NEUROLOGY

Assessment The process and interpretation of the neurologic examination vary with age. The examination of a newborn is unique with transient and primitive reflex patterns. The examination of an adolescent is similar to that of an adult.

Primary symptoms of neurological disease Visual or hearing loss. Impairment of swallowing or respiration. Weakness. Numbness or parasthesia. Difficulty walking or talking. Incontinence. Deterioration in thinking. Change in personality. Seizures. Headache and dizziness.

Congenital anomalies of the nervous system

Spina bifida: Meningocele : It range in severity from simple defect at L5 or S1 vertebral arch, to major defect that uncovered by skin or bone on baby's back(myelocele).or the defect involve the skull (encephalocele). Symptoms: Total paralysis , loss of sensation in the legs , incontinence of bowel and bladder. Meningocele : The spinal canal and the cystic meninges are exposed but the spinal cord is functionally and anatomically normal. Prevention: folic acid

Macrocephaly and microcephaly Macrocephaly : (large head ) 1-Macrocrania- 2-Hydrocephalus- 3-Megalencephaly- Microcephaly: (small head) Is defined as a head circumference that measures more than 3 standerd deviations below the mean for age and sex.

Etiology of microcephaly 1- familial : AD ,AR. 2-syndromes : Down , Edward, Cri-du-chat. 3- congenital infections : cytomegalovirus , rubella , toxoplasmosis. 4- drugs: fetal alcohol syndrome, radiation, meningitis / encephalitis . 5-hypoxic-ischemic encephalopathy.

CNS reflexes of infancy Moro :start at birth disappear at 4 months Grasp : Rooting : Placing : Tonic neck: Trunk incurvation: Parachute: start at 6-8 months and never disappear.

Increased intracranial pressure(ICP) A symptom of serious intracranial pathology and a cause of irreversible neurological injury. The skull is rigid container ,brain accounts for 80-85% of volume, CSF 10- 15%,blood 5-10%, Brain herniation: It occur when the brain shift in response to the continuing elevation of ICP Etiology: mass lesion , hydrocephalus , brain swelling Symptoms: headache ,vomiting , lethargy ,irritability ,6th nerve palsy , diplopia , papilledema . Sign: bulging fontanel , suture diastasis , distended scalp veins , sun set eyes , rapid growth of OFC. Cushing triad :↑ICP ,↓pulse , irregular respiration.

Hydrocephalus Physiology: Hydrocephalus is not a specific disease, it represents a diverse group of conditions that result from impaired circulation and absorption of CSF or, in rare circumstance, from increased production of CSF by a choroid plexus papilloma. Physiology: CSF is formed primarily in the ventricular system by the choroid plexus, which is situated in the lateral , 3rd and 4th ventricles. In a normal child, about 20 ml /hr of CSF is produced. The total volume of CSF approximates 50 ml in an infant and 150ml in adult. Flow from lateral v →3rd V through foramen of Monro then through aqueduct of sylvius →4th V then through foramen of Magendie and Luschka to the subarachnoid space. Absorption: by arachnoid villi.

Etiology Obstructive (noncommunicating) 1-congenital: a- Arnold-Chiari malformation . b-Dandy-Walker. c- aqueduct stenosis. 2-mass lesions: a- abscess b- hematoma c- tumors of neurocutaneous disorders d- vein of Galen malformation

Non obstructive (communicating) 1- achondroplasia 2-beningn enlargement of subarachnoid space 3-choroid plexus papilloma 4-meningeal malignancy. 5-meningitis. 6-posthemorrhagic.

Clinical feature 1-symptoms&sign of ↑ICP. 2-ataxia&spasticity (lower limb) 3-endocrine dysfunction. 4-visual dysfunction. 5-˝sun set eyes˝. 6-dilated scalp veins. 7-widly opening fontanel. 8-rappid ↑in OFC by serial measures. 9- percussion of the skull might produce a cracked pot sound or McEwan's sigh.

Diagnosis 1-clinically 2-imiging : * Plain skull films show separation of the sutures (beaten -silver appearance) *The CT scan and /or MRI along with the ultrsonography in an infant are the most important studies to identify the specific cause and severity of hydrocephalus.

Treatment Medical : Surgery : acetazolamide -shunt placing. (Meningitis, subarachnoid Hr) acetazolamide Surgery : -(cyst ,tumer ,AVM) Removal. -shunt placing.

Dandy-walker

Floppy baby

Neonatal immobility: Clinical questions: The 1st step in evaluations of an infant who doesn't move spontaneously or in response to stimuli is to determine whether awareness is intact ? The cortical activity is intact if the baby is bright eyed able to follow and try to smile but is unable to move . If the face and eyes are immobile the problem is more difficult . Clinical questions: Does the infant response to :flashlight , bell , nasal cotton stimuli? Does the eyes follow if the lids are lifted? Are reflexes increase?

Neuromuscular disease or If the clinical exam. Doesn't settle the question then EEG will be helpful EEG Sever slowing of EEG +↑reflex Normal or near normal EEG and immobility+ ↓reflex Cerebral disease Neuromuscular disease or Spinal cord disorder

Etiology of neonatal immobility Sever hypoxic ischemic encephalopathy: Flaccidity Areflexia Complete ptosis Ophthalmoplegia Absent cornea l& gag reflex Intoxication : Mg sulfate Barbiturate Narcotics Benzodiazepines General anesthesia Metabolic encephalopathy: Hypoglycemia hyperbilirubinemia

Hypotonia without major weakness *acute systemic illness. *Mental retardation: *Specific syndromes: -Down syndrome -Cerebrohepatorenal syndrome -Kinky hair syndrome -Prader-willi syndrome *Connective tissue disorder: -Ehlers-Danlos syndrome -Marfan syndrome

*Nutritional-metabolic disease: Rickets Renal tubular acidosis Celiac disease Biliary Artesia Congenital heart disease *Benign congenital hypotonia: Exhibit the condition at 9-12 months. Delayed motor milestone. Normal social , intelligence , fine motor movement. Head lag , slip-through ventral suspension. Complete lab. Investigation are necessary. Most of children become normal by 3 yrs.