swallowing. When swallowing difficultly prevents them meeting their nutritional requirements they may require an NG tube or PEG tube to be fitted.

Dieticians can help ensure they meet nutritional requirements. Some children may require PEG feeding through a port on their abdomen that gives direct access to the stomach.

Orthopaedic surgeons can perform procedures to release contractures or lengthen tendons (tenotomy).

Paediatricians will regularly see the child to optimise their medications. This may involve:

Muscle relaxants (e.g. baclofen) for muscle spasticity and contractures
Anti-epileptic drugs for seizures
Glycopyrronium bromide for excessive drooling
Social workers to help with benefits and support.

Charities and support groups provide opportunities to connect with others affected by cerebral palsy and learn and share information on the condition.

Question 18

Epidemiology of CP

Answer 18

Cerebral palsy is the most common cause of physical disability in children and young people in the developed world, with a prevalence of around 2 to 2.5 per 1,000

Question 19

How do children with CP generally present to services?

Answer 19

either by identification of atypical motor patterns in those considered at high risk because of antenatal or perinatal complications, or
because of atypical motor development picked up during background population assessment

Question 20

What is choreoathethetoid CP?

Answer 20

Congenital choreoathetosis accounts for about 10% of all cases of cerebral palsy, and is the characteristic formm of cerebral palsy following kernicterus, although it may also follow hypoxia.

This condition is characterised by involuntary movements, usually chorea, athetosis and dystonia of the limbs, trunk, face and bulbar muscles

Question 21

What is Hypoxic- Ischaemic encephalopathy?

Answer 21

(HIE) occurs in neonates as a result of hypoxia during birth. Hypoxia is a lack of oxygen, ischaemia refers to a restriction in blood flow to the brain and encephalopathy refers to malfunctioning of the brain

Question 22

What can HIE lead to?

Answer 22

HIE can lead to permanent damage to the brain, causing cerebral palsy. Severe HIE can result in death

Question 23

When is there suspected HIE in neonates?

Answer 23

when there are events that could lead to hypoxia during the perinatal or intrapartum period: acidosis (pH < 7) on the umbilical artery blood gas
poor Apgar scores
features of mild, moderate or severe HIE (see below) or evidence of multi organ failure

• foetal tachycardia
• late decelerations on the CTG
• scalp pH of less than 7.2
• meconium stained liquor
• poor response to delivery

Question 24

What are the causes of HIE?

Answer 24

Anything that leads to asphyxia (deprivation of oxygen) to the brain can cause HIE. For example:

• Maternal shock
• Intrapartum haemorrhage
• Prolapsed cord, causing compression of the cord during birth
• Nuchal cord, where the cord is wrapped around the neck of the baby
• maternal asphyxia - lung, heart disease
• poor uterine perfusion - shock, posture, vascular disease
• disease and separation of the placenta
• foetal anaemia and heart failure

Question 25

HIE Grades (Sarnat staging)

Answer 25

• Mild

Poor feeding, generally irritability and hyper-alert
Resolves within 24 hours
Normal prognosis

• Moderate
  Poor feeding, lethargic, hypotonic and seizures
  Can take weeks to resolve
  Up to 40% develop cerebral palsy
• Severe
  Reduced consciousness, apnoeas, flaccid and reduced or absent reflexes
  Up to 50% mortality
  Up to 90% develop cerebral palsy

Question 26

Management of HIE

Answer 26

Management will be coordinated by specialists in neonatology, on the neonatal unit. This involves supportive care with neonatal resuscitation and ongoing optimal ventilation, circulatory support, nutrition, acid base balance and treatment of seizures. Therapeutic hypothermia is an option in certain circumstances to help protect the brain from hypoxic injury.

Children will need to be followed up by a paediatrician and the multidisciplinary team to assess their development and support any lasting disability

Question 27

What is a therapeutic hypothermia?

Answer 27

• Babies near or at term considered to have HIE can benefit from therapeutic hypothermia. - Therapeutic hypothermia involves actively cooling the core temperature of the baby according to a strict protocol.
• baby is transferred to neonatal ICU and actively cooled using cooling blankets and a cooling hat.
• The temperature is carefully monitored with a target of between 33 and 34°C, measured using a rectal probe. This is continued for 72 hours, after which the baby is gradually warmed to a normal temperature over 6 hours.
• The intention of therapeutic hypothermia is to reduce the inflammation and neurone loss after the acute hypoxic injury. It reduces the risk of cerebral palsy, developmental delay, learning disability, blindness and death

Question 28

Epidemiology of HIE

Answer 28

occurring at 1 to 2 per 1000 deliveries

Question 29

What is some further management of HIE?

