The Impact of Revascularization in a Patient with Atypical Manifestations of Hypoperfusion.

Background and Objectives: Carotid revascularization is one of the most effective treatment options in patients with severe carotid artery stenosis causing hypoperfusion in basal ganglia. Atypical manifestations include hyperkinetic movements, noted as extremely rare. We report a case about a patient with 2-months-long complaints of Uncontrollable movements in his right side of the body subsided after carotid revascularization. Case presentation: A 71-year-old male was admitted to Pauls Stradins Clinical University Hospital with the main complaints of 2-months-long uncontrollable movements in his right hand and his right leg. When performing coordination tasks, slight inaccuracy was noted with the right-side extremities. Hyperkinetic movements-choreoathetosis in the right side of the patient's face, arm, and leg-were seen. Computed tomography angiography revealed subocclusion in the proximal segment of the left internal carotid artery and 30% stenosis in the proximal segment of the right internal carotid artery. The patient was consulted by a vascular surgeon. Eversion endarterectomy of the left internal carotid artery was performed. The early postoperative period occurred without complications. The patient was discharged from the hospital 2 days after the surgery in good overall health condition. Two months later, choreoathetotic movements in his right side of the body had markedly decreased. No focal neurologic deficits were noted. Conclusions: Revascularization may be effective by eliminating emboli and stenosis, leading to hypoperfusion in watershed territories. A case of a 71-year-old male patient with the main complaints of 2-months-long uncontrollable movements in his right side of the body subsiding after carotid revascularization was demonstrated. It is vital to recognize atypical manifestations of hypoperfusion, associated with stenosis in internal carotid arteries, to early make a diagnosis, to perform an appropriate treatment, and to reduce the risk of cerebral infarction in the future, resulting in a longer high-quality life for the patient.

Association of gallbladder hyperkinesia with acalculous chronic cholecystitis: A case-control study.

BACKGROUND: This is the first case-control study investigating an association between gallbladder hyperkinesia and symptomatic acalculous chronic cholecystitis. METHODS: This retrospective study in a single academic center compared resolution of biliary pain in adults with gallbladder hyperkinesia, defined as a hepatobiliary iminodiacetic acid scan ejection fraction ≥80%, undergoing cholecystectomy (study group) with those treated medically without cholecystectomy (control group). Of 1,477 hepatobiliary iminodiacetic acid scans done between 2013 and 2018, a total of 296 adults without gallstones had an ejection fraction ≥80%, of whom 46 patients met predetermined eligibility criteria. Demographic data, hepatobiliary iminodiacetic acid scan ejection fraction, chronicity of pain, and resolution of pain were compared between groups. RESULTS: Demographics (mean ± standard deviation) in the control group (n = 25) and in the study group (n = 21) were, respectively, age 40 y ± 16 y and 39 y ± 14 y, body mass index 28.9 ± 5.2 and 29.1 ± 7.1 kg/m2, with 15 (60%) and 18 (86%) females in each. Resolution of pain after cholecystectomy occurred in 18 of 21 patients (86%); however, pain persisted in 20 of 25 patients (80%) treated medically after mean follow-up of 36 ± 28 months (range 10-120 months) (P < .01). Pain resolution with cholecystectomy was independent of demographic variables, hepatobiliary iminodiacetic acid scan ejection fraction, and chronicity of pain. The odds of pain resolution was 19.7 times greater with cholecystectomy than without (odds ratio, 19.7; 95% confidence interval, 4.34, 89.43; P < .01), and remained robust even with the odds adjusted for each covariate. Gallbladder histopathology confirmed chronic cholecystitis in all 21 cholecystectomy specimens. CONCLUSION: Symptomatic gallbladder hyperkinesia could be a new indication for cholecystectomy in adults.

Understanding the reward system functioning in anorexia nervosa: crucial role of physical activity.

