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Pediatrics and Neonatology Mar 2024
Topics: Humans; Blepharoptosis; Reflex, Abnormal; Jaw Abnormalities; Intubation, Intratracheal; Heart Defects, Congenital; Nervous System Diseases; Congenital Cranial Dysinnervation Disorders
PubMed: 37951831
DOI: 10.1016/j.pedneo.2023.10.003 -
Clinical Neurophysiology : Official... Dec 2023Cortical myoclonus is thought to result from abnormal electrical discharges arising in the sensorimotor cortex. Given the ease of recording of cortical discharges,... (Review)
Review
Cortical myoclonus is thought to result from abnormal electrical discharges arising in the sensorimotor cortex. Given the ease of recording of cortical discharges, electrophysiological features of cortical myoclonus have been better characterized than those of subcortical forms, and electrophysiological criteria for cortical myoclonus have been proposed. These include the presence of giant somatosensory evoked potentials, enhanced long-latency reflexes, electroencephalographic discharges time-locked to individual myoclonic jerks and significant cortico-muscular connectivity. Other features that are assumed to support the cortical origin of myoclonus are short-duration electromyographic bursts, the presence of both positive and negative myoclonus and cranial-caudal progression of the jerks. While these criteria are widely used in clinical practice and research settings, their application can be difficult in practice and, as a result, they are fulfilled only by a minority of patients. In this review we reappraise the evidence that led to the definition of the electrophysiological criteria of cortical myoclonus, highlighting possible methodological incongruencies and misconceptions. We believe that, at present, the diagnostic accuracy of cortical myoclonus can be increased only by combining observations from multiple tests, according to their pathophysiological rationale; nevertheless, larger studies are needed to standardise the methods, to resolve methodological issues, to establish the diagnostic criteria sensitivity and specificity and to develop further methods that might be useful to clarify the pathophysiology of myoclonus.
Topics: Humans; Myoclonus; Evoked Potentials, Somatosensory; Electroencephalography; Reflex; Neurophysiology; Electromyography
PubMed: 37948946
DOI: 10.1016/j.clinph.2023.10.007 -
Frontiers in Neurology 2023A perilymphatic fistula (PLF) presents with abnormal traffic in the otic capsule, causing cochlear and vestibular symptoms. However, the mechanisms underlying symptom...
A perilymphatic fistula (PLF) presents with abnormal traffic in the otic capsule, causing cochlear and vestibular symptoms. However, the mechanisms underlying symptom recurrence remain controversial. Herein, we report the case of a 27-year-old female who complained of hearing disturbance in her right ear and recurrent vertigo after sudden onset of hearing loss with vertigo. The caloric test revealed unilateral weakness in the right ear, and the video head impulse test (vHIT) showed decreased vestibulo-ocular reflex (VOR) gain. Contrast-enhanced magnetic resonance imaging (MRI) using hybrid of reversed image of positive endolymph signal and negative image of perilymph signal (HYDROPS) indicated a collapsed endolymphatic space. As the vestibular symptoms did not improve, an exploratory tympanotomy was performed on the right ear. Although perilymph leakage was not noted in the oval or round windows, both windows were sealed with connective tissue. The patient's vestibular symptoms rapidly improved after surgery, and postoperative contrast-enhanced MRI showed improvement in the collapsed endolymphatic space. Although the caloric test revealed unilateral weakness, the VOR gain on the vHIT improved to normal on the right side. Thus, these findings indicated that recurrent symptoms caused by PLF are associated with a collapsed endolymphatic space. We speculate that the collapsed endolymphatic space was due to a ruptured Reissner's membrane. We hypothesized that sealing the fistula would promote normalization of perilymph pressure. The ruptured Reissner's membrane may have been gradually repaired as vestibular symptoms improved. This case adds to the existing literature on the occurrence of the "double-membrane break syndrome". Collapse of the endolymph due to a ruptured Reissner's membrane may be the cause of PLF symptoms.
