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Intraoperative neurophysiological monitoring in pediatric neurosurgery: why, when, how?

INTRODUCTION: This review is primarily based on peer-reviewed scientific publications and on the authors' experience in the field of intraoperative neurophysiology. The purpose is a critical analysis of the role of intraoperative neurophysiological monitoring (INM) during various neurosurgical procedures, emphasizing the aspects that mainly concern the pediatric population. Original papers related to the field of intraoperative neurophysiology were collected using medline. INM consists in monitoring (continuous "on-line" assessment of the functional integrity of neural pathways) and mapping (functional identification and preservation of anatomically ambiguous nervous tissue) techniques. We attempted to delineate indications for intraoperative neurophysiological techniques according to their feasibility and reliability (specificity and sensitivity). DISCUSSION AND CONCLUSIONS: In compiling this review, controversies about indications, methodologies and the usefulness of some INM techniques have surfaced. These discrepancies are often due to lack of familiarity with new techniques in groups from around the globe. Accordingly, internationally accepted guidelines for INM are still far from being established. Nevertheless, the studies reviewed provide sufficient evidence to enable us to make the following recommendations. (1) INM is mandatory whenever neurological complications are expected on the basis of a known pathophysiological mechanism. INM becomes optional when its role is limited to predicting postoperative outcome or it is used for purely research purposes. (2) INM should always be performed when any of the following are involved: supratentorial lesions in the central region and language-related cortex; brain stem tumors; intramedullary spinal cord tumors; conus-cauda equina tumors; rhizotomy for relief of spasticity; spina bifida with tethered cord. (3) Monitoring of motor evoked potentials (MEPs) is now a feasible and reliable technique that can be used under general anesthesia. MEP monitoring is the most appropriate technique to assess the functional integrity of descending motor pathways in the brain, the brain stem and, especially, the spinal cord. (4) Somatosensory evoked potential (SEP) monitoring is of value in assessment of the functional integrity of sensory pathways leading from the peripheral nerve, through the dorsal column and to the sensory cortex. SEPs cannot provide reliable information on the functional integrity of the motor system (for which MEPs should be used). (5) Monitoring of brain stem auditory evoked potentials remains a standard technique during surgery in the brain stem, the cerebellopontine angle, and the posterior fossa. (6) Mapping techniques (such as the phase reversal and the direct cortical/subcortical stimulation techniques) are invaluable and strongly recommended for brain surgery in eloquent cortex or along subcortical motor pathways. (7) Mapping of the motor nuclei of the VIIth, IXth-Xth and XIIth cranial nerves on the floor of the fourth ventricle is of great value in identification of "safe entry zones" into the brain stem. Techniques for mapping cranial nerves in the cerebellopontine angle and cauda equina have also been standardized. Other techniques, although safe and feasible, still lack a strong validation in terms of prognostic value and correlation with the postoperative neurological outcome. These techniques include monitoring of the bulbocavernosus reflex, monitoring of the corticobulbar tracts, and mapping of the dorsal columns. These techniques, however, are expected to open up new perspectives in the near future.

Brain Mapping↗

Prediction of the neurophysiological diagnosis of carpal tunnel syndrome from the demographic and clinical data.

OBJECTIVE: To use demographic and clinical data to identify the clinical pattern that best predicts the diagnosis of carpal tunnel syndrome (CTS), as defined by neurophysiologic studies. METHODS: A diagnostic cross-sectional study in 2535 consecutive patients (3907 upper limbs) older than 12 years old who were referred for nerve conduction studies in the upper limbs between August 2001 and January 2003 in 3 university hospitals and 2 private neurophysiology services in the state of Rio Grande do Sul, Brazil. RESULTS: A neurophysiologic diagnosis of CTS was established in 39.1% of these upper limbs. The presence of paresthesias or pain at least 2 of the first 4 digits in association with one of the following: female gender, symptoms worsening at night or on awakening, an BMI > or =30, thenar atrophy, or other sign (Tinel's, Phalen's, or Reversed Phalen's signs); were the best pattern associated with the diagnosis. CONCLUSIONS: We have found that the clinical picture alone does not seem sufficient, in majority of the population, to correctly predict the diagnosis of CTS, as defined by median nerve neuropathy at the carpal tunnel. We believe that a compressive lesion of the median nerve at the carpal tunnel can be present both in patients with no typical symptoms of CTS (including asymptomatic individuals) and in patients in which neurophysiologic studies are negative. SIGNIFICANCE: Further studies separating patients into these groups will allow us to identify the long-term prognosis as well as the ideal therapeutic approach for each of these clinical situations.

