disorders of cranial nerves

1. CEREBRAL PALSY

2. Definition
 This refers to a chronic disorder of movement, posture and
coordination that arise from an early permanent and non-
progressive lesion of the brain.
 The damage to the brain occurs below 2 years of age and persist
throughout life.
 It is usually associated with epilepsy, impaired hearing, learning
and vision.

3. Aetiology/ causes of cerebral palsy
 The cause may be classified as the following
a) Prenatal cause
 Intrauterine infection (toxoplasmosis, cmv, rubella, herpes zoster)
 Difference in blood btn mother and child
 Metabolic disorders (diabetes mellitus)
 Radiation
 Hypoxia
 Cerebral infarction
 hypotension

4. b) Perinatal
 Intrapartum trauma
 Low birth weight
 Prematurity
 HIE in Birth asphyxia
 Periventricular leukomalacia (in preterm)
 Hydrocephalus

5. c) Postnatal causes
 Infection like meningitis
 Kernicterus
 Hypoglyceamia
 Acute infantile hemiplegia
 Subdural haematoma
 Head trauma
 Cardiopulmonary arrest

6. WHAT ARE THE RISK FACTORS?
•Breech presentation (feet first instead of head first)
•Complicated labor and delivery
•Low birthweight and premature birth
•Multiple births (twins, triplets and other)
•Nervous system malformations
•Maternal bleeding or severe proteinuria
•Maternal hyperthytiroidism, mental retardation
or seizure
•Seizure in the newborn

7. Classification of cerebral palsy
Physiological Topographical Functional
Spastic Hemiplegia Class I- No limitation of activity
Dystonic/ athetoid Diplegia Class 2- Slight to moderate limitation
Ataxic Quadriplegia Class 3- moderate to severe limitation
Hypotonic Double hemiplegia Class 4- No useful physical activity
Mixed Triplegia
Monoplegia

8. WHAT ARE THE DIFFERENT FORMS?
By the type of movement problem
•SPASTIC (inability of a muscle to relax)
•ATETOID or DYSKINETIC (uncontrolled
slow writhing movements)
•ATAXIC (poor coordination, walk unsteadily
with a wide-based gait, intention tremor)
•MIXED FORMS (symptoms of more than one
of the previous three forms)

10. 1. Spastic palsy
 It is the commonest form of cerebral palsy; usually associated with preterm babies.
 It is caused by lesions of the motor cortex, its associated connections and descending tracts
(pyramidal)
 Shows signs of upper motor neuron defects such as
 Spastic hypertonicity/ paralysis
 Exaggerated deep tendon reflexes
 Ankle clonus
 Extensor plantar response

11. Forms of spastic cerebral palsy
A) Spastic hemiplegia
 This affects only one side of the body
 The right side is more commonly affected with the upper limb > lower limb
 Usually caused by
 Congenital malformation of brain
 Congenital cyanotic disease
 Cerebral thrombosis
 Acute cns infection

12. Characteristics of spastic cp
 Delayed movement to about 18-24 months
 Pronation of affected upper limb
 Adduction of the affected lower limb at the hip joint
 Flexion of the knee, elbow and wrist joints
 Tip toeing while walking

13. B) Spastic diplegia (little’s disease)
 It commonly affects preterm babies. Some babies develop hypotonia and dystonia before
spasticity sets in
 They experience extensor hypertonia and when supported by the shoulders there is scissoring of
the lower limbs
SPd may occur in two forms
- Tonic spasticity of flexors
- Phasic spasticity of extensors

14.  The phasically tonic extensor muscles show brisk tendon reflex and clasp knife phenomenon.
 The flexors have decreased lengthening and easily develop contractures
 The main tonic group in lower limb comprises of
 Hip flexors and adductors
 Hamstring muscles
 Calf

15.  The upper limbs are less affected but show flexion of the elbow, wrist, and fingers over an
adducted thumb
 Its commonly associated with epilepsy, speech and behavior disorders
 Commonest neuropathological finding is periventricular leukomalacia

16. c) Spastic quadriplegia
 It’s the most severe form of spastic cp. It results from intrauterine infection or malformation
and is closely associated with HIE (Hypoxic Ischeamic Encephalopathy).
 There is impaired motor function in all four limbs.
 Characterized by;
 feeding difficulties, GERD, learning impairement,
 Scoliosis (lateral curvature of spine)
 Seizures
 Commonest neuropathological finding is severe pvl, cerebral dysgenesis, multicystic
encephalomalacia

17. 2. Dyskinetic cerebral palsy
 Results from lesions of extrapyramidal tracts usually due to neonatal jaundice or acute perinatal
asphyxia
 Characterized by defects in posture and voluntary movt (athetosis, choreoathetosis, rigidity or
dystonia)
 The neuropathological lesion is in globus pallidus and subthalamic nucleus and status
marmoratus (marble appearance of basal ganglia)
 Athetosis involves slow rythmic involuntary movements of the distal ends of extremities.
 Choreoathetosis is characterized by slow rhythmic involuntary movt of distal limbs with
additional jerking of the face and proximal extrimities

18. 3. Ataxic cp
 Its rare and caused by a lesion of the cerebellum.
 Its characterized by hypotonia, intentional tremor and past pointing

19. Associated disabilities
 Epilepsy
 Speech, visual and hearing impairment
 Feeding problems
 Delayed movement and other developmental milestones
 Squinted eyes
 Respiratory disorders
 GERD

20. Diagnosis
 Mainly clinical involving observation of
 Motor delays
 Persistent primitive reflexes
 Abnormal postural reactions
 Children are usually irritable and difficult to handle or cuddle

21. Investigation
 History which may reflect key causes such as hypoxic ischaemic insults or
prematurity
 Imaging with particular concern to the pyramidal tracts (for spastic cp) and
basal ganglia for other forms of cp.

22. Treatment/ management
 Physiotherapy, speech therapy and occupational therapy
 Botulinum toxin to hamstring muscle
 Wheelchair, sleeping or standing devices
 Identification and management of associated complications such as seizures/
epilepsy
 Surgery to release contractures

23. Can cerebral palsy be prevented?
• Breast feed (breast milk helps prevent infection)
• Vaccinate the baby
• Treatment of fever
• Rehydration Drink when the baby has diarrhea preventig deydration helps
prevent fits and brain damage.
• Knowledge the signs of meningitis and begin treatment quickly

24. CAN CEREBRAL PALSY BE PREVENTED?
•Good nutrition of the mother
•Avoid pregnancy until full grown (16-17 years)
•Avoid unnecessary medicines during pregnancy
•Try to avoid getting near persons with German Measles
during pregnancy
•Health check-ups during pregnancy
•Emergency measures of childbirth (learn what to do if the
baby is born blue and does not breathe right away)