Hyperactivity is a potential neurobiological marker and a core psychopathological trait in anorexia nervosa (AN). We investigated the processing of hyperactivity-related information in fifteen AN patients, 15 athletes and 15 non-athletes to examine if they represent disorder-related reward information using eye tracking. We assessed the extent of individually performed physical activity, mood, trait reward sensitivity and serum leptin levels. Results revealed a pronounced bias in overall attentional engagement toward stimuli associated with physical activity in patients and athletes as compared to non-athletes. In patients, relevant correlations were found: trait reward sensitivity and attentional orienting were strongly correlated and amount of physical activity correlated with attentional orienting and engagement. Compared to non-athletes, patients and athletes rated exercise stimuli as more pleasant. Findings suggest that exercise-related stimuli are perceived as rewarding by AN patients. Positive motivational valence of physical activity might contribute to disorder development and maintenance.

Clinical case of anti-N-methyl-D-aspartate receptor encephalitis in an 8-month-old patient with hyperkinetic movement disorder.

This article describes an 8-month-old boy with the full clinical spectrum anti-N-methyl-d-aspartate receptor encephalitis. He was admitted to the hospital with involuntary orofacial head movements, behavioral changes, and fluctuation in consciousness. His examination showed tongue thrusting, decreased responsiveness, and hypotonia without fever. Analysis of the cerebrospinal fluid revealed increased protein levels (62 mg/dL). The next day he developed oral dyskinesia and choreoathetosis. Video-electroencephalogram polygraphy showed coreo-dystonic movements without electrographic correlation. A putative diagnosis of autoimmune encephalopathy was made, and treatment with intravenous immunoglobulin and methylprednisolone was started, with improvement in the abnormal movements. Antibodies to the N-methyl-d-aspartate receptor were identified in the cerebrospinal fluid and blood. He began receiving immunoglobulin once a month for a year. Two months after the treatment had started, the involuntary movement disappeared and his development has been normal. N-methyl-d-aspartate receptor encephalitis is a recently identified disorder. This is the youngest case reported. Prompt diagnosis and treatment are important to obtain full recovery.

Loss of CDKL5 disrupts kinome profile and event-related potentials leading to autistic-like phenotypes in mice.

Mutations in the X-linked cyclin-dependent kinase-like 5 (CDKL5) gene have been identified in neurodevelopmental disorders including atypical Rett syndrome (RTT), autism spectrum disorders (ASDs), and early infantile epileptic encephalopathy. The biological function of CDKL5 and its role in the etiology of these disorders, however, remain unclear. Here we report the development of a unique knockout mouse model of CDKL5-related disorders and demonstrate that mice lacking CDKL5 show autistic-like deficits in social interaction, as well as impairments in motor control and fear memory. Neurophysiological recordings reveal alterations in event-related potentials (ERPs) similar to those observed in RTT and ASDs. Moreover, kinome profiling uncovers disruption of multiple signal transduction pathways, including the AKT-mammalian target of rapamycin (mTOR) cascade, upon Cdkl5 loss-of-function. These data demonstrate that CDKL5 regulates signal transduction pathways and mediates autistic-like phenotypes and together establish a causal role for Cdkl5 loss-of-function in neurodevelopmental disorders.

Bromocriptine for control of hyperthermia in a patient with mixed autonomic hyperactivity after neurosurgery: a case report.

Mixed autonomic hyperactivity disorder (MAHD) among patients with acquired brain injury can be rare. A delayed diagnosis of MAHD might exacerbate the clinical outcome and increase healthcare expenses with unnecessary testing. However, MAHD is still an underrecognized and evolving disease entity. A 25-yr-old woman was admitted the clinic due to craniopharyngioma. After an extensive tumor resection, she complained of sustained fever, papillary contraction, hiccup, lacrimation, and sighing. An extensive evaluation of the sustained fever was conducted. Finally, the cause for MAHD was suspected, and the patient was successfully treated with bromocriptine for a month.

Double disruption of α2A- and α2C-adrenoceptors results in sympathetic hyperactivity and high-bone-mass phenotype.