Adult↗

Ventilatory effects of neurophysiological facilitation and passive movement in patients with neurological injury.

Thirteen intubated, high dependency patients with neurological injuries were studied in order to investigate the short term respiratory effects of neurophysiological facilitation and passive movement on tidal volume (VT), minute ventilation (VE), respiratory rate (VR) and oxygen saturation (SpO2). The subjects were studied under four conditions: no intervention (control) and during periods of neurophysiological facilitation, passive movement and sensory stimulation. All periods were standardised to three minutes duration and all parameters were recorded before and after each intervention. Neurophysiological facilitation produced significant increases (p < 0.01) in VE and SpO2 (p < 0.05) when compared with control values, with an overall mean increase in VE of 14.6%. Similarly, passive movement increased VE (p < 0.01) by an average of 9.8% and also increased SpO2 (p < 0.01). In contrast, sensory stimulation produced significant increases (p < 0.01) in SpO2 with control levels, with no significant change in VT or VE. There was no significant difference in VR with all treatments. This study provides preliminary evidence of improved short term ventilatory function following neurophysiological facilitation, independent of generalised sensory stimulation, which has not been previously examined in the literature, supporting its use in the management of high dependency neurological patients.

Adolescent↗

Meningomyelocele: a clinical, urodynamic and neurophysiological evaluation.

A total of 11 children with chronic meningomyeloceles underwent a clinical neurological, urodynamic and neurophysiological evaluation to define further the level and type of lesion present in the lumbosacral spinal area. The neurophysiological evaluation was correlated with detrusor activity. Detrusor hyperreflexia was present in 45 per cent of the patients and functional activity of the somatic sacral reflex arc was demonstrated in 4. Absolute interruption of the somatic sacral reflex arc was found in the remaining patients with detrusor areflexia. All patients had evidence of a lower motor neuron lesion on clinical neurological evaluation, with weak or absent myotatic and musculocutaneous reflexes. Electromyographic evaluation revealed signs of severe partial to complete denervation of the affected muscle groups. However, increased motor unit potentials were observed in 5 patients after coughing and stimulation of the perianal skin. The bulbocavernosus reflex was absent clinically and electrophysiologically in all 6 patients tested. However, this finding did not indicate absolute interruption of the reflex arc, since further temporal and spatial stimuli resulted in an increase in motor unit potentials. Somatosensory cortical evoked potentials were useful in demonstrating an intact afferent input system to the spinal cord and brain area. However, lumbosacral evoked potentials appeared to be the least useful neurophysiological method to demonstrate partial preservation of the input to the spinal cord level. Therefore, detrusor and external urethral sphincter function may be documented on urodynamic and neurophysiological evaluation when the clinical examination has revealed a complete lower motor neuron lesion. The complexity of the lesion caused by the malformation in patients with meningomyelocele is shown.

Adolescent↗

[Postural tremor: clinical and neurophysiological study in a consecutive series of 300 patients].

BACKGROUND: There are few studies analysing the clinical and neurophysiological characteristics of postural tremor in Spain. PATIENTS AND METHOD: We studied prospectively 300 consecutive patients referred to a Movement Disorders Section because of postural tremor of the upper limbs. Syndromic diagnosis of postural tremor was made according to clinical criteria with the aid of neurophysiological criteria (accelerometric and EMG data). In patients diagnosed with essential tremor (ET), diagnostic sensitivity studies, correlation studies of clinical and neurophysiological data and multivariate analysis were performed. RESULTS: Most frequent syndromic diagnoses were ET (77%), parkinsonian postural tremor (10%) and exaggerated physiological tremor (6%). Fifty percent of ET patients reported having affected relatives, and 7% reported that their tremor improved with alcohol. Mostly specific variables for the diagnosis of ET were: affected relatives (77%), cephalic tremor (80%), alcohol improvement (100%), and synchronous EMG pattern (79%). The presence of affected relatives and a synchronous EMG pattern were significant predictive variables on a multivariate analysis. We found a significant correlation between age at time of consulting and frequency (rs = 0.561; p < 0.0005) and amplitude (rs = 0.470; p < 0.0005) of tremor. CONCLUSIONS: In the present study, ET was the most common cause of reference for postural tremor. Selective clinical data and neurophysiological evaluation are useful for the diagnosis of postural tremor.