Answer 29

The management is based on the severity of the asphyxia. Full resuscitation is required, and weaned only when blood gases are normal.

• Ventilation should be considered as appropriate - ie in more severe hypoxic ischaemic encephalopathy, where there is more severe lung disease, apnoea or poor blood gases.
• Acidosis should be corrected with ventilation or with volume replacement as indicated.
• Convulsions may be managed with phenobarbitone, using second line drugs if the fits are not controlled.
• Clotting should be measured and abnormality corrected with fresh frozen plasma or platelets.

Question 30

What is bulbar palsy and what is it due to?

Answer 30

refers to bilateral impairment of function of the lower cranial nerves IX, X, XI and XII, which occurs due to lower motor neuron lesion either at nuclear or fascicular level in the medulla or from bilateral lesions of the lower cranial nerves outside the brain-stem.

Question 31

What does bulbar palsy give rise to?

Answer 31

• dysarthria
• dysphagia (often with choking episodes and nasal regurgitation of fluids),
• dysphonia
• poor cough
• susceptibility to aspiration pneumonia.

Question 32

What is the disturbance of the motor nuclei due to?

Answer 32

The disturbance is of the motor nuclei rather than of the corticobulbar tracts. It is distinguished from pseudobulbar palsy by the presence of lower motor neurone signs. Autonomic features are uncommon.

Question 33

What are the causes of bulbar palsy include?

Answer 33

motor neurone disease
Guillain Barre syndrome
syringobulbia
poliomyelitis
diphtheria
myasthenia gravis
neurosyphilis

Question 34

What are the clinical features of bulbar palsy?

Answer 34

• atrophy and fasciculations in bulbar muscles:
• tongue appears wasted and folded; fasciculations are prominent and produce a writhing appearance
• the palate and the extrinsic muscles of the pharynx and larynx are affected after the tongue
  protrusion of the tongue is weakened and lost
• dysarthria and dysphonia - from paresis of the lips, - tongue and palate; nasal speech is typical
  dysphagia - food may regurgitate through the mouth; may be most noticeable with fluids
  jaw jerk and gag reflex are absent

Question 35

What are the differences between bulbar and pseudobulbar palsy?

Answer 35

Pseudobulbar Palsy

Bulbar Palsy

degeneration of corticobulbar pathways to V,VII,X,XI,XII

disturbance to X, XI, XII,sometimes VII, rather than the corticobulbar tracts

lower motor neurone signs absent

lower motor neurone signs present

gag reflex (+/n)

gag reflex (-)

spastic tongue

wasted tongue, fasciculations

jaw jerk (+)

jaw jerk (n)

spastic dysarthria

nasal speech

labile emotions

normal emotions

bilateral UMN

signs in limbs

Question 36

What is juvenile bulbar palsy?

Answer 36

Juvenile bulbar palsy is an autosomal recessive disorder which present in late childhood with drooling and dysarthria, progressing to aspiration pneumonia and respiratory failure. Limb involvement and pyramidal tract signs may develop.

Most die within the first two decades of life

Question 37

What is a facial nerve palsy?

Answer 37

Facial nerve palsy refers to isolated dysfunction of the facial nerve. This typically presents with a unilateral facial weakness. It is important to understand some basics about the pathway and function of the facial nerve

Question 38

What is the facial nerve pathway?

Answer 38

The facial nerve exits the brainstem at the cerebellopontine angle. On its journey to the face, it passes through the temporal bone and parotid gland.

It then divides into five branches that supply different areas of the face:

Temporal
Zygomatic
Buccal
Marginal mandibular
Cervical

Question 39

What are the 3 functions of the facial nerve?

Answer 39

motor, sensory and parasympathetic

Question 40

What are the motor, sensory and parasympathetic functions of the facial nerve?

Answer 40

Motor

It supplies the muscles of facial expression, the stapedius in the inner ear and the posterior digastric, stylohyoid and platysma muscles in the neck.

