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1、Neurologic & Muscular Disorders Zhou Shuizhen (周水珍) Department of Neurology Childrens Hospital of FuDan University,Part I: General Consideration, Anatomical and physiology features of the child CNS Neurological examination of children Key facts about neurological disorders in children: Infections of
2、 the CNS Neurocutaneous syndromes Cerebral palsy Progressive muscular dystrophy Seizure disorders (epilepsies & non-epilepsy seizures) Convulsions & Weakness (PBL),Physiologic and anatomical features of the child CNS,1. Brain - weight : at birth 6m 1y 2y 4-6y adult 370 700 900 1000 1300 1500 (g) - c
3、erebra / pallium / cell number - myelinization: fetal period 4yrs. 2. Spinal Cord - weight: 26g (at birth) - terminal : L3 (fetal); L1(4y) - myelinization: 3y,Neurological examination of children, A childs nervous system is continuous development Methods of neurological must be adapted to the childs
4、 developmental stage Neurological signs are altered by external factors Examination reveals presence of abnormalities, locations of lesions and clues of etiology,I. General observation,1. General observation: Hyperactivity, attention, emotional state 2. Consciousness: Level; Quality 3. Speech Bruit,
5、 transillumination of head 5. Neck: Movement, deformity, signs of meningeal irritation 6. Skin: Cafe au lait spots, telangiectasis, haemangiomas,II. Cranial nerves,1. Smell 2. Vision-field, acuity, abnormal eye movement 3. Pupil size, reactions 4. Fundi 5. Ocular movement, squint, nystangmus, ptosis
6、, sun-set sign 6. Jaw movement, jaw jerk, facial sensation & corneal reflex 7. Ear examination, hearing, vestibular function 8. Paltal movement, gag reflex 9. Rotate head, elevate shoulders, extend neck 10. Tongue-protrusion, atrophy, fasciculation, percussion-myotonia,III. Motor Functions,1. Observ
7、e spontaneous posture & movement: Lying, sitting, standing, walking, running, climbing & descending, grasping, manipulation. 2. Muscle bulk, consistency 3. Muscle tone fasciculation, myotonia 4. Muscle power, joint by joint 5. Ataxia Truncal while sitting & standing Limds: Reaching, Finger-nose, Hee
8、l-knee Rapid alternating movements 6. Involuntary movement Chorea, athetosis, dystonia tremor, myoclonus, at rest and on movement,VI. Sensation,1. Superficial: touch, pain, temperature 2. Proprioceptive: position joint, vibration 3. Cortical: stereognosis, graphaesthesia, 2 point discrimination, tex
9、ture, shape, weight 4. Special: hearing( and comprehension), taste, Sight ( and comprehension ), smell 5. Sensorimotor co-ordination,V. Reflexes,1. Tendox reflexes 2. Superficial anal, plantar 3. Clonus 4. Babinski, Gordon, Chaddock, Oppenheim, Hoffmann 5. Meningeal irritation stiff neck, Kerning/Br
10、udzinsk signs, Clinical Findings: fever, malaise, hypothermia headache, mental status, seizures, focal deficits meningeal irritation(+) / a bulging fontanelle Babinski, Gordon, Chaddock, Oppenheim (+) Laboratory Findings: CSF / EEG / CT & MRI (+) Differential microbes: viral, bacterial, tuberculous,
11、 cryptococcus,1. Infectious Disease of CNS, Neurofibromatosis 17q11.2 ; 22q11 （1:3000; 1:3300040000） Tuberous sclerosis 9q34 ; 16p13.3 （ 1:9500 1:20000） Sturge-Weber syndromes (Encephalofacial angiomatosis),2. Neurocutaneous syndromes,(1) Neurofibromatosis, Skin: Cafe-au-lait Axillary freckles Perip
12、heral neurofibromas Eye: Pigmented bumps on the iris (Lisch nodules) Brain: Plexiform neurofibromas Optic pathway glioma Epilepsy,Neurofibromatosis,Neurofibromatosis,(2) Tuberous sclerosis, Skin: hypomelanotic macules ungual fibroma facial angiofibroma Shagreen plaque Brain: mental retardation seizu
13、res Eyes: Retinal depigmented spots Brain CT: Cortical tubers, subependymal nodules, subependymal giant cell astrocytomas,Tuberous sclerosis,Tuberous sclerosis,(3) Sturge-Weber syndrome, Skin: Facial angioma or Port-wine stain Facial angiona Brain: Seizures Hemiplegia Hemianopsia Leptomeningeal veno
14、us angiomatos Eyes: Choroidal angioma Glaucoma,Sturge-Weber syndrome,3. Cerebral Palsy, Causes：Antenatal (80%), Intrapartum (10%), Postnatal (10%) Clinical features: 1) Non-progressive lesion of the developing brain ( 1 month ) 2) Delayed motor milestone 3) Clinical examination show abnormalities of
15、 : Tone hypertonia or hypotonia; Power hemiparesis Reflexes brisk tendox reflexes or persistence primitive reflexes Abnormal movements & posture or gait Classification: Spastic (70%) hemiplegic, diplegic, quadriplegic Dyskinetic(10%) athetoid, dystonic Ataxic (10%) hypotonic Mixed (10%),Cerebral palsy,4. Progressive muscular dystrophy, Causes:x-linked recessive disease (male 1:4000), Xp21 Clicical featur