Aged↗

[Neurophysiologic study of tremor].

Tremor is frequently encountered in neurologic practice. Clinical examination supplies information regarding its approximative frequency, regularity, amplitude, topography and activation conditions. The neurophysiological study helps in confirming the tremor, in differentiating it from other movement disorders like myoclonus, and may provide distinctive features which are important for the aetiological diagnosis. The neurophysiological investigation includes accelerometry, which analyses the mechanics of the movement. Spectral analysis with FFT allows the determination of frequency and amplitude. Accelerometry is always associated with surface EMG of at least two antagonistic muscles. It may show rhythmic bursts, their frequency, duration and activation pattern (alternating or synchronous). This neurophysiological approach to tremor has multiple interests. It may help in differentiating a true rest tremor from a postural tremor seemingly persisting at rest. Brief interruptions or rhythm breaks during distraction manoeuvers are seen in psychogenic tremors. Surface EMG may also demonstrate positive myoclonic bursts, or brief silent periods corresponding to negative myoclonus, sometimes pseudorhythmic, thus appearing clinically like a tremor, but investigations, aetiologies and treatment are different. Several features, especially slow frequency, may suggest a midbrain tremor, thus requiring brain imagery centered around the posterior fossa. Finally, the neurophysiological examination is the only way to demonstrate a primary writing tremor, or a primary orthostatic tremor, the frequency of which is pathognomonic while clinical symptoms are rather misleading.

Diagnosis, Differential↗

Interventional neurophysiology of the sacral nervous system.

Clinical neurophysiological tests have been introduced for the sacral neuromuscular system to aid with diagnosis of neurogenic conditions involving the lower urinary tract, anorectal and sexual dysfunction. The tests have, however, the potential to be of value in different interventions outside of the neurophysiological laboratory. EMG monitoring can be used for exact application of botulinum toxin by the relatively non-invasive transcutaneous approach in treatment of male detrusor sphincter dyssynergia. Checking for compound muscle action potentials of the external anal sphincter is proposed as the best method for exact placement of wire electrodes close to the 3rd sacral roots in treating lower urinary tract dysfunction by 'neuromodulation'. Presently the most established use of clinical neurophysiological techniques--outside the laboratory--as related to the sacral neuromuscular system is in the operating theatre. These tests have been introduced to identify relevant structures, for instance pudendal afferents within dorsal sacral roots, which should be spared during rhizotomy procedures for treatment of spasticity. Modified techniques are used intraoperatively to monitor the integrity of the lower sacral reflex arc (the bulbocavernosus reflex) or the lower sacral afferents throughout the spinal cord (pudendal SEP). Clinical neurophysiological tests are expected to become established in several interventions involving the sacral neuromuscular system.

Botulinum Toxins↗

Neurophysiological evaluation of children with traumatic radiculopathy, plexopathy, and peripheral neuropathy.

Neurophysiological evaluation of children with traumatic peripheral nervous system injury is accomplished with electromyography, motor and sensory nerve conduction studies, and somatosensory and motor-evoked potentials. Preoperative neurophysiological tests are performed if motor deficits persist for more than 3 months. Evidence of reinnervation on these neurophysiological tests predates clinical recovery by weeks, hence they help determine the site of the lesion and provide objective measures for selecting candidates for surgical exploration. Intraoperative neurophysiological tests help to identify and confirm the integrity of nerves and to develop the optimal surgical strategy. Postoperative evaluations every 3 to 6 months may determine efficacy of treatment.

Brachial Plexus Neuropathies↗

Neurophysiological evaluation of late effects of adjuvant high-dose chemotherapy on cognitive function.