PubMed: 37928144
DOI: 10.3389/fneur.2023.1276991 -
Cureus Oct 2023Aim/objective Neurological Pupil Index (NPi), measured by automated pupillometry (AP), allows the objective assessment of pupillary light reflex (PLR). NPi ranges from 0...
Aim/objective Neurological Pupil Index (NPi), measured by automated pupillometry (AP), allows the objective assessment of pupillary light reflex (PLR). NPi ranges from 0 (non-reactive) to 5 (normal). In this study, we aimed to compare neurologic and functional outcomes in children admitted for neurologic injury with normal (≥3) versus abnormal (<3) NPi measured during their pediatric intensive care unit (PICU) stay. Materials and methods We conducted a retrospective chart review of children between one month and 18 years admitted to our PICU with a diagnosis of neurologic injury between January 2019 and June 2022. We collected demographic, clinical, pupillometer, and outcome data, including mortality, Pediatric Cerebral Performance Category (PCPC), Pediatric Overall Performance Category (POPC), and Functional Status Score (FSS) at admission, at discharge, and at the three to six-month follow-up. We defined abnormal pupil response as any NPi <3 at any point during the PICU stay. Using the student's t-test and chi-square test, we compared the short-term and long-term outcomes of children with abnormal NPi (<3) versus those with normal NPi (≥3). Results There were 49 children who met the inclusion criteria and who had pupillometry data available for analysis. The mean (SD) Glasgow Coma Scale (GCS) in the study cohort was 5.6 (4.3), and 61% had low (<3) NPi during ICU stay. Mortality was significantly higher among patients with an abnormal NPi as compared to those with normal NPi. Children with abnormal NPi exhibited significant worsening of neurologic and functional status (ΔPCPC, ΔPOPC, and ΔFSS) from admission to discharge (mean (SD): 3.55(1.5), 3.45(1.43), 16.75(7.85), p<0.001) as compared to those with normal NPi (mean (SD): 1.45(0.93), 1.73(0.90), 3.55(2.07), p>0.05). The significant difference in neurologic and functional status persisted at the three to six-month follow-up between the two groups - children with abnormal NPi (mean (SD): 2.0(1.41), 2.08(1.38), 6.92(6.83), p<0.01) and children with normal NPi (mean (SD): 0.82(1.01), 0.94(1.03), 1.53(1.70), p>0.05). Conclusion In our retrospective cohort study, children admitted to the PICU for a neuro injury and with abnormal NPi (< 3) have higher mortality, and worse short-term and long-term neurologic and functional outcomes as compared to those with normal NPi (≥ 3) measured during the PICU course. AP provides an objective assessment of PLR and has potential applications for neuro-prognostication. More research needs to be done to elucidate the prognostic value of NPi in pediatrics.
PubMed: 37927706
DOI: 10.7759/cureus.46480 -
European Journal of Medical Research Nov 2023Older patients (aged ≥ 60 years) with severe brain injury have a high mortality and disability rate. The objective of this retrospective study was to assess the...
BACKGROUND
Older patients (aged ≥ 60 years) with severe brain injury have a high mortality and disability rate. The objective of this retrospective study was to assess the clinical risk factors of prognosis in older patients with severe brain injury after surgical intervention and to analyze the prognosis of the surviving group of patients 1 year after discharge.
METHODS
Clinical data of older patients with severe brain injury who were admitted to two neurosurgical centers between January 2010 and December 2020 were collected. Patient age, sex, Glasgow Coma Scale (GCS) score at admission, underlying disease, mechanisms of injury, abnormal pupillary reflex, head computed tomography imaging findings (such as hematoma type),intraoperative brain swelling and other factors were reviewed. All the patients were categorized into a good prognosis (survival) group and a poor prognosis (death) group by the Glasgow Outcome Score (GOS); also, the related factors affecting the prognosis were screened and the independent risk factors were identified by the Binary logistic regression analysis. GOS was used to evaluate the prognosis of the surviving group of patients 1 year after discharge.
