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Aatif M Husain

Publications and source records attributed to Aatif M Husain.

17 recordsLinked to original sources

Neurophysiologic assessment of mucopolysaccharidosis III.

OBJECTIVE: To describe finding of various neurophysiologic tests in patients with mucopolysaccharidosis III (MPS III) early in the disease course. METHODS: Patients were evaluated with flash visual evoked potentials (VEP), brainstem auditory evoked potentials (BAEP), electroencephalography (EEG), and nerve conduction studies (NCS) before they underwent hematopoietic stem cell transplantation (HSCT). RESULTS: Thirteen children underwent at least one neurophysiologic test before HSCT. The mean age at testing was 2.7 years. Ten of 11 (91%) patients had a normal flash VEP, and all 9 who had BAEP had normal central conduction. EEG was normal in 7/13 (54%), with the others showing diffuse slowing. NCS was normal in 10/11 (91%) patients. CONCLUSIONS: Despite extensive central nervous system involvement in MPS III, flash VEP and BAEP are almost always normal. EEG is often abnormal early in the disease. SIGNIFICANCE: This is the first report of neurophysiologic tests in a large series of MPS III patients.

Child, Preschool↗

Guest editorial.

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Brain Diseases↗

Electroencephalographic assessment of coma.

SUMMARY: Altered mental status ranging from confusion to deep unresponsiveness can be described as coma. Electroencephalography is an important tool in assessing comatose patients. Some EEG patterns are seen with lighter stages of coma and have a good prognosis, whereas others are seen in deep, often irreversible coma. These EEG patterns carry a much more grave prognosis. This paper discusses the various EEG features seen in coma, ranging from intermittent rhythmic delta activity to electrocerebral inactivity. A discussion regarding etiology and prognosis is presented after the EEG pattern is described in detail. Special EEG features, such as alpha coma, beta coma, spindle coma, etc., are discussed toward the end.

Coma↗

Normal adult EEG and patterns of uncertain significance.

A thorough understanding of a normal EEG is critical in defining those patterns that are abnormal. Because EEG is unique in the ability to support a clinical diagnosis of epilepsy, epileptiform patterns merit careful consideration. Certain benign patterns maybe epileptiform, yet can occur in healthy individuals without epilepsy. Understanding normal EEG and the benign variants will help to minimize over-interpretation and possibly avoid overtreatment of patients during routine clinical practice.

Brain↗

Brainstem auditory evoked potential monitoring: when is change in wave V significant?

BACKGROUND: The probability of hearing loss during cerebellopontine angle (CPA) surgery can be reduced by using brainstem auditory evoked potential (BAEP) intraoperative monitoring (IOM). A wave V latency prolongation of 1.0 milliseconds or amplitude decrement of greater than 50% is arbitrarily considered the point when damage to hearing occurs. OBJECTIVE: To determine the accuracy of wave V changes in predicting hearing impairment. METHODS: Patients undergoing BAEP IOM for surgery in the CPA region were evaluated. The greatest wave V latency and amplitude change was determined. Patients were divided into four groups depending on degree of change of wave V: Group 1 consisted of minimal change, whereas Group 4 was permanent loss of wave V. The frequency of hearing loss in each group was compared. RESULTS: Data from 156 patients were reviewed. When all patients were analyzed, the frequency of hearing loss was not significantly different between the groups. When patients with CPA tumor were excluded, a significantly higher number of patients in Group 4 had hearing loss. Analysis of the patients with CPA tumor showed no difference in the frequency of hearing loss in any of the groups; even a large number (50%) of Group 1 patients had hearing impairment. CONCLUSIONS: During brainstem auditory evoked potential intraoperative monitoring, the type of surgery is important when interpreting significance of changes of wave V. For non-cerebellopontine angle tumor surgery, hearing loss occurs usually only with permanent loss of wave V; much smaller changes may be important in cerebellopontine angle tumor surgery.

Acoustic Stimulation↗

Review of neonatal EEG.