OBJECTIVES: To evaluate late neurotoxicity of adjuvant high-dose (HD) chemotherapy versus standard-dose (SD) chemotherapy by event-related potentials (ERP) and quantitative electroencephalography (qEEG). PATIENTS AND METHODS: From a randomized study in high-risk breast cancer patients on the efficacy of high-dose versus standard-dose adjuvant chemotherapy, late effects on cognitive functioning were analyzed by neuropsychological tests. Cognitive impairment was found in 32% of the HD group, 17% of the SD group and in 9% of a control group of stage I breast cancer patients not treated with chemotherapy. In 17 consecutive patients in the HD group and 16 consecutive patients in the SD group neurophysiological tests were performed, consisting of P300 and qEEG. Results of patients treated with chemotherapy were compared with results of 14 control patients not treated with chemotherapy. All patients were tested two years after treatment. RESULTS: Asymmetry of the alpha rhythm of > or =0.5 Hz was found in 7 HD patients, 2 SD patients and in none of the control patients (p = 0.01). No differences were found between the groups with regard to frequency of alpha rhythm, alpha blocking and latency of P300. No correlation was found between neurophysiological parameters and neuropsychological performance, except for an overall relation between the P300 latencies and the total number of deviant test scores. CONCLUSION: Although the neurophysiological differences are subtle and the relation with the cognitive functioning in individual patients as measured by the neuropsychological examination is equivocal, the results suggest that there is neurophysiological support for cognitive dysfunction as a late complication of high-dose systemic chemotherapy in breast cancer.

Alpha Rhythm↗

The effect of flumazenil on subclinical psychometric or neurophysiological alterations in cirrhotic patients: a double-blind placebo-controlled study.

It is not yet clear if benzodiazepine receptor ligands, implicated in the pathophysiology of hepatic coma, also have a role in subclinical cognitive or neurophysiological alterations in cirrhotic patients. Therefore, we carried out a double-blind, placebo-controlled study to evaluate the effectiveness of flumazenil, a benzodiazepine antagonist, on brainstem auditory evoked responses and on the number connection test in cirrhotic patients with subclinical neurophysiological or cognitive alterations. Thirteen cirrhotic subjects with subclinical neurophysiological or cognitive alterations were studied. A total of 3 mg of flumazenil or saline was infused intravenously. Before and after the infusion, the number connection test was administered and brainstem auditory evoked responses recorded. After 72 h, patients were crossed over. Flumazenil did not influence brainstem auditory evoked responses or the number connection test. A screening test for benzodiazepines was negative in all subjects. We conclude that benzodiazepine receptor ligands have a negligible role, if any, in the pathophysiology of subclinical neurophysiological or cognitive alterations of cirrhotic patients.

Cognition↗

Neurophysiology not required before surgery for typical carpal tunnel syndrome.

Sixty-eight patients with typical carpal tunnel syndrome underwent neurophysiological investigations preoperatively, but these were not assessed until the end of the study. Open carpal tunnel release was performed and the clinical diagnosis of carpal tunnel syndrome was considered as confirmed when there was a prompt resolution of the preoperative symptoms. Sixty-three of the 68 patients responded well to surgery, three had equivocal outcomes and two did not improve, and thus were considered not to have carpal tunnel syndrome. The neurophysiological tests were normal in these two patients, but were also normal in 14 of the 63 patients who improved with carpal tunnel surgery. Preoperative neurophysiology might therefore have led to up to 14 of the 63 cases of carpal tunnel syndrome being turned down for surgery. We conclude that neurophysiological studies contribute little to the diagnosis in typical cases of carpal tunnel syndrome, and are more often confounding than of assistance.

Adult↗

Cobalamin (Cbl) C/D deficiency: clinical, neurophysiological and neuroradiologic findings in 14 cases.

The early onset type of cobalamin (Cbl) C/D deficiency is characterised by feeding difficulties, failure to thrive, hypotonia, seizures, microcephaly and developmental delay. It has an unfavourable outcome, often with early death and significant neurological impairment in survivors. While clinical and biochemical features of Cbl C/D deficiency are well known, only a few isolated case reports are available concerning neurophysiological and neuroimaging findings. We carried out clinical, biochemical, neurophysiological and neuroradiologic investigations in 14 cases with early-onset of the Cbl CID defect. Mental retardation was identified in most of the cases. A variable degree of supratentorial white matter atrophy was detected in 11 cases by MR imaging and tetraventricular hydrocephalus was present in the remaining 3 patients. Waking EEG showed a clear prevalence of epileptiform abnormalities, possibly related to the high incidence of seizures in these cases. Increased latency of evoked responses and/or prolongation of central conduction time were the most significant neurophysiological abnormalities. The selective white matter involvement, shown both by neuroradiologic and neurophysiological studies, seems to be the most consistent finding of Cbl C/D deficiency and may be related to a reduced supply of methyl groups, possibly caused by the dysfunction in the methyl-transfer pathway.