Evidence demonstrates that sympathetic nervous system (SNS) activation causes osteopenia via ß(2)-adrenoceptor (ß2-AR) signaling. Here we show that female mice with chronic sympathetic hyperactivity owing to double knockout of adrenoceptors that negatively regulate norepinephrine release, α(2A)-AR and α(2C)-AR (α(2A) /α(2C)-ARKO), present an unexpected and generalized phenotype of high bone mass with decreased bone resorption and increased formation. In α(2A) /α(2C)-ARKO versus wild-type (WT) mice, micro-computed tomographic (µCT) analysis showed increased, better connected, and more plate-shaped trabeculae in the femur and vertebra and increased cortical thickness in the vertebra, whereas biomechanical analysis showed increased tibial and femoral strength. Tibial mRNA expression of tartrate-resistant acid phosphatase (TRACP) and receptor activator of NF-κB (RANK), which are osteoclast-related factors, was lower in knockout (KO) mice. Plasma leptin and brain mRNA levels of cocaine amphetamine-regulated transcript (CART), which are factors that centrally affect bone turnover, and serum levels of estradiol were similar between mice strains. Tibial ß(2)-AR mRNA expression also was similar in KO and WT littermates, whereas α(2A)-, α(2B)- and α(2C)-AR mRNAs were detected in the tibia of WT mice and in osteoblast-like MC3T3-E1 cells. By immunohistochemistry, we detected α(2A)-, α(2B)-, α(2C)- and ß(2)-ARs in osteoblasts, osteoclasts, and chondrocytes of 18.5-day-old mouse fetuses and 35-day-old mice. Finally, we showed that isolated osteoclasts in culture are responsive to the selective α(2)-AR agonist clonidine and to the nonspecific α-AR antagonist phentolamine. These findings suggest that ß(2)-AR is not the single adrenoceptor involved in bone turnover regulation and show that α(2)-AR signaling also may mediate the SNS actions in the skeleton.

Subtypes of pediatric delirium: a treatment algorithm.

Delirium in adult populations of hospitalized patients has been well characterized into hyperactive, hypoactive, and mixed subtypes. The degree to which these subtypes apply to pediatric populations has yet to be fully demonstrated. In this case report, the authors present two cases of delirium that serve as examples of the hyperactive and hypoactive/mixed types and then discuss treatment. They find marked differences in the response of different delirium subtypes to haloperidol and risperidone and theorize as to the neurochemical pathways by which these pharmacological agents might work. This framework provides an algorithm for the treatment of pediatric delirium.

Thyroxine-induced hypermotor seizure.

Thyroxine-induced epilepsy is a very rare condition occurring in epileptic patients. Here we report a boy with thyroxine-induced hypermotor seizure (HMS) following thyroxine administration for his central hypothyroidism secondary to surgery and cranial radiation for his brain tumor. After 3 years seizure-free period, he had repeated HMS, seven to eight attacks per day, after initiation L-thyroxine treatment. Following reduction of the daily thyroxine dose, his seizures decreased in frequency. To our knowledge, this is the first reported case of HMS associated with L-thyroxine administration.

Effects of vibrissae removal on methamphetamine-induced damage to rat somatosensory cortical neurons.

Repeated methamphetamine (mAMPH) damages forebrain monoamine terminals and causes degeneration of nonmonoaminergic cell bodies in rat primary somatosensory cortex (S1). These degenerating cortical neurons can be labeled with the fluorochrome dye Fluoro-Jade (FJ) and are found almost exclusively in layers II/III and IV of the vibrissae representation in S1. Within S1, layer IV is organized into discrete, anatomically identifiable units termed barrels, each of which receives information from a single whisker. We previously reported that mAMPH-damaged neurons in S1 were located within the whisker barrels, suggesting that the prolonged mAMPH-induced whisking contributes to S1 neuronal injury. Here, we investigate effects of vibrissae removal on mAMPH-induced damage to S1 neurons. Rats were anesthetized and vibrissae were trimmed from either the left, right, or neither side of the snout. The next day they were given four injections of either saline (1 ml/kg, s.c.) or mAMPH (4 mg/kg, s.c.) at 2-h intervals. Three days later, cortical sections were processed for FJ histochemistry. The hemivibrissotomy produces a hemispheric asymmetry in FJ-positive neurons in barrel cortex, with fewer damaged neurons contralateral than ipsilateral to whisker removal. Taken together with the demonstration that acute injection of this dose of mAMPH induces the immediate early gene zif/268 and Fos protein in barrel cortex, these data suggest that the prolonged behavioral activity involving the vibrissae contributes to the mAMPH-induced damage to S1 neurons. Thus, some of the injurious effects of drugs may depend on afferent activity occurring as a result of the abnormal behaviors evoked by their administration.