RESULTS
Out of 269 patients, 171 (63.6%) survived, and 98 (36.4%) died during hospitalization. Univariate analysis showed that age, GCS score at admission, underlying diseases, abnormal pupillary reflex, the disappearance of ambient cistern, the midline structure shift, intraoperative brain swelling, oxygen saturation < 90%, and cerebral hernia were risk factors for the prognosis of older patients with severe brain injury after surgical intervention. Multivariate analysis showed that age, underlying diseases, disappearance of ambient cistern, Oxygen saturation < 90% and intraoperative brain swelling were independent risk factors of the prognosis in the population. The effect of surgical intervention differed among various age groups at 1-year follow-up after surgery.
CONCLUSIONS
The results of this retrospective study confirmed that age, underlying diseases, disappearance of ambient cistern, intraoperative brain swelling, and oxygen saturation < 90% are associated with poor prognosis in older postoperative patients with severe brain injury. Surgical intervention may improve prognosis and reduce mortality in older patients (age < 75 years). But for those patients (age ≥ 75 years), the prognosis was poor after surgical intervention.
Topics: Humans; Aged; Brain Edema; Retrospective Studies; Prognosis; Risk Factors; Brain Injuries; Treatment Outcome
PubMed: 37925438
DOI: 10.1186/s40001-023-01473-0 -
Sleep & Breathing = Schlaf & Atmung May 2024This study aimed to evaluate the functionality of the brainstem structures through the blink reflex (BR) test in patients with obstructive sleep apnoea (OSA) and to...
PURPOSE
This study aimed to evaluate the functionality of the brainstem structures through the blink reflex (BR) test in patients with obstructive sleep apnoea (OSA) and to assess the effects of continuous positive airway pressure (CPAP) treatment on BR responses.
METHODS
Patients with moderate-severe OSA and controls underwent BR testing. Patients with OSA who were adherent to CPAP therapy repeated BR testing at 6 months follow-up. CPAP adherence was defined as CPAP use for ≥ 4 hour per night on > 5 nights per week with residual apnoea-hypopnea index less than 5 events per hour.
RESULTS
A total of 22 patients with OSA (86% male, mean age 57.8 ± 10.6 years) and 20 controls (60% male, mean age 55.3 ± 9.3 years) were included. Patients with OSA showed longer right and left R1 latency, as well as delayed right ipsilateral and contralateral R2 latencies compared to controls. Patients with OSA who were compliant with CPAP treatment (n = 16; 88% men, mean age 58.8 ± 9.7 years) showed a significant decrease in latency of the right ipsilateral and contralateral R2 responses at 6 months.
CONCLUSION
This study showed an abnormal pattern of BR responses in patients with OSA, consistent with a significant impairment of brainstem functionality in OSA. CPAP treatment partially improved the BR responses, suggesting the importance of treating OSA.
Topics: Humans; Continuous Positive Airway Pressure; Male; Sleep Apnea, Obstructive; Middle Aged; Female; Blinking; Brain Stem; Adult; Aged; Polysomnography
PubMed: 37923871
DOI: 10.1007/s11325-023-02944-8 -
Yakugaku Zasshi : Journal of the... 2023p-Hydroxyamphetamine (p-OHA) is an active metabolite of amphetamine (AMPH) and methamphetamine (METH), and can be detected in the brain for a relatively long period...
p-Hydroxyamphetamine (p-OHA) is an active metabolite of amphetamine (AMPH) and methamphetamine (METH), and can be detected in the brain for a relatively long period after high-dose administration of AMPH in rodents. p-OHA may be involved in the abnormal behavior observed during the withdrawal period after a chronic administration of AMPH or METH. Therefore, the author investigated the effect of an intracerebroventricular (i.c.v.) administration of p-OHA on the changes of locomotor activity and prepulse inhibition (PPI) in the acoustic startle response in rodents. The i.c.v. administration of p-OHA significantly increased locomotor activity in mice. This effect was prevented by a pretreatment with a dopamine (DA) uptake inhibitor. Furthermore, local infusion of p-OHA into the nucleus accumbens (NAc) significantly increased locomotor activity in rats. Together these results suggest that dopaminergic systems in the rodent NAc may play important roles in p-OHA-induced locomotor activity. Next, the author tested the effects of the i.c.v. administration of p-OHA on PPI in mice. p-OHA induced PPI disruptions that were significantly improved by the pretreatment with a typical or an atypical antipsychotic, D or D receptor antagonists, respectively. p-OHA-induced PPI disruptions were also improved by a serotonin (5-HT) receptor antagonist, a 5-HT synthesis inhibitor or a 5-HT neurotoxin. These results suggest that p-OHA-induced PPI disruptions were mediated by DA and 5-HT release and subsequent stimulation of D, D and 5-HT receptors. Our recent series of reports indicate that the study of p-OHA may provide new insights into drug abuse as well as psychiatric disorders such as schizophrenia.
