Neurological examination of the newborn Author Suresh Kotagal, MD Section Editors Joseph A Garcia-Prats, MD Douglas R Nordli, Jr, MD Deputy

Editor Melanie S Kim, MD All topics are updated as new evidence becomes available and our peer review process is complete. Literature review current through: Jul 2013. | This topic last updated: Jan 10, 2013. INTRODUCTION A comprehensive neurologic assessment should be performed in any newborn suspected to have a neurologic abnormality either based upon history (eg, perinatal asphyxia) or a physical finding detected during the routine neonatal assessment. (See "Assessment of the newborn infant", section on 'Neurologic examination'.) There have been a number of publications that describe the newborn neurologic examination [1-15]. The discussion on the neonatal neurologic assessment presented here is based upon a review of the literature and the experience of the author. Other aspects of the routine assessment of the newborn are discussed separately. (See "Assessment of the newborn infant".) OVERVIEW The goals of the neurologic examination are to assist in localization of the disturbance, establish a diagnosis, and to some extent, predict the long-term outcome [16]. The following factors may alter the results of the neurologic examination and, therefore, must be taken into consideration: Conceptional age (CA) of the infant The normal neurologic examination changes with maturation. In particular, passive tone and posture vary with gestational age and are used by the Ballard score to assign gestational age (figure 1 and table 1). (See "Postnatal

assessment of gestational age", section on 'New Ballard score' and 'Passive tone/posture' below.) Level of alertness The neurologic examination varies dependent upon the level of alertness of the infant. (See 'Level of alertness' below.) Experience of the clinician Many neonatal neurologic maneuvers are dependent upon the skill of the examiner (eg, elicitation of deep tendon reflexes). Serial examinations are sometimes necessary to reliably establish the neurologic finding. The neurologic examination includes the following components: General assessment, including determining the vital signs, level of alertness, and examination of the skin, head, and spine Motor function Cranial nerves Reflexes (deep tendon, superficial, and developmental) Sensory examination Behavioral assessment GENERAL EVALUATION Key components of the general examination that are important in a neonatal neurologic evaluation include vital signs, gestational age (figure 1 and table 1), determination of whether the infant's birth weight is appropriate for gestation, thorough examination of the head and spine, and identification of dysmorphic features that suggest a congenital anomaly or inborn errors of metabolism. Other components of the general newborn assessment are discussed separately. (See "Assessment of the newborn infant".) Observation A brief period of observation before handling the infant is recommended to determine the level of alertness, the presence of involuntary movements [11], posture, and respiratory rate. (See 'Passive tone/posture' below.) Level of alertness The neurologic examination varies depending upon

the following five levels of alertness (state). Quiet sleep (also referred to as nonrapid eye movement sleep) Active sleep (also referred to as rapid eye movement sleep) Awake/drowsy (eyes open without gross movements) Alert Crying As an example, muscle tone is diminished during active sleep compared to quiet sleep. It increases in the alert infant, and is greatest in the hungry, crying infant. The optimal time for assessment is when the infant is quiet and awake, which is typically after feeding and before the infant has fallen back to sleep. In very premature infants (gestational age before 28 weeks), it is often difficult to differentiate among the various states [14]. An abnormal level of alertness is one of the most commonly observed neurologic deficits in the neonate [14]. An altered level of alertness is defined as a diminished or absent response to arousal maneuvers (ie, persistent and gentle shaking, perioral stimulation, pinching, shining a light, or ringing a bell). The following classification of altered alertness states for infants greater than 28 and 30 weeks gestation is based upon the neonatal response to arousal maneuvers and a more noxious stimulus, such as sternal rub or pressure on the supraorbital nerve [14]. Normal The infant appears awake, and has normal arousal and noxious stimuli responses. Mild stupor (lethargic) The infant appears sleepy with slightly diminished arousal and noxious stimuli responses. Moderate stupor The infant is unresponsive with moderately diminished arousal and noxious stimuli responses. Deep stupor The infant is unresponsive with an absent arousal response and a markedly diminished reaction to noxious stimuli.

