Cerebral Palsy PDF

**Cerebral Palsy** **(Little's Disease)** **[Definition ]** ƒ A group of permanent disorders of movement and posture causing activity limitation ƒ Resulting from non-progressive lesions to the developing fetal or infant brain ƒ Affecting mainly the motor centers; cerebral cortex , cerebellum...

**Cerebral Palsy** **(Little's Disease)** **[Definition ]** ƒ A group of permanent disorders of movement and posture causing activity limitation ƒ Resulting from non-progressive lesions to the developing fetal or infant brain ƒ Affecting mainly the motor centers; cerebral cortex , cerebellum , and basal ganglia ƒ With frequent neurologic associations including: - Mental retardation - Epilepsy - Impaired hearing ;deafness - Impaired vision - Emotional disturbances - Behavioral disturbances **[Causes ]** ƒ Pre-natal (80%) - Antenatal Infections - Congenital malformations - Fetal asphyxia ƒ Natal (10%) - Birth asphyxia - Birth trauma ƒ Post-natal (10%) - VLBW with intracranial hemorrhage - Meningitis, encephalitis - Metabolic e.g. phenyle ketonuria - Hypoglycemia - Hyper bilirubinemia - Hydrocephalus. **[Topographic classification:]** **(distribution of motor defect)** 1- Monoplegia → Only one limb is affected 2- Hemiplegia → Upper and lower limbs on one side are affected 3- Diplegia → All limbs are affected, the lower more affected than the upper limbs 4- Paraplagia → Only both lower limbs are affected 5- Quadriplegia → All the four limbs are affected Cerebral Palsy **Clinical Types** **[1. Spastic cerebral palsy ]** [Criteria ] ƒ The commonest type ƒ Pyramidal tract lesion (UMNL) signs: - Hypertonia - Hyper reflexia - Positive Babinski sign - May be clonus Persistence of primitive reflexes Pesudobulbar palsy o feeding disorder (poor suckling & swallowing ), squint and speech disorders. **[Types ]** 1. **Spastic diplegia: 35%** Bilateral spasticity of the legs that is greater than in the arms ƒ More in premature with periventricular leucomalacia - Crawling is commando like rather than four limbed crawling. - Lower limbs scissoring (application of a diaper is difficult) - With paraspinal muscle involvement, the child may be unable to sit. ƒ MRI typically shows scarring and shrinkage in the periventricular white matter with compensatory enlargement of the cerebral ventricles **2. Spastic hemiplegia: 25%** ƒ Due to in utero or neonatal stroke - Decreased spontaneous movements on the affected side - Shows hand preference at a very early age - Walking is delayed until 18-24 mo (tiptoe walking); gait is circumdactive - Examination of the extremities may show growth arrest, particularly in the hand and thumbnail \- Upper extremity assumes a flexed posture when the child runs **3. Spastic quadriplegia: 20%** ƒ More ischemia and infection ƒ The most severe type ƒ Marked motor impairment of all extremities and the high association with mental retardation and seizures **4. Spastic monoplegia** **5. Spastic paraplegia** **[2- Ataxic cerebral palsy ]** [Criteria ] - Hypotonia and hyporeflexia - Cerebellar ataxiao incoordination of voluntary movements, nystagmus, staccato speech, intention tremors. **[3. Extrapyramidal (dyskinetic, asthetoid) cerebral palsy ]** [Commonest causes ] - Asphyxia, kernicterus [Criteria ] - Hypotonia (replaced with time with hypertonia & rigidity) - Chorio asthetoid movements. - Deafness. **[4. Atonic cerebral palsy ]** \- Profound hypotonia → floppy infant \- Preserved deep tendon reflexes **[5. Mixed cerebral palsy ]** **[Diagnosis ]** 1\. Clinical: A thorough history and physical examination should rule out a progressive disorder of the CNS, including degenerative diseases, metabolic disorders, spinal cord tumor, or muscular dystrophy 2\. Investigations: **[Value]**: Exclude progressive brain insults and may detect a cause or association a\. CT & MRI \- May detect the cause e.g. brain malformations and spinal cord lesions \- Rule out brain tumors & degenerative brain disease. \- CT scan may be useful for detecting calcifications associated with congenital infections b\. TORCH screen. c\. Genetic evaluation d\. Metabolic screen. e\. For associations: Test for Hearing, Visual function , EEG for seizures **N.B Conditions that can mimic cerebral palsy** \* Spinal cord tumors \* Channelopathies \* Sandifer syndrome \* MECP2 duplication \* Congenital dopa-responsive disorders \* Genetic spastic paraplegia \* Some metabolic conditions (GLUT1 deficiency, glutaric aciduria type1) \* Ataxia telangiectasia **[Treatment]** ƒ Multidisciplinary approach is most helpful in the assessment and treatment of such children; A team of physicians from various specialties as: - Occupational and physical therapists - Speech pathologists - Social workers - Educators - Developmental psychologists ƒ **Assist**: - Feeding & defecation - Vision and hearing - Walking: Walkers, standing frames, motorized wheel chair - Communication by talking typewriters and special computers - Rehabilitation according to the degree of motor disability **Medications** **Anti spastic drugs** +-----------------------+-----------------------+-----------------------+ | **drug** | **Action** | **Side effect** | +-----------------------+-----------------------+-----------------------+ | **Diazepam** | \- GABA agonist | \- Sedation | | | | | | | \- Useful in short | \- Dependency with | | | term relief of | long term use | | | painful | | +-----------------------+-----------------------+-----------------------+ | **Baclofen** | \- GABA agonist and | \- Sedation | | | inhibit spinal | | | | neuronal transmission | | | | | | | | \- When used | | | | intrathecally, via a | | | | surgically implanted | | | | continuous-delivery | | | | pump → greater | | | | efficacy with fewer | | | | adverse effects | | +-----------------------+-----------------------+-----------------------+ | **Tizanidine** | \- Alpha-2 adrenergic | \- Sedation | | | receptor agonist and | | | | inhibit spinal | | | | neuronal transmission | | | | | | | | \- Useful in severely | | | | disabled by cerebral | | | | palsy and in those | | | | with night-time | | | | spasms | | +-----------------------+-----------------------+-----------------------+ | **Dantrolene** | \- Block calcium | \- Hepatic | | | intake by skeletal | dysfunction | | | | | | | muscles→ ↓free | \- Blood dyscrasia | | | intracellular calcium | | +-----------------------+-----------------------+-----------------------+ **Botox A**: injection in spastic muscles and salivary glands to reduce drooling. It stopsthe release of acetylcholine at the synapse and blocks neurotransmission. The effects gradually wear off (over about 3--6 months) **Levodopa:** Small doses may be helpful for dystonia and rigidity **Surgery:** For marked spasticity of the lower extremities → surgical soft tissue procedures that reduce muscle spasm e.g. adductor tenotomy or psoas transfer and rhizotomy (roots of the spinal nerves are divided)