Topics: Humans; Rats; Mice; Animals; Dopamine; p-Hydroxyamphetamine; Serotonin; Rodentia; Reflex, Startle; Amphetamine; Methamphetamine; Synaptic Transmission; Dose-Response Relationship, Drug
PubMed: 37914334
DOI: 10.1248/yakushi.23-00120 -
Infectious Diseases of Poverty Oct 2023Rabies continues to pose significant public health challenges in many developing countries including Bhutan. A probable case of rabies was admitted to our hospital and...
BACKGROUND
Rabies continues to pose significant public health challenges in many developing countries including Bhutan. A probable case of rabies was admitted to our hospital and its reporting led to the uncovering of an outbreak in domestic and wild animals. We discuss the challenges in the diagnosis and management of rabies in a resource-limited setting.
CASE PRESENTATION
A 35-year-old male presented with intermittent fever, bilateral lower limb weakness that was rapidly progressive, urinary incontinence with episodes of palpitations and sweating. He had sustained a Category III bite on the right lower thigh with four bite marks, inflicted by a stray dog. He had received post-exposure prophylaxis with intra-dermal anti-rabies vaccine. On initial examination, the patient was in distress but cooperative for the interview. He had pulse rate ranging from 60 to 100/min with episodes of diaphoresis and palpitations, but with normal capillary blood glucose. In the lower limb, the muscle power was zero with absent tendon reflexes in the lower limb and impaired abdominal reflex below T level. He had hyperaesthesia below T, hydrophobia, aerophobia and photophobia. He had multiple spontaneous fasciculations in both the thighs and right deltoid and these later involved the intercostal muscles, neck and face muscles. He had altered sensorium and desaturation for which he required mechanical ventilation. Polymerase chain reaction for rabies virus was negative in cerebrospinal fluid and saliva. Rabies virus neutralizing antibody was negative in cerebrospinal fluid but had high titres in the serum. He received Human Rabies Immunoglobulin after admission. He was managed in the intensive care unit and died 23 days later. After this case was notified, a rapid response team was deployed in the field, and uncovered rabies outbreak in animals in the locality.
CONCLUSIONS
This case called for a serious evaluation of the country's efforts in achieving zero rabies deaths by 2030. The management of this case identified several critical areas of context-specific interventions in Bhutan. There is also an urgent need to improve diagnostic capabilities at the national reference laboratory and enhance the technical competencies of healthcare workers in the management of dog bite cases.
Topics: Male; Humans; Animals; Dogs; Adult; Rabies; Bhutan; Rabies Vaccines; Bites and Stings; Animals, Wild; Disease Outbreaks; Encephalomyelitis
PubMed: 37845744
DOI: 10.1186/s40249-023-01148-2 -
Etiologies of nontraumatic feline uveitis in the UK: A retrospective observational study of 72 cats.Open Veterinary Journal Sep 2023Uveitis is a common ophthalmic diagnosis in cats, that can lead to discomfort and loss of vision. Identification of nonidiopathic cases facilitates treatment and could...
BACKGROUND
Uveitis is a common ophthalmic diagnosis in cats, that can lead to discomfort and loss of vision. Identification of nonidiopathic cases facilitates treatment and could reduce morbidity associated with this condition.
AIM
To evaluate etiologies of nontraumatic uveitis in the UK, to compare diagnostic features between idiopathic cases and those with an established underlying etiology, and to investigate the association of clinical signs and abnormal diagnostic findings with a confirmed etiology.