 Coma The infant is unresponsive with absent arousal and noxious stimuli. Altered levels of alertness are seen in infants with cortical dysfunction. (See "Clinical features, diagnosis, and treatment of neonatal encephalopathy".) Head The examination includes measuring its size, noting the skull shape, presence of abnormal hair patterns, scalp defects, and unusual lesions or protuberances. Additional evaluation includes auscultation for bruits and transillumination. (See "Assessment of the newborn infant", section on 'Head'.) Head size The fronto-occipital head circumference (FOC) should be measured at its maximum using a cloth tape. The tape should be positioned in the front just above the nasion (ie, bridge of the nose where the frontal and nasal bones of the skull meet), and in the back, across the external occipital protuberance (picture 1). At 40 weeks gestation, the average FOC is 35 cm (10th to 90th percentile, 33 to 37 cm). Microcephaly, defined as a FOC more than 2 standard deviations (SD) below the mean (<3rd percentile), is usually due to a genetic abnormality or toxic, metabolic, or infectious insult that affects brain development (table 2 and table 3). (See "Etiology and evaluation of microcephaly in infants and children", section on 'Etiology'.) In contrast, macrocephaly is defined as a FOC greater than 2 SD above the mean (>97th percentile). Macrocephaly is caused by an increase in size of any of the components of the cranium, such as the brain parenchyma, cerebrospinal fluid, blood, or bone, or can be attributable to increased intracranial pressure (table 4). (See "Etiology and evaluation of macrocephaly in infants and children", section on 'Etiology'.) Fontanel The fontanels should be palpated, preferably with the infant in the sitting position. The following findings may be associated with a specific neurologic condition. Anterior fontanel tends to be small in infants with microcephaly, and enlarged in those with hydrocephalus, megalencephaly,

hypothyroidism, or peroxisomal disorders, such as cerebro-hepatorenal syndrome. A tense and bulging fontanel in an infant who is placed in the sitting position and is not crying may be an indication of raised intracranial pressure, which may be present in patients with a subdural hematoma, bacterial meningitis, or cerebral edema. (See "Neonatal birth injuries", section on 'Subdural hemorrhage' and "Neurologic complications of bacterial meningitis in the neonate".) Sutures The principal sutures of the skull (sagittal, coronal, lambdoid, and metopic) should be palpated. Passage through the birth canal may result in molding, a temporary asymmetry of the skull caused by overlapping or overriding of the sutures. However, an asymmetric skull that persists for longer than two to three days after birth, or a persistent palpable ridge along the suture line is abnormal, and might suggest craniosynostosis. (See "Overview of craniosynostosis", section on 'Categorization of cranial deformities'.) Scalp swelling Edema or bleeding into various compartments within the scalp and skull may be due to injuries that occur during delivery, particularly vacuum assisted deliveries. These injuries include caput succedaneum, cephalohematoma, and subgaleal hematoma, and are discussed separately (figure 2). (See "Neonatal birth injuries", section on 'Extracranial injuries'.) Auscultation Auscultation of the head may detect a venous hum, which is a benign finding. However, an asymmetric systolic-diastolic bruit that is heard over the eyeballs and anterior fontanel may indicate the presence of an arteriovenous malformation, which involves the vein of Galen. This finding is best heard by using the bell of the stethoscope. It also may also be associated with high output cardiac failure. Transillumination Transillumination of the head is performed by placing a bright light with a darkened rim on to the scalp surface in a darkened room. The normal zone of transillumination is generally 1 to 2 cm from the rim of light, and is somewhat larger over the frontal than the parieto-

