复旦大学：《儿科学》课程教学资源（PPT课件讲稿）第四讲 神经系统疾病

Neurologic Muscular Disorders Zhou shuzhen(周水珍) Department of Neurology Childrens Hospital of FuDan University 复旦大学附属儿科医院

Neurologic & Muscular Disorders Zhou Shuizhen (周水珍) Department of Neurology Children’s Hospital of FuDan University

Part I: General Consideration o Anatomical and physiology features of the child cns o Neurological examination of children o Key facts about neurological disorders in children Infections of the cns Neurocutaneous syndromes Cerebral pals Progressive muscular dystrophy Seizure disorders(epilepsies non-epilepsy seizures) Convulsions Weakness (PBL) 回复旦大学上海医学院儿科学系

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 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 ly 2y 4-6y adult 370700900100013001500(g) cerebra/pallium /cell number myelinization: fetal period w 4yrs 2. Spinal Cord weight: 2N6g(at birth) terminal 3(feta: L1(4y myelinization: 3y MADAM 回复旦大学上海医学院儿科学系

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) - cerebra / pallium / cell number … - myelinization: fetal period ~ 4yrs. 2. Spinal Cord - weight: 2~6g (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 developmental stage Neurological signs are altered by external factors o EXamination reveals presence of abnormalities\ . ay locations of lesions and clues of etiole 回复旦大学上海医学院儿科学系

Neurological examination of children  A child’s nervous system is continuous development  Methods of neurological must be adapted to the child’s 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 language: Eye to eye contact Gestures Articulation; Understanding; Expression 4. Facies and Head: Size, shape of head Fontanelle, size tension Bruit transillumination of head 5. Neck: Movement, deformity, signs of meningeal irritation 6. Skin: Cafe au lait spots, telangiectasis, haemangiomas 回复旦大学上海医学院儿科学系

I. General observation 1. General observation: Hyperactivity, attention, emotional state 2. Consciousness: Level; Quality 3. Speech & language: Eye to eye contact ; Gestures Articulation; Understanding; Expression 4. Facies and Head: Size, shape of head; Fontanelle, size & tension; Bruit, 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, nystagmus, ptosis, 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-myotonla 回复旦大学上海医学院儿科学系

II. Cranial nerves 1. Smell 2. Vision-field, acuity, abnormal eye movement 3. Pupil size, reactions 4. Fundi 5. Ocular movement, squint, nystangmus, ptosis, 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. Observe 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, Heel-knee Rapid alternating movements 6. Involuntary movement-Chorea, athetosis, dystonia tremor, myoclonus at rest and on movement 回复旦大学上海医学院儿科学系

III. Motor Functions 1. Observe 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, Heel-knee Rapid alternating movements 6. Involuntary movement – Chorea, athetosis, dystonia tremor, myoclonus, at rest and on movement

VI ensation 1. Superficial: touch pain, temperature 2. Proprioceptive: position - joint vibration 3. Cortical: stereognosis, graphesthesia, 2 point discrimination, texture, shape, weight 4. Special: hearing( and comprehension),taste, Sight( and comprehension ), smell 5. Sensori-motor co-ordination 回复旦大学上海医学院儿科学系

VI. Sensation 1. Superficial: touch, pain, temperature 2. Proprioceptive: position – joint, vibration 3. Cortical: stereognosis, graphaesthesia, 2 point discrimination, texture, shape, weight 4. Special: hearing( and comprehension), taste, Sight ( and comprehension ), smell 5. Sensori–motor 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/ Brudzinsk signs 回复旦大学上海医学院儿科学系

V. Reflexes 1. Tendox reflexes 2. Superficial – anal, plantar 3. Clonus 4. Babinski, Gordon, Chaddock, Oppenheim, Hoffmann 5. Meningeal irritation – stiff neck, Kerning/Brudzinsk signs

1. Infectious Disease of cns ◆ 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, cryptococcus Ce ebrospirel fluid drawn trom botcon two wcrtchrso Metnig's sign bnkmncts nsc sian ADAM PADAM 國)复旦大学上海医学院儿科学系

 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, cryptococcus…… 1. Infectious Disease of CNS