METHODS
Records of cats diagnosed with uveitis at a UK referral center between August 2009 and April 2018 were retrospectively reviewed, excluding traumatic (and reflex) cases. Cases were categorized based on whether an underlying etiology had been established in cases with confirmed etiology, idiopathic, and inconclusive cases. All cases had a minimum of 12-month follow-up unless an underlying etiology had been established. Population characteristics, clinical signs, diagnostic investigation features, and results were reported.
RESULTS
72 cases of uveitis were included, of which male cats and domestic breeds were overrepresented. An underlying etiology was determined in 23.6% of cases: 9.7% had infectious diseases, 5.6% had systemic neoplasia, 4.2% had primary ocular neoplasia, and 4.2% had metabolic disease. Idiopathic uveitis comprised 37.5% of cases, and the remaining 38.9% were inconclusive, of which 35.7% died or were euthanased within the follow-up period. Among the study population, no significant age difference was found between cats with idiopathic disease or confirmed etiology. The unilateral disease was reported in 56.9% of cases and was not different across the idiopathic cases and confirmed etiology groups. The most common ophthalmic clinical sign was an aqueous flare, followed by keratic precipitates and hypotony. Iris color change ( = 0.015) and the presence of an intraocular mass ( = 0.025) were associated with an underlying etiology.
CONCLUSION
Idiopathic uveitis was found to be the most common diagnosis in this study population. However, a similar proportion of cases had possible underlying etiologies as a high proportion manifested systemic disease within the follow-up time. An underlying etiology could be established only in a quarter of cases. Further studies are required to standardize the investigations required when assessing cats with uveitis to minimize patient morbidity.
Topics: Animals; Cats; Male; Cat Diseases; Neoplasms; Retrospective Studies; United Kingdom; Uveitis
PubMed: 37842107
DOI: 10.5455/OVJ.2023.v13.i9.15 -
Frontiers in Neurology 2023Video head impulse tests (vHITs), assessing the vestibulo-ocular reflex (VOR), may be helpful in the differential diagnosis of acute dizziness. We aimed to investigate...
BACKGROUND
Video head impulse tests (vHITs), assessing the vestibulo-ocular reflex (VOR), may be helpful in the differential diagnosis of acute dizziness. We aimed to investigate vHITs in patients with acute posterior circulation stroke (PCS) to examine whether these findings could exhibit significant abnormalities based on lesion locations, and to evaluate diagnostic value of vHIT in differentiating dizziness between PCS and vestibular neuritis (VN).
METHODS
We prospectively recruited consecutive 80 patients with acute PCS and analyzed vHIT findings according to the presence of dorsal brainstem stroke (DBS). We also compared vHIT findings between PCS patients with dizziness and a previously studied VN group ( = 29). Receiver operating characteristic (ROC) analysis was performed to assess the performance of VOR gain and its asymmetry in distinguishing dizziness between PCS and VN.
RESULTS
Patients with PCS underwent vHIT within a median of 2 days from stroke onset. Mean horizontal VOR gain was 0.97, and there was no significant difference between PCS patients with DBS ( = 15) and without ( = 65). None exhibited pathologic overt corrective saccades. When comparing the PCS group with dizziness ( = 40) to the VN group ( = 29), patients with VN demonstrated significantly lower mean VOR gains in the ipsilesional horizontal canals (1.00 vs. 0.57, < 0.001). VOR gain and their asymmetry effectively differentiated dizziness in the PCS from VN groups, with an area under the ROC curve of 0.86 (95% CI 0.74-0.98) and 0.91 (95% CI 0.83-0.99, < 0.001), respectively.
CONCLUSION
Significantly abnormal vHIT results were rare in patients with acute PCS, even in the presence of DBS. Moreover, vHIT effectively differentiated dizziness between PCS and VN, highlighting its potential for aiding differential diagnosis of acute dizziness.
PubMed: 37808489
DOI: 10.3389/fneur.2023.1256826