occipital regions. Increased transillumination is suggestive of a fluid-filled, intracranial lesion, such as hydrocephalus, hydranencephaly, or porencephalic cyst. Spine The spine is inspected and palpated to detect abnormalities, such as a neural tube defect. Myelomeningocele is a readily apparent neurulation defect that is most often localized to the lumbar region, and less frequently at the thoracic or cervical level. (See "Pathophysiology and clinical manifestations of myelomeningocele (spina bifida)".) The gluteal folds should be separated to determine presence of a sacrococcygeal cleft or dimple, tuft of hair, hemangioma (picture 2), or discoloration may indicate an underlying postneurulation defect, such as tethered cord syndrome or diastematomyelia (split cord). (See "Assessment of the newborn infant", section on 'Trunk and spine'.) Associated findings The following findings may be associated with a congenital disorder with neurologic abnormalities: Hypopigmented skin patches may be associated with tuberous sclerosis. (See "Tuberous sclerosis complex: Genetics, clinical features, and diagnosis".) Facial hemangioma involving the ophthalmic and maxillary divisions of the trigeminal nerve distribution is suggestive of the Sturge Weber syndrome. (See "Sturge-Weber syndrome".) The combined findings of petechiae and hepatosplenomegaly are suggestive of congenital cytomegaloviral and disease in infection. (See "Cytomegalovirus infection newborns, infants,

children and adolescents", section on 'Congenital infection'.) Congenital brain anomalies may accompany infants with dysmorphic facial features, such as hypotelorism, hypertelorism, low set ears, narrow palpebral fissures, and cleft lip and/or palate. (See "Congenital cytogenetic abnormalities" and "Primary (congenital) encephalocele".) Atrophy or hypoplasia of the orbicularis oris muscle that accompanies congenital myopathies confers an inverted U-shape appearance of

the upper lip. (See "Congenital myopathies".) Arthrogryposis (contractures around multiple joints) may be associated with congenital myasthenia gravis, myopathy, or anterior horn cell disease. (See "Spinal muscular atrophy", section on 'Arthrogryposis multiplex congenita'.) MOTOR FUNCTION This is based upon evaluation of passive tone and posture, and active muscle activity. Passive tone/posture Passive tone is generally assessed by observing the resting neonatal posture, and may be measured by the resistance to passive movement of the limbs. The normal resting posture and passive tone of the neonate varies with conceptional age (CA) (figure 1 and table 1) [1,15]. The normal resting posture is elevated with the head placed in midline. It should be symmetric in all four extremities and varies with CA as follows (figure 1): 28 weeks CA All the infant's limbs are passively extended 32 weeks CA Slight flexion starting at the knee, then the hip with extended upper extremities 34 weeks CA Increasing flexion of the knee and hip with extended upper extremities 36 weeks CA Weak flexion of all four extremities 40 weeks CA (term infant) Strong flexion of all four extremities Passive tone can be measured in a supine infant starting at 28 weeks CA by using the following passive maneuvers (figure 1) [1,3]. During these manipulations, the head should remain in the midline position. Popliteal angle At 28 weeks CA, extension of the lower extremity at the knee (while the hips are still in contact with the bed) is about 150 degrees (popliteal angle), whereas in the 40 week CA term infant, the range of the popliteal angle diminishes to 80 degrees.

 Scarf sign The scarf sign measures the range of shoulder adduction. At 28 weeks CA, it is possible to adduct the shoulder and bring the elbow to touch the opposite shoulder, whereas by 40 weeks CA, passive adduction decreases so that the elbow only reaches midline. Active muscle function By 32 to 34 weeks gestation, infants should exhibit symmetric, smooth, and spontaneous movements in all extremities. Persistent asymmetry may indicate weakness of the side that is less active. As an example, the affected upper limb is generally immobile in infants with a brachial plexus lesion. (See "Brachial plexus syndromes", section on 'Congenital brachial plexus palsy'.) In most normal term infants, small amplitude, choreo-athetoid movements of the hands are seen. Common normal findings during active sleep include fragmentary myoclonus that migrates from one limb to another, facial twitches, and irregular respiratory rate. Although jitteriness or tremulousness can occur in normal awake infants, sustained tremulousness beyond the fourth day of life may be a marker of cortical dysfunction [ 4]. (See "Hyperkinetic movement disorders in children", section on 'Benign neonatal sleep myoclonus'.) Active motor tone and strength can be measured in the axial and appendicular musculature by the following: Stepping response can be obtained in infants born 32 weeks CA by holding the infant in a vertical position with his/her feet in contact with a flat surface [15]. This initiates a slow alternate stepping action of flexion and extension of the legs (figure 3). Vertical suspension measures the strength of the neonate's shoulder girdle. The examiner holds the infant in an upright position by placing the hands under the arms and around the chest with feet unsupported (figure 4). Vertical suspension is also helpful in evaluating for subtle increases in muscle tone in the lower extremities. In the presence of lower extremity hypertonicity, there may be adduction at the hips combined with hyperextension at the knees and ankles. The lower