5-Methyltetrahydrofolate-Homocysteine S-Methyltran↗

Neurophysiological function in farm workers exposed to organophosphate pesticides.

To investigate neurophysiological effects of low-level exposure to foliar organophosphate residues during one season among agricultural workers, the authors performed a cross-sectional study of 67 Hispanic farm workers and 68 age-, gender-, ethnicity-, and education-matched reference subjects. The neurophysiological examination included sensory and motor nerve conduction and neuromuscular junction testing. Erythrocyte cholinesterase activity was measured at the time of examination. No statistically significant neurophysiological differences between the exposed and reference groups were observed. Farm workers and reference subjects had similar sensory nerve latency and amplitude (sural), motor nerve conduction velocity (ulnar), and neuromuscular junction function (ulnar). No relationship between duration of exposure during the season and electrophysiological measures of nerve function was found. Exposure of farm workers to the low levels of organophosphate pesticides during one season experienced by farm workers in this study was not associated with impaired peripheral neurophysiological function.

Adolescent↗

Neurophysiological assessment of the feasibility and safety of neural tissue transplantation in patients with syringomyelia.

The feasibility and safety of a procedure involving fetal spinal cord tissue transplantation in patients with syringomyelia was assessed using a neurophysiological protocol designed to quantitate peripheral nerve function, spinal cord reflex excitability, and spinal cord conduction pathways essential for somatosensory evoked potentials. We report here data obtained before and for 18 months following the transplantation procedure performed on the first two patients in this study. The neurophysiological assessment protocols included measures of cortical and spinal cord evoked potentials, H-reflex excitability, and peripheral nerve conduction. Prior to the procedure, both patients had significant deficits on some of the neurophysiological measures, for example, lower extremity cortical evoked potentials. However, robust measures of intact pathways, such as upper extremity cortical evoked potentials, were also observed preoperatively in both patients. Thus, it was anticipated that conduction in these intact pathways could be at risk either from complications from the transplantation procedure and/or from continued expansion of the syrinx. Following the transplantation procedure, no negative changes were observed in any of the neurophysiological measures in either patient. In addition, patient 1 showed a decrease in the rate potentiation of tibial H-reflexes on the right side and an increase in the response probability of left tibial H-reflexes. The results of this postoperative longitudinal assessment provide a first-level demonstration of the safety of the intraspinal neural tissue transplantation procedure. However, the consideration of safety is currently limited to the grafting procedure itself, since the long-term fates of the donor tissue in these two patients remain to be shown more definitively.

Action Potentials↗

Characterizing impaired functional alertness from diphenhydramine in the elderly with performance and neurophysiologic measures.

STUDY OBJECTIVES: Psychometric task performance measures can be highly sensitive to manipulations that impair functional alertness in young adults; such measures have been shown to be less sensitive to reduced alertness in older adults. The purpose of this study is to determine whether neurophysiologic measures can aid in the detection and characterization of impairments in functional alertness in the elderly. DESIGN: Double-blind, placebo-controlled, counter-balanced, crossover study. SETTING: Research laboratory. PARTICIPANTS: Twelve healthy older adults (62-75 years of age). INTERVENTIONS: Diphenhydramine, 50 mg. MEASUREMENTS AND RESULTS: Behavioral performance and electroencephalogram measures were obtained while participants completed spatial working memory and word recognition episodic memory tasks in a baseline interval before drug ingestion and in 4 hourly test intervals following drug ingestion. Relative to placebo, diphenhydramine had marginal effects on task performance yet was effective in reducing alertness, as evidenced by subjective ratings and objective neurophysiologic (electroencephalogram) markers. Diphenhydramine significantly reduced the amplitude of alertness-sensitive event-related potentials recorded during working memory task performance, including the N160 and P300. It also affected neurophysiologic processes underlying episodic memory, as evidenced by a reduction in the difference in event-related potentials between old and new words in the word recognition task. Discriminant analyses incorporating neurophysiologic measures showed that the time course of the central effects of diphenhydramine in older subjects was similar to that previously observed in young adults. CONCLUSIONS: These results demonstrate that diphenhydramine has adverse neurocognitive effects in elderly individuals and highlights the utility of incorporating direct measures of brain function into assessments of functional alertness.