Neonatal electroencephalography (EEG) presents some of the most difficult challenges in EEG interpretation. It differs significantly in many ways from EEG of older children and adults. Technologically, acquisition of a neonatal EEG is significantly more difficult and different than an adult EEG. There are numerous features that are age-specific and change almost week-to-week in the preterm infant. Some features may be normal at one age and abnormal if they persist for several weeks. Many of these features also have different implications in neonates as compared to older individuals. These issues mandate a different approach to neonatal EEG interpretation. In this article an overview of neonatal EEG is presented. After a brief discussion of relevant technical issues, various normal EEG features encountered in neonates are discussed. This is followed by a discussion of the ontogeny of EEG, starting from the age of viability to the first few months of life. A description of various abnormalities follows. Finally, an approach to analysis of a neonatal EEG is presented.

Brain Diseases↗

Krabbe disease: neurophysiologic studies and MRI correlations.

BACKGROUND: Krabbe disease (KD) is a rare hereditary leukodystrophy affecting children mostly in the first 6 months of life; later onset has been reported as well. OBJECTIVE: To review abnormalities in neurophysiologic studies in children with KD and determine if there is a correlation between these studies and disease severity as measured by MRI scans. METHODS: KD patients with at least one neurophysiologic study and one MRI scan at the authors' institution were reviewed. Relationships between KD type, neurophysiologic studies, and severity of disease as measured by MRI were explored. RESULTS: Data were available for 26 children: 20 with early infantile KD (EIKD) and 6 with late-onset KD (LOKD). Flash visual evoked potentials were abnormal in 53% of EIKD children, whereas none of the LOKD children had an abnormal study. Brainstem auditory evoked potentials were abnormal in 88% of EIKD and 40% of LOKD children. EEGs were abnormal in 65% of EIKD and 33% of LOKD children. Nerve conduction studies were abnormal in all children with EIKD and in 20% of LOKD children. Abnormal neurophysiologic studies correlated with more extensive disease as measured by MRI scans. CONCLUSIONS: Children with early infantile Krabbe disease and late-onset Krabbe disease have different patterns of abnormalities in neurophysiologic studies. These studies offer an objective means of assessing KD and correlate well with disease severity measured by MRI scans.

Adolescent↗

CPAP compliance in sleep apnea patients with and without laboratory CPAP titration.

Advances in auto-adjusting positive airway pressure technology for obstructive sleep apnea now permit this treatment to be initiated outside of the sleep laboratory environment, bypassing the need for laboratory-based titration studies. Thus far, little research has addressed how such developments may affect compliance to continuous positive airway pressure (CPAP). We tested the effect of laboratory CPAP exposure and technologist support in a retrospective chart review of 98 veterans with obstructive sleep apnea to determine whether patients who received standard laboratory CPAP titration complied better with CPAP than did patients who received no laboratory CPAP titration. Fifty patients underwent standard technician-attended polysomnography (PSG) with CPAP titration, and 48 patients underwent unattended PSG with no laboratory trial of CPAP (first CPAP exposure was at home). Objective CPAP compliance measures were obtained from CPAP units at follow-up visits. Attended-PSG patients wore CPAP significantly longer per night on average (5.0 hours vs 3.9 hours) and tended to wear CPAP on more nights (76.5% vs 64.2%) compared with unattended-PSG patients. These findings suggest that patients' sleep laboratory experience with CPAP and the support and education provided by sleep technologists are important factors in facilitating CPAP compliance.

Continuous Positive Airway Pressure↗

Brain-stem auditory and visual evoked potentials in children with Krabbe disease.

OBJECTIVE: There are few reports in the literature detailing brain-stem auditory and visual evoked potentials (BAEP and VEP) in children with Krabbe disease (KD). The purpose of this study was to provide a descriptive analysis of the BAEP and VEP findings in these children. METHODS: Charts of children with KD were reviewed. BAEP and VEP studies performed on these children were reviewed; findings in the early infantile and late onset KD (EIKD, LOKD, respectively) groups were also examined. Likelihood ratios (LR) for the neurophysiologic tests being abnormal in the various groups were also determined. RESULTS: BAEP abnormalities were seen in 15/17 (88%) children with EIKD and 2/5 (40%) children with LOKD, LR statistically significant. In the EIKD group, all 13 symptomatic children had an abnormal BAEP, whereas 2/4 (50%) of the pre-symptomatic children had similar findings, LR statistically significant. VEP abnormalities were noted in 8/15 (53%) children with EIKD and none of the children with LOKD, LR statistically significant. In the EIKD group, the 8/12 (67%) symptomatic children had an abnormal VEP, whereas none of the pre-symptomatic children did, LR statistically significant. Subgroup analyses of the LOKD subgroups did not yield significant findings. CONCLUSIONS: BAEP abnormalities are among the first objective indications of central nervous system disease in children with EIKD. VEP abnormalities occur later in the course of the illness. Both tests are less helpful in children with LOKD. SIGNIFICANCE: BAEP and VEP testing is helpful in objective evaluation of children with KD.