extremities may appear to cross over each other (scissoring posture). Head control By 40 weeks CA, the infant has sufficient neck and truncal strength to maintain the head in line with the trunk for one to two seconds while being pulled from a supine to sitting position (figure 5). Ventral suspension measures the strength of the infant's trunk and neck. The infant is held in a suspended prone position in the air by placing a hand under the chest. A normal term infant will keep his/her head in the horizontal plan momentarily with flexion of both the upper and lower extremities (figure 6). Abnormal motor exam Hypotonia (decrease muscle tone) is the most common motor abnormality. When associated with weakness and absent tendon reflexes, it suggests the likelihood of a disorder of the anterior horn cells of the spinal cord or the peripheral nerves or muscle (the motor unit). When hypotonia is associated with relative preservation of mobility and with tendon reflexes that are brisk or exaggerated, it is likely that hypotonia is of the central nervous system origin, such as seen in an infant with Down syndrome. Of note, it is difficult to identify a motor abnormality in premature infants with gestational ages below 28 to 30 weeks because of their premature tone, posture, and motility. Hypotonia The hypotonic term infant lies supine in a frog-like position with the hips abducted and the limbs abnormally extended [6]. Spontaneous activity is decreased. Decreased muscle tone can also be recognized when the following are observed: Vertical suspension Decreased tone of the shoulder girdle allows the infant to slip through the examiner's hands and the legs are extended (figure 4). Ventral suspension The infant appears limp with the extended limbs and the head drooping (figure 6). Head control The head lags behind as the infant is pulled from the supine to sitting position and continues to lag when the sitting

position is reached (figure 5). Neonatal hypotonia is caused by a variety of conditions that affect the central nervous system (brain or spinal cord), peripheral nervous system, or skeletal muscles (table 5). These disorders and the approach to an infant with hypotonia are presented separately. (See "Overview of peripheral nerve and muscle disorders causing hypotonia in the newborn" and "Approach to the infant with hypotonia and weakness".) Hypertonia Hypertonia is less common than hypotonia. It is associated with dysfunction of the pyramidal or extrapyramidal systems. Spasticity is a form of hypertonia that accompanies pyramidal tract dysfunction. It is characterized by an abnormal lengthening-shortening reaction of the muscle that is most apparent in the distal portion of extremities. On passive movement of the limb, a "clasp knife" type of resistance is felt. Rigidity is associated with increased resistance to movement throughout the range of motion of the joint. It may be proximal or distal, and has the feel of bending a "lead pipe" or a "cog wheel". Sometimes it is difficult to determine whether the newborn hypertonia is due to spasticity or rigidity. Congenital brain anomalies and hypoxic-ischemic lesions are the most common etiologies of hypertonia. Opisthotonus (ie, persistent arching of the neck and trunk) is caused by decreased cerebral cortical inhibition of the labyrinthine-brainstem-spinal cord motor projections that mediate extension of the trunk and proximal extremities. It is associated with moderate to severe acute bilirubin encephalopathy or a severe, diffuse disturbance of cortical function. (See "Clinical manifestations of unconjugated hyperbilirubinemia in term and late preterm infants" and "Congenital cytogenetic abnormalities".) CRANIAL NERVES CN I and XI Although a response to peppermint smell in the form of alerting or stereotyped sucking can be elicited as early as 32 weeks CA, smell (I) is rarely tested in the neonate [17]. Cranial nerve (CN) XI is also rarely tested because it is difficult to assess the function of the sternocleidomastoid muscle in the newborn.