Sensory

It carries taste from the anterior 2/3 of the tongue.

Parasympathetic

It provides the parasympathetic supply to the:

Submandibular and sublingual salivary glands
Lacrimal gland (stimulating tear production)

Question 41

Why should we distinguish between UMN and LMN facial nerve palsy?

Answer 41

in a patient with a new-onset upper motor neurone facial nerve palsy, you should be referring immediately with a suspected stroke. In contrast, patients with a lower motor neurone facial nerve palsy can be managed with less urgency.

Question 42

Forehead and UMN and LMN innervation?

Answer 42

Each side of the forehead has upper motor neurone innervation by both sides of the brain. However, each side of the forehead only has lower motor neurone innervation from one side of the brain.

In an upper motor neurone lesion, the forehead will be spared, and the patient can move their forehead on the affected side.

In a lower motor neurone lesion, the forehead will NOT be spared, and the patient cannot move their forehead on the affected side.

Question 43

How can you differentiate between an UMN and LMN lesion?

Answer 43

asking the patient to raise their eyebrows. If they can raise both eyebrows and wrinkle both sides of the forehead, the patient has an upper motor neurone lesion. If the eyebrow on the affected side cannot be raised and the forehead remains smooth, the patient has a lower motor neurone lesion.

Question 44

Examples of UMN lesions

Answer 44

Unilateral upper motor neurone lesions occur in:

Cerebrovascular accidents (strokes)
Tumours

Bilateral upper motor neurone lesions are rare. They may occur in:

Pseudobulbar palsies
Motor neurone disease

Question 45

What is Bell’s palsy?

Answer 45

Bell’s palsy is a relatively common condition. It is idiopathic, meaning there is no apparent cause. It presents as a unilateral lower motor neurone facial nerve palsy. The majority of patients fully recover over several weeks, but recovery may take up to 12 months. A third are left with some residual weakness.

Question 46

If patients present within 72 hours of developing symptoms, NICE clinical knowledge summaries (updated 2019) recommend for Bulbar palsy….

Answer 46

considering prednisolone as treatment, either:

50mg (25 mg twice a day) for 10 days
60mg for 5 days followed by a 5-day reducing regime of 10mg a day
treatment is probably more effective before 72 hours and less effective after seven days

Question 47

Clinical features of Bell’s Palsy

Answer 47

usually of sudden onset and complete within 24 hours; less frequently, the condition is progressive developing over a course of less than 4 days
almost always unilateral
onset may be preceded by post-auricular pain which develops over a 48 hour period
there is paralysis of the upper and lower facial muscles
the eyebrow droops and the wrinkles of the brow are smoothed out; frowning and raising the eyebrows are impossible
if there is an upper motor neurone lesion affecting the facial nerve then the ability to wrinkle the brow is preserved; in Bell’s palsy this ability is lost
the lower lid is everted. Tear production (lachrymation) is decreased. Eye irritation often occurs due to lack of lubrication and constant exposure. The eye may appear to tear excessively due to loss of lid control, which may cause tears to spill freely from the eye (3)
the mouth sags and the patient dribbles. The patient is unable to blow out the cheeks. The lips cannot be pursed and whistling is impossible. The effects tend to be more pronounced in the elderly.

Question 48

Why do patients with facial palsy require careful examination of other cranial nerves and cerebellar function?

Answer 48

a central upper motor neurone deficit causes weakness of the lower face only. More complex segmental deficits may be caused by peripheral facial nerve lesions. Therefore patients with facial palsy require careful examination of the other cranial nerve and cerebellar function

Question 49

Bell’s palsy - left untreated stats

Answer 49

left untreated, 70-75% of patients make a full recovery

Question 50

What do bulbar palsy patients also possibly require…?

Answer 50

Patients also require lubricating eye drops to prevent the eye on the affected side from drying out and being damaged. If they develop pain in the eye, they need an ophthalmology review for exposure keratopathy. The eye can be taped closed at night

inability to close the eye on the affected side, can lead to irritation and corneal ulceration
requires urgent consultation with an ophthalmologist
the inability to close the eye on the affected side can, if ignored, lead to irritation and even corneal ulceration
can be avoided if good advice on eye care is offered at the outset
eye care focuses on lubricating the eye regularly, taping the eye closed overnight and manual blink and eyelid stretching

Question 51

Typical presentations of Bell’s palsy?