Age of Onset↗

Changes in depressive symptoms after continuous positive airway pressure treatment for obstructive sleep apnea.

It is generally believed that obstructive sleep apnea (OSA) causes depression in some patients, yet it is unknown whether this depression is an actual clinical phenomenon or purely a result of overlapping somatic/physical symptoms shared by both disorders. The present study investigated changes in both somatic and affective/cognitive symptoms of depression associated with the introduction of continuous positive airway pressure (CPAP) treatment for OSA. Participants were 39 outpatients (35 males, 4 females) with no current or past mental health problems, diagnosed with OSA in a hospital sleep disorders clinic. The Beck Depression Inventory (BDI) was administered prior to treatment and again 3 months after CPAP. Total BDI scores improved after CPAP, independent of objectively monitored CPAP compliance rates. Both somatic and affective/ cognitive symptoms of depression improved in a similar manner after treatment. Our findings suggest that depressive symptoms experienced by OSA patients are not solely the result of physical OSA symptoms but include a mood component as well. We introduce a hypothetical model to conceptualize the relationship between OSA and depression.

Adolescent↗

Should women with obstructive sleep apnea syndrome be screened for hypothyroidism?

This study was conducted to determine if there is a significant increase in prevalence of hypothyroidism in women with OSAS such that screening might be warranted. Women undergoing polysomnography (PSG) at Duke University between January 1, 2000 and August 21, 2001 were considered for enrollment. Those with a respiratory disturbance index (RDI) >/= 10 were included for further analysis. Demographic data and documentation of thyroid testing was obtained. Thyroid testing obtained within 1 year of the PSG was used for this study. Demographic data of the euthyroid and hypothyroid groups were compared. Prevalence of hypothyroidism was calculated and compared with the prevalence of hypothyroidism in the Framingham study. A total of 118 women had OSAS per PSG. Seventy-five patients had thyroid function testing within 1 year of PSG evaluation. The mean age, body mass index, and RDI in the euthyroid and hypothyroid groups were not significantly different. The prevalence of hypothyroidism was 9.3%; the established prevalence of hypothyroidism in women in the general population is 5.9%. There was no statistically significant difference in the prevalence between these groups. The prevalence of hypothyroidism in women with OSAS is no higher than that seen in the general population. Screening women with symptoms of OSAS for hypothyroidism is unlikely to be useful.

Body Mass Index↗

Delayed visual maturation associated with auditory neuropathy/dyssynchrony.

Delayed visual maturation is a term used to describe infants who initially seem blind but subsequently have a marked improvement. The mechanism of visual loss and the subsequent improvement remains unknown. Auditory neuropathy/dyssynchrony is a condition of hearing impairment associated with absent or severely abnormal brainstem auditory evoked potentials but normal cochlear functions as measured by otoacoustic emissions. In this report, a 9-month-old infant who had no visual fixation for the first 3 months of life and congenital hearing impairment is described. Her brainstem auditory evoked potential study at 2.5 months of age showed no response to click stimuli presented at 90 dB nHL, whereas her otoacoustic emissions were normal. Subsequently, her vision and hearing improved. A brainstem auditory evoked potential study at 9 months of age showed reproducible waveforms. This case suggests the need for a detailed hearing evaluation of children with delayed visual maturation. Furthermore, this case highlights the need for follow-up brainstem auditory evoked potential testing prior to pursuing any audiologic intervention.

Evoked Potentials, Auditory, Brain Stem↗

Reflex seizures and non-ketotic hyperglycemia: an unresolved issue.