Cranial nerve II Visual responses mature with CA [14]: At 26 weeks CA, infants consistently blink to light. At 32 weeks CA, infants begin to show signs of fixation. At 34 weeks CA, most infants can track a fluffy ball of red wool. At 37 weeks CA, infants will turn their eyes towards a soft light. A response to an opticokinetic stimulus is present in the majority of infants by 36 weeks CA and can be observed by using commercially available drums or with tapes made of alternating black and white stripes. It is difficult to directly examine the optic fundi in the newborn because of the small pupil size. Indirect ophthalmoscopy can be requested dependent upon the clinical setting. Intact cranial nerves II and III are necessary for a pupillary light response, which is consistently present by 35 weeks gestation [14]. CN III, IV, and VI Extraocular movements can be evaluated by observation of spontaneous eye movements or by the use of the Doll's eye phenomenon, which assesses the vestibuloocular response (VOR). The Doll's eye maneuver test can be performed as early as 25 weeks CA [14]. The test entails moving the head and neck from side to side, which leads to eye deviation to the opposite side. The conjugate deviation of the eyes in the opposite direction demonstrates intact eye adduction (III) and abduction (VI). Another method to demonstrate VOR is for the examiner to spin in one direction while holding the infant upright and supporting the neck. The eyes should deviate in the opposite direction to the spin. The neck can be flexed and extended in a similar manner to the doll's test to produce vertical eye movements (IV). Abnormal eye movements The following abnormal eye movements may be associated with neurologic problems: Spontaneous, horizontal, unidirectional, jerky eye movements may be associated with a seizure originating in the opposite frontal lobe. Tonic horizontal deviation of the eyes to one side suggests a destructive

central nervous system (CNS) lesion in the hemisphere, towards which the eyes are deviated. Tonic downward gaze deviation may indicate raised intracranial pressure due to pressure in the pretectal region of the brainstem. Skew eye deviation, in which the eyes are at different horizontal planes, suggest metabolic brainstem dysfunction. It is most commonly observed after hypoxic ischemic encephalopathy. A unilaterally dilated and poorly reactive pupil in a comatose patient is a marker for ipsilateral uncal herniation. A unilaterally constricted pupil on the side of a flaccid, immobile upper extremity may indicate pupillary sympathetic paralysis due to brachial nerve injury involving the C8-T1 nerve roots (ie, partial Horner's syndrome). (See "Horner's syndrome".) Cranial nerve V The response to tactile stimuli, such as tissue paper or pinprick, over the face assesses cranial nerve V. This includes the corneal reflex in which gentle, tactile stimulation of the cornea with a tissue paper results in an ipsilateral and consensual blink response. Cranial nerve VII The normal newborn is able to close both eyelids during vigorous crying. In the presence of a peripheral VII nerve lesion, the eyelid may not close, and the nasolabial furrow may be less prominent on the affected side. In addition, sucking may be less vigorous with drooling of saliva or milk from the affected side. Cranial nerve VIII By 28 weeks gestation, the infant startles or blinks to a sudden loud noise [14]. During quiet wakefulness, term infants respond to the sound of a bell, rattle, hand clap, or voice by a startle response or an increase in their state of alertness. The consistent absence of a response does not signify hearing loss but does indicate that audiometry should be performed. (See "Evaluation of hearing impairment in children".) Sucking/swallowing Muscle function for sucking and swallowing is mediated through branches of cranial nerves V, VII, IX, X, and XII. Sucking patterns that are similar to term infants begin to appear around 33 and 34 weeks gestation. (See "Sucking and swallowing disorders in the newborn".)