Adults with ADHD. An overview.

Attention-Deficit Hyperactivity Disorder (ADHD) is a common, genetically transmitted neurological disorder, with onset in childhood, probably mediated by decreased brain dopaminergic functioning. The first author was one of the earliest to describe the persistence of symptoms into adulthood. Prevalence and natural history data suggest that of the 3 to 10% of children diagnosed with ADHD, one- to two-thirds (somewhere between 1 and 6% of the general population) continue to manifest appreciable ADHD symptoms into adult life. This paper describes how ADHD in adults can be readily diagnosed and treated, despite resembling or coexisting with other psychiatric disorders. The Wender Utah diagnostic criteria address adult characteristics of the disorder. Informant and patient interviews and rating scales are used to determine the psychiatric status of the patient as a child, make a retroactive diagnosis of childhood ADHD, and establish the current diagnosis of the adult. Stringent diagnosis is key to determining effective treatment. Dopamine agonist stimulant medications appear to be the most effective in treating ADHD. About 60% of patients receiving stimulant medication showed moderate-to-marked improvement, as compared with 10% of those receiving placebo. The core symptoms of hyperactivity, inattention, mood lability, temper, disorganization, stress sensitivity, and impulsivity have been shown to respond to treatment with stimulant medications. Non-dopaminergic medications, such as the tricyclic antidepressants and SSRIs have generally not been useful in adults with ADHD in the absence of depression or dysthymia. Pemoline is no longer approved for use in these patients, despite early favorable reports. Appropriate management of adult patients with ADHD is multimodal. Psychoeducation, counseling, supportive problem-directed therapy, behavioral intervention, coaching, cognitive remediation, and couples and family therapy are useful adjuncts to medication management. Concurrent supportive psychosocial treatment or polypharmacy may be useful in treating the adult with comorbid ADHD.

[Hyperactivity of the muscles of the lip and chin and indications for surgery to reduce it]. / Dysfonctionnement des muscles labio-mentonniers et indications de la chirurgie d'affaiblissement.

Hyperactivity of the muscles controlling the lower lip and chin (muscles of the chin, buccinator, orbicularis, etc.) can be demonstrated at different levels of the muscular complex and may act as an elastic force against the mandibular alveolar process. Dysfunction in this region may lead to reduction of the alveolar bone quantity, reducing the tooth-bone equilibrium. If this balance is disturbed, periodontal lesions, a lower incisal crowding and a retrognathic mandibular process may result. Surgery to correct overactivity of the chin muscles is described here step by step. Resection of the muscles may be undertaken on a greater or lesser scale and striation of the muscle may also allow reduction of muscular strength. Reducing the muscular activity creates a better environment for the development of the mandible and its alveolar process. Depending on the clinical situation, these techniques can be associated with genioplasty, bone graft and/or mandibular orthognathic surgery.

Botulinum toxin A for the treatment of facial hyperkinetic wrinkle lines in Koreans.