Reflex seizures are a rare form of epilepsy, the pathogenesis of which is unclear. They have been reported in the setting of non-ketotic hyperglycemia (NKH) and are considered to be neuroendocrine in origin. We report a diabetic patient with movement-induced seizures whose presentation suggests that brain ischemia may be the precipitating event in focal seizures seen in the setting of NKH. We recommend that in such instances a focal lesion such as stroke should be ruled out.

Aged↗

Epilepsy associated with lupus anticoagulant.

INTRODUCTION: Lupus anticoagulant (LA) is commonly present in patients with systemic lupus erythematosus (SLE) who present with an ischemic cerebral stroke. Reports have noted the presence of LA in patients with epilepsy who do not have SLE. These patients are usually elderly, and it has been postulated that their epilepsy is due to subclinical ischemic infarcts. METHODS: Two cases are presented in young patients (age < 35 years) who developed epileptic seizures and were LA positive. These patients did not have SLE or have cerebral infarcts that could explain the presence of their seizures. RESULTS: A 28-year-old woman was admitted for aortic insufficiency and new onset seizures. The clinical history, physical examination and magnetic resonance imaging did not reveal an antecedent cortical ischemic event. Serological testing revealed the presence of LA. The second patient was a 33-year-old man with medically intractable epilepsy in whom serological testing revealed the presence of LA. The clinical history, physical examination, and MRI did not reveal any evidence of an antecedent ischemic event. Neither patient had SLE. CONCLUSIONS: In young patients without SLE and cerebral infarcts, LA may be associated with epileptic seizures.

Adult↗

Neurophysiological monitoring for the nucleus caudalis dorsal root entry zone operation.

OBJECTIVE: The purpose of this report is to describe a neurophysiological monitoring technique that can decrease the incidence of complications while maintaining the effectiveness of the nucleus caudalis dorsal root entry zone (DREZ) operation. METHODS: Needle electrodes were used to stimulate the supraorbital, infraorbital, mental, and median nerves after the nucleus caudalis was surgically exposed. The DREZ electrode was used to record responses from the various areas in and near the nucleus. The target site was localized. Before lesioning, the site was stimulated with the DREZ electrode and electromyographic activation was sought. If no activation was observed, a lesion was made. RESULTS: Five patients underwent a total of seven nucleus caudalis DREZ procedures with complete neurophysiological monitoring. The mean number of lesions per procedure in this series was 5.4. Six procedures (86%) resulted in immediate pain relief, and five (71%) produced persistent benefit after a mean follow-up period of 12 months. Only one patient (20%) (one of seven procedures) who underwent a unilateral DREZ procedure had ataxia, which resolved within a few days. No complications were noted at follow-up. CONCLUSION: Despite patients in this series receiving fewer lesions, the efficacy of the DREZ operation was comparable to that reported in earlier studies. There were fewer complications when neurophysiological monitoring was used. Such monitoring should be considered for nucleus caudalis DREZ operations.

Adult↗

Evaluation and comparison of Tranquility and AutoSet T autotitrating CPAP machines.

The objective of this study was to determine the effectiveness of two autotitrating continuous positive airway pressure (auto-CPAP), the Tranquility (Respironics, Inc., Murrysville, PA, U.S.A.) and the AutoSet T (ResMed Corp., Poway, CA, U.S.A). Patients with obstructive sleep apnea syndrome (OSAS) underwent attended auto-CPAP titration with either the Tranquility or AutoSet T machine. The auto-CPAP machine was given a performance score between 0 and 9. A score of 3 or lower indicated that autotitration had to be discontinued. Sixty patients were studied, 35 patients with the Tranquility machine and 25 patients with the AutoSet T machine. The mean age was 55.4 years, whereas the mean body mass index (BMI) was 32.7. The mean respiratory disturbance index (RDI) was 37.5, and the mean arousal index before use of auto-CPAP was 41.4. The two treatment groups were comparable in age, BMI, RDI, and arousal index. The mean performance scores for the Tranquility and AutoSet T machines were not statistically different. Autotitration with the Tranquility machine had to be discontinued in 10 of the 35 patients (29%), whereas with the AutoSet T machine, autotitration was discontinued in 11 of the 25 patients (44%). Despite advances in auto-CPAP machines, there remains a large minority of patients with OSAS in whom these machines do not accomplish treatment goals. At this time, these machines are not a substitute for in-laboratory CPAP titration polysomnograms.

Adult↗