Answer 51

facial distortion, loss of taste, hyperacusis and a watery eye.

Question 52

Incidence of Bell’s palsy

Answer 52

Bell’s palsy accounts for almost three quarters of all acute facial palsies, with the highest incidence in the 15 to 45 year old age group (1)
The reported incidence is 23-32 cases per 100,000 per year (1) with men and women equally affected. (1)
It occurs more commonly in patients with diabetes and in pregnant women (2)
a general practitioner expects to see one case every 2 - 3 years

Question 54

Herpes and Bell’s palsy?

Answer 54

there has been increasing evidence to suggest that the main cause of Bell’s palsy is latent herpes viruses (herpes simplex virus type 1 and herpes zoster virus), which are reactivated from cranial nerve ganglia

inflammation of the nerve initially results in a reversible neurapraxia - however ultimately Wallerian degeneration ensues
herpes zoster virus appears to exhibit more aggressive biological behaviour than herpes simplex virus type 1 - this is because it spreads transversely through the nerve by way of satellite cells

Question 55

What about the facial nerve makes it vulnerable to neoplastic, traumatic and infectious events?

Answer 55

the course of the facial nerve through the posterior fossa, temporal bone, and parotid gland makes it vulnerable to many neoplastic, traumatic, and infectious events (1)

Question 56

What does a peripheral facial paralysis require?

Answer 56

requires a complete head and neck examination including the following:
otoscopy
cranial nerve evaluation. (1)

Question 57

What are the unilateral upper motor neurone causes of facial nerve palsy?

Answer 57

vascular disease
tumour

Question 58

What are the unilateral lower motor neurone palsy causes of facial nerve palsy?

Answer 58

pontine causes:
vascular
tumour
multiple sclerosis
posterior fossa causes:
acoustic neuroma
meningioma

Question 59

Extracranial causes of facial nerve palsy

Answer 59

traumatic
facial lacerations / blunt forces, newborn paralysis

Question 60

Neoplastic causes of facial nerve palsy

Answer 60

parotid tumors,tumors of the external canal and middle ear, facial nerve neurinomas, metastatic lesions

Question 61

What are the intratemporal causes of facial nerve palsy?

Answer 61

traumatic
- fractures of petrous pyramid, penetrating injuries
neoplastic
- glomus tumors, cholesteatoma, facial neurinomas, acoustic neurinomas, squamous cell carcinomas,metastatic
infectious
- herpes zoster oticus ( Ramsey-Hunt syndrome), acute otitis media, chronic otitis media, malignant otitis externa
idiopathic
- Bell’s palsy (2)
although traditionally defined as idiopathic it is thought to be associated with herpes simplex virus type 1
intracranial
- neoplastic lesions, stroke (2)

Question 62

DDs of Bells palsy

Answer 62

Lyme disease
Otitis media
Ramsay hunt syndrome
Sarcoidosis
GBS
HIV
Tumours
MS
Stroke
Tumours
Horners syndrome

Question 63

Referral criteria from primary care - Bell’s Palsy

Answer 63

Referral criteria to secondary care: (1)

urgent referral for ear, nose, and throat (ENT) or neurological review if there is:

diagnostic uncertainty

bilateral Bell’s palsy

recurrent Bell’s palsy

refer for urgent ophthalmological review if the cornea remains exposed after attempting to close the eyelid

refer urgently to ENT if the paralysis shows no sign of improvement after 1 month, or there is suspicion of a serious underlying diagnosis (e.g. cholesteatoma, parotid tumour, malignant otitis externa)

consider referral to a plastic surgeon with a special interest in facial reconstructive surgery If there is residual paralysis after 6-9 months
NICE guidance suggests (2):

do not routinely refer adults with an uncomplicated episode of Bell’s palsy (unilateral lower motor neurone pattern facial weakness affecting all parts of the face and including weakness of eye closure) and no evidence of another medical condition such as middle ear disease

advise adults with Bell’s palsy about eye care, and explain that Bell’s palsy improves at different rates and maximum recovery can take several months

consider referring adults with Bell’s palsy who have developed symptoms of aberrant reinnervation (including gustatory sweating or jaw-winking) 5 months or more after the onset of Bell’s palsy for neurological assessment and possible treatment.