CNs IX and X Impairment of cranial nerves IX and X is associated with difficulty in swallowing, diminished soft palatal movements, and a weak or absent gag reflex. Cranial nerve XII The tongue is innervated by cranial nerve XII. Atrophy and fasciculations of the tongue (slow undulating movement of the surface of the tongue) suggest degeneration of the hypoglossal nucleus, which is seen in patients with type I (infantile) spinal muscular atrophy. Deviation of the tongue to one side usually implies ipsilateral XII nerve palsy. (See "Spinal muscular atrophy".) REFLEXES Testing of reflexes provides information on the integrity of the central and peripheral nervous system. Tendon In the newborn, tendon reflexes may be difficult to obtain and interpret. The assessment is most useful when the reflexes are consistently absent or asymmetric. As with all parts of the neurologic examination, the information should be interpreted in the context of other findings. As an example, the absence of a biceps reflex is likely not significant when the infant has a normal resting posture and symmetrical spontaneous movement. The following tendon reflexes can be elicited in the newborn infant generally after 33 weeks gestation. An examiner's finger that is placed over the tendon to be tested can be lightly struck with a percussion hammer to elicit the reflex. Jaw Tapping the chin with the mouth slightly open leads to slight jaw closure. Biceps With the elbow flexed, tapping the biceps tendon in the antecubital fossa leads to flexion at the elbow. Brachioradialis (supinator) Tapping above the wrist on the radial aspect of the forearm leads to flexion at the elbow. Knee (patellar) Tapping the quadriceps tendon below the patella leads to extension of the knee. Ankle (Achilles) With the knee in the flexed position, tapping the Achilles tendon leads to flexion at the ankle.

In the newborn, it is difficult to elicit the triceps reflex because of strong flexion of the elbows in the normal neonatal resting posture. Lower motor neuron lesions are generally associated with diminished to absent tendon reflex responses, while upper motor neuron lesions elicit exaggerated responses. Altitudinal dissociation in the amplitude of the tendon reflexes is helpful in determining the level of a spinal cord lesion. An intact jaw jerk combined with absent biceps, brachioradialis, knee, and ankle reflexes in a flaccid immobile infant suggests a cervical spinal cord lesion. Similarly, the combination of normal jaw, biceps, and supinator reflexes with an absent patellar response may indicate a thoracic or lumbar spinal cord lesion. Superficial The following superficial reflexes can be elicited in the newborn: Abdominal reflexes in each of the four quadrants of the abdomen are elicited by gentle stroking in an axial to peripheral direction, resulting in contraction of the abdominal wall. The absence of response may be consistent with an ipsilateral pyramidal tract lesion. In males, the cremasteric reflex can be demonstrated by stroking the inner thigh area in an anterior to posterior direction, which results in ipsilateral scrotal retraction and testicular rise due to contraction of the scrotal dartos muscle. The lack of response or an asymmetrical response may be consistent with a corticospinal tract abnormality. Anal wink reflex is elicited by gentle stroking of the perianal region, which results in contraction of the perianal muscle. The absence of may be consistent compatible with a spinal cord lesion. Corneal reflex (see 'Cranial nerve V' above). In the newborn infant, an extensor plantar response (Babinski) may be normally elicited in a term infant when the plantar surface of the foot is stroked with a pointed but not sharp object. However, an extensor plantar response may be indicative of corticospinal tract lesions, if it is consistent and persistent on one side, and there is a

plantar flexor response on the contralateral side. Developmental Developmental reflexes, also called primitive reflexes, are mediated at the brainstem or spinal cord level and are generally present at birth (table 6) [15,18-22]. They resolve in a fairly set time course with maturation of descending, inhibitory projections from the cerebral cortex. These reflexes may persist or reappear later in life in the presence of degenerative disorders. The developmental reflexes include the following: Moro reflex Moro reflex is elicited by the sudden dropping of the infant's head in relation to the trunk. It results in abduction and extension of the infant's arms and opening of the hands, followed by flexion (figure 7). It is present starting at 32 weeks gestation, well-established by 37 weeks gestation, and disappears by three to six months of age [14]. Stepping reflex is obtained by holding the infant in a vertical position with the feet in contact with a flat surface [15]. This initiates a slow alternate stepping action of flexion and extension of the legs (figure 3). It is present starting at 32 weeks gestation and disappears by one to two months of age [14]. Grasp reflexes (palmar and plantar) are well established by 32 weeks of age [14] and disappear by three months of age (figure 8). The absence of the plantar grasp reflex in the term newborn has been reported to be associated with an increased risk of developing cerebral palsy [23]. Asymmetrical tonic neck reflex (ATNR) is characterized by extension of the upper and lower extremities on the side to which the head and neck is turned with flexion of the contralateral upper extremity (fencing posture) (figure 9). This reflex is due to decreased inhibition of the labyrinthine-brainstem-spinal cord pathway that subserves limb extension. ATNR that is seen as a resting posture rather than being elicited is never normal. ATNR appears at 35 weeks gestation,