Aged↗

Interventional neurophysiologic monitoring.

PURPOSE OF REVIEW: Intraoperative neurophysiologic monitoring provides useful information on the functional status of the nervous system. This review focuses on recently published data concerning the impact of monitoring on patient outcome. RECENT FINDINGS: There is level I evidence to support the use of bispectral index monitoring to prevent awareness during anesthesia in high-risk patients. A number of randomized trials have shown that monitoring-guided anesthesia using the bispectral index or other devices will expedite recovery and improve perioperative drug utilization. There are also preliminary reports suggesting that anesthesia dictated by bispectral index monitoring may alter long-term outcome and reduce mortality. In surgical procedures, however, it is less clear whether neurophysiologic monitoring will improve patient outcome. Currently, the majority of data are derived from respective case series. Nonetheless, monitoring with somatosensory evoked potential has been shown to reduce postoperative neurologic deficits after spinal surgery. There is also evidence to suggest that electromyography and motor evoked potential are essential complements to somatosensory evoked potential for monitoring of spinal cord surgery. SUMMARY: Brain monitoring facilitates anesthetic drug administration. An increasing number of neurosurgical procedures will require some form of intraoperative neurophysiologic monitoring to achieve higher degrees of safety and accuracy. In many instances, the data derived from monitoring will guide and influence surgical decisions. In this context, neurophysiologic monitoring should be regarded as interventional.

Journal Article↗

Neurophysiologic studies in Morvan syndrome.

This study was conducted to clarify the clinical and neurophysiologic characteristics of patients with Morvan syndrome, and to compare and contrast this syndrome with other forms of autoimmune encephalitis. A retrospective chart review of the clinical features and neurophysiologic studies of two cases of Morvan syndrome seen at the Mayo Clinic was performed. Neurophysiologic studies included polysomnography, comprehensive autonomic testing, MRI, positron emission tomography, EEG, and single-photon emission computed tomography. In two cases of Morvan syndrome, the clinical features, electrophysiologic findings, and immunologic studies (high levels of voltage-gated potassium channel antibodies) were consistent with previously reported findings. Several novel observations were made. Autonomic testing demonstrated peripheral autonomic neuropathy in addition to autonomic hyperactivity. Polysomnography showed complete absence of sleep. Neuroimaging study findings were largely normal. Morvan syndrome is an autoimmune disorder affecting both the peripheral and central nervous system. Neurophysiologic studies demonstrate hyperexcitability of peripheral nerves, autonomic dysfunction, and severe insomnia. The absence of abnormalities on imaging studies suggests that central nervous system symptoms are related to functional rather than structural disruption of neural networks.

Adult↗

Maternal panic disorder: infant temperament, neurophysiology, and parenting behaviors.

OBJECTIVE: To determine whether 4- and 14-month-old infants of mothers with panic disorder (PD) would be more likely to show differences in temperament, neurophysiology (salivary cortisol and sleep), and relationships with their mothers than controls. METHOD: Two cohorts were recruited: 4-month-old infants with PD mothers (n = 25) and 4-month-old controls (n = 24), and 14-month-old infants with PD mothers (n = 27) and 14-month-old controls (n = 18). Mothers completed diagnostic interviews and questionnaires concerning infant temperament, sleep, and parenting. Infant salivary cortisol samples and standard observational procedures to measure infant temperament, sleep, attachment, and parenting were also used. RESULTS: Infants with PD mothers did not show more high reactivity, behavioral inhibition, or ambivalent/resistant attachment but did demonstrate different neurophysiology (higher salivary cortisol and more disturbed sleep) than controls. PD mothers also displayed less sensitivity toward their infants and reported parenting behaviors concerning infant sleep and discipline that have been associated with child problems. CONCLUSIONS: While infants with PD mothers did not show early behavioral differences from controls, they did display neurophysiological divergences consistent with higher arousal/arousability. Such neurophysiological divergences (elevated salivary cortisol and disturbed sleep) might be important early indicators of risk. Helping PD mothers parent their more highly aroused/arousable infants could reduce the development of psychopathology.

Adult↗