There are a number of different causes for facial wrinkle lines, such as aging, gravity, and chronic pulling of mimetic muscles on the face. Among these, pulling by mimetic muscles on the skin not only involves facial expression but also has a great role in forming facial wrinkle lines as a result of repetitive action, such as dynamic or hyperkinetic wrinkle lines. Botulinum toxin A is currently being used for eliminating facial hyperkinetic wrinkles by causing paralysis of the underlying mimetic muscles. Because there are some histologic differences between Asians and Caucasians, such as thick dermis and more abundant collagen fiber, etc., the chronic pulling by mimetic muscles on the skin is expected to affect facial wrinkles differently. Therefore, the purpose of this study was to determine the efficacy of botulinum toxin A injection in eliminating facial hyperkinetic wrinkle lines among Korean patients. This study included 38 patients and 59 injection sessions from January of 1996 to April of 1997. We used Botox containing 100 U. Toxin was diluted with 4 ml of sterile normal saline and yielded 2.5 U for each 0.1 cc. A dose of 5 to 10 U was used in each muscle. Ages ranged from 26 to 56 years. There were 33 women and 5 men included in this study. Thirty-two of the patients were followed from 3 months up to 12 months after injections. The number of injection sessions that were performed on each patient was as follows: one session, 23 patients; two sessions, 10 patients; three sessions, 4 patients; four sessions, 1 patient. The number of injections per target site among these 38 patients was as follows: lateral canthal area, 33; glabellar area, 9; forehead, 9; nasal dorsum, 5. The most common duration of effective response was about 4 months, but in eight patients the period was over 5 months. After the response, complete recovery took about 1 or 2 months. Two patients felt unsatisfied, 5 patients felt slightly improved, and 25 patients retained only a slight line and were satisfied with the results. None of the patients experienced complete removal of wrinkle lines. Adverse effects included altered facial looks or appearances, mild local swelling, and ecchymosis at the injection sites. No systemic side effects were noted. Based on these results, the injection of botulinum toxin A seems to be an effective method of eliminating wrinkle lines on the upper third of the face in Korean patients, and it was a simple and effective nonsurgical procedure.

Chronic nephritis, sensorineural deafness, growth and developmental retardation, hyperkinesis, and cleft soft palate in a 5-year-old boy. A new combination?

A 5-year-old Japanese boy showed nephritis similar to, but distinct from, that in Alport syndrome. Nephrotic syndrome without hematuria was noticed at age 2, although renal biopsy at age 4 revealed widespread irregular thickening of the glomerular basement membrane with splitting of the lamina densa on electron microscopy, characteristic of nephritis in Alport syndrome. Sensorineural deafness was noticed at age 4 weeks by no auditory brain stem response, unusually early for Alport syndrome. Goodpasture antigen and amyloid P component were found in the glomerular basement membrane. Thus, the antigenicity of the glomerular basement membrane was different from that in male patients with X-linked Alport syndrome. In addition, growth and developmental retardation, hyperkinesis, and cleft soft palate were seen. These features are a hitherto undescribed combination. The family history was negative for any of the features of the boy.

[Stereotaxic surgery of temporal lobe epilepsy]. / Stereotaksicheskaia khirurgiia visochnoi épilepsii

Forty patients with temporal epilepsy were operated on with a stereotaxic technique. Eleven patients had a unilateral localization of the epileptic focus, and in 29 bitemporal foci were diagnosed. In evaluating the results of the treatment the dynamics of the epileptic fits, the peculiarities of changes in the mental status, and the degree of social adaptation were taken into consideration. A postoperative improvement was achieved in 73% of the patients with monotemporal lesions, while in those with bitemporal epilepsy and distinct persistent mental disorders the state was improved in 44% of the cases. Indications for the choice of the zone of destruction depending on the clinical peculiarities of the lesion are presented.

Sleep apnea in eight children.

Eight children, 5 to 14 years of age, were diagnosed by means of nocturnal polygraphic monitoring with a sleep apnea syndrome similar to that seen in adults. Excessive daytime sleepiness, decrease in school performance, abnormal daytime behavior, recent enuresis, morning headache, abnormal weight, and progressive development of hypertension should suggest the possibility of a sleep apnea syndrome when any of these symptoms is associated with loud snoring interrupted by pauses during sleep. Surgery may eliminate the clinical symptomatology.