is well-established by one month postnatal age, and disappears by three to four months of age in a term infant [14]. Galant reflex (trunk incurvation) is obtained by placing the baby in ventral suspension and then stroking the paravertebral region from the thorax to the lumbar area. This will elicit movement of the infant's trunk and hips towards the side of the stimulus. SENSORY The neonatal assessment of sensation is challenging because it is difficult to accurately determine an infant's response to sensory stimuli. As a result, sensory assessment is limited or not generally performed as part of the neonatal neurologic examination. Perioral tactile sensation can be evaluated by the rooting response in which gentle stroking of the cheek results in the infant turning towards the stimulus with an open mouth ready to latch onto the object [20]. In a patient with a spinal cord lesion, there may be loss of pinprick sensation on the trunk or neck, below the level of the lesion. This is probably the one situation in the newborn where testing sensation with a pinprick provides useful information. Otherwise, testing in the newborn with pinpricks provides little useful information. Assessment of pain is also difficult in the newborn and is discussed separately. (See "Assessment of neonatal pain".) BEHAVIORAL EVALUATION Evaluation of neonatal behavior assesses higher cortical function. Several behavioral neonatal assessment tools have been developed, including the Neonatal Behavioral Assessment Scale (NBAS). The NBAS includes 27 behavioral responses and 20 reflex items, and takes about 20 minutes to administer [2]. The following two key elements from the NBAS are incorporated into the neurological examination: Consolability Consolability is easiest to assess in a crying infant. A number of techniques are used to try to calm the infant within 15 seconds. They include infant sucking on a pacifier or the examiner's finger, holding, rocking, placing a hand on the infant's belly,

restraining one or both arms of the infant, soothing by voice, or a combination of a soothing voice and the examiner's face approaching the infant. Infants with brain injury are more difficult to console than a normal infant. Habituation Habituation is a marker of cortical inhibitory function and measures the infant's ability to learn to diminish their response to repetitive stimuli. The use of repeating light or auditory stimuli are used to test for habituation. The shining of a soft light initially elicits a blinking response that diminishes in intensity over the first four to five trials, and afterwards ceases with subsequent trials. Similarly, auditory stimuli, such as hand claps, elicit a startle response to the first four or five stimuli, and after which there is little to no response. The lack of habituation is compatible with cortical dysfunction, which may be present in an infant with prenatal substance abuse exposure. INTERPRETATION AND PROGNOSIS The newborn neurologic

examination has some limitations in its specificity and its ability to predict long-term outcome, in part due to the number of factors that can affect the results of the newborn neurologic examination. Nevertheless, abnormal neurologic findings can be useful in guiding care and predicting outcome in the following manner: It can define the site and extent of injury, which guides the choice of further management decisions. As an example, the neurologic examination is an important component in making the diagnosis of encephalopathy and determining its severity. The severity of encephalopathy guides management decisions, including the use of hypothermia. (See "Clinical features, diagnosis, and treatment of neonatal encephalopathy".) Persistent neurologic dysfunction is associated with an increased risk of permanent disability. As an example, preterm infants with abnormal neurologic examination at the time of discharge compared to those

with a normal examination have an increased risk of long-term motor impairment (eg, cerebral palsy) [24,25]. The risk of cerebral palsy increases in infants with persistent marked hypotonia, weak cry, poor sucking, and decreased level of activity. (See "Epidemiology and etiology of cerebral palsy".) Persistent asymmetric findings often are associated with an underlying abnormality, and should be further evaluated. Composites of neurologic findings appear to be better predictors of outcome than isolated neurologic findings. As an example, the Prechtl test, which is based upon classifying neurologic abnormalities into five neurologic syndromes, is reported to be a good predictor of neurological outcome at two years of age [4,26]. Results of testing performed at corrected term age are divided into normal (none of the syndromes), abnormal (presence of one or more syndromes), or suspect examinations (some symptoms of a syndrome). In one study of preterm infants (gestational age <30 weeks), the group with an abnormal Prechtl test was most likely to have abnormal Bayley Mental and Psychomotor Developmental Index scores at 24 months corrected age, followed by those with suspect results, and the normal group was least likely to have an abnormal Bayley scores [26]. SUMMARY AND RECOMMENDATIONS A comprehensive neurologic assessment should be performed in any neonate that is suspected to have a neurologic abnormality either based upon history (eg, perinatal asphyxia) or a finding during routine neonatal assessment. (See "Assessment of the newborn infant".) Findings on the neurologic examination vary with gestational age and the infant's level of alertness. Serial examinations are sometimes necessary to reliably confirm a neurologic finding. (See 'Overview' above and 'Level of alertness' above.) The newborn neurologic examination can be useful in guiding care and predicting outcome. However, there are limitations in its specificity and

prognostic ability, partly due to the number of factors that affect the results of the examination. (See 'Interpretation and prognosis' above.) The neurologic examination includes the following components: General assessment includes the vital signs, determination of the conceptional age (CA) of the infant and whether the infant's birth weight is appropriate for CA, a thorough examination of the head and spine, and identification of abnormalities suggestive of an underlying congenital disorder with associated neurologic problems. (See 'General evaluation' above.) Motor function assessment is based upon evaluation of passive tone and posture, and active motor activity. Hypotonia is the most commonly identified motor abnormality, and is due to a variety of neurologic and muscular disorders (table 2). (See 'Motor function' above.) Cranial nerve (CN) development varies with CA. In general, CNs I and XI are not tested in the neonates. The assessment of extraocular movements (CNs III, IV, VI) is based upon observation of spontaneous eye movements or the infant's response to the Doll's eyes test. Observation of the infant's sucking and swallowing assesses CNs V, VII, IX, X, and XII. Impaired gag reflex and decreased movement of the soft palate suggest dysfunction of cranial nerves IX or X. Fasciculations over the tongue are seen with degeneration of the hypoglossal (cranial nerve XII) nucleus. (See 'Cranial nerves' above.) Reflex examination provides information on the integrity of the central and peripheral nervous system, and includes evaluation of deep tendon, superficial, and developmental (also referred to as primitive) reflexes. Developmental reflexes (eg, Moro, stepping, grasp, and asymmetrical tonic neck reflex) require coordination of multiple muscles and nerves (figure 7 and figure 3 and figure 8 and figure 9). (See 'Reflexes' above.) Sensory Sensory assessment is not generally performed as part of neonatal neurologic examination because it is difficult to accurately

determine an infant's response to sensory stimuli. (See 'Sensory' above.) Behavioral Evaluation of neonatal behavior including consolability and habituation assesses higher cortical functions. (See 'Behavioral evaluation' above.)

Ventriculo-cisternostomy is an established procedure for the treatment of noncommunicating hydrocephalus, if the underlying pathology cannot be removed. In cases in which the foramina of Monro and the proximal part of the third ventricle are obstructed, it is not possible to make a communication between the internal and external CSF-spaces by perforation of the floor of the third ventricle, the so-called third ventriculo-cisternostomy. For such cases the authors describe a new method of drainage of both lateral ventricles to the interpeduncular cistern. This new method has been used successfully in a case with obstructive hydrocephalus of both lateral ventricles, caused by a pilocytic astrocytoma of the optic chiasma and hypothalamus with obstruction of both foramina of Monro.