The most severe form of spastic cerebral palsy (CP), which affects the arms and legs and often the face, is known as spastic quadriplegia. In addition to other developmental disabilities such as intellectual disability and seizures, it can cause difficulty in walking. Children with CP often have seizures as a result of brain injury, and spastic quadriplegic CP is typically associated with global developmental delay. For the purpose of addressing the unique motor and functional challenges associated with spastic quadriplegia, neurophysiotherapy is essential. This treatment includes neurodevelopmental techniques, posture and balance training, and activities aimed at improving gait. The purpose of this case study is to demonstrate how early and continuous physical therapy interventions can maximize a child\'s functional abilities and prevent further complications. In this instance, a five-year-old boy with a documented history of spastic quadriplegia, seizure disorder, and global developmental delay reported experiencing challenges with sitting, walking, and speech. He had three episodes of fever, which led to his hospital admission. The child\'s medical history included acute hemorrhagic encephalitis, mild hydroureteronephrosis on the left side, and persistent convulsions that affected only one side of the body. Bilateral thalamic altered signal intensities were observed in the brain\'s MRI, and multiple calcifications were detected in the periventricular cortex, thalamus, and basal ganglia on the brain\'s CT scan. To enhance the independence, strength, and coordination of voluntary movement in individuals with CP, a variety of techniques are used in addition to physical therapy, such as occupational therapy, speech therapy, aquatic therapy, constraint-induced movement therapy, functional electrical stimulation, orthotic devices, injections of botulinum toxin, and hippotherapy.

Typical or atypical presentations of rare diseases may be confounded by co-morbidities in critically-ill patients. It is imperative to diagnose and treat appropriately, despite this difficulty. Scleroderma renal crisis mimics many other conditions, and can be potentially fatal if not caught early enough. Particularly, in critically-ill patients with multiple pathologies, it can be difficult to distinguish scleroderma renal crisis from other diseases, such as thrombotic thrombocytopenic purpura (TTP), hypertensive emergency, posterior reversible encephalopathy syndrome (PRES), or atypical hemolytic uremic syndrome (HUS). Herein, a patient who presented with encephalopathy and seizures was initially treated for thrombotic thrombocytopenic purpura, but was ultimately diagnosed with scleroderma renal crisis. Given her numerous laboratory abnormalities, such as thrombocytopenia, hemolytic anemia, kidney and liver dysfunction, and elevated inflammatory markers, various differentials were considered. During her hospitalization, she suffered a cardiac arrest, seizures, nosocomial infections and worsening kidney disease requiring dialysis, making the final diagnosis of scleroderma renal crisis a diagnosis of exclusion. Subsequently, the management of a patient with multiple co-morbidities and confounding laboratory abnormalities difficult to treat. This article highlights these intricacies and formulates the thought process behind the diagnosis of Scleroderma Renal Crisis.

UNASSIGNED: This is a case report presenting the behavioral and dental management of a 9-year-old child recently diagnosed with autoimmune encephalitis, seizure disorder, and psychosis and treated with anticonvulsant and antidepressant medications. In this case, seizure semiology was presented as eye blinking during the attack, and the child was usually not conscious. It is absorbed by itself and associated with a post-sleeping attack that lasts for minutes.
UNASSIGNED: Parents presented to the pediatric dental clinic with a chief complaint of decayed teeth that needs to be restored. The child has mixed dentition and is diagnosed with anterior crossbite and single posterior crossbite. The child was cleared by the pediatric neurologist and psychiatrist and had no contraindications to dental treatment under local anesthesia. The child\'s treatment was completed on the dental chair without any seizure attacks, and the patient\'s chief complaint was resolved.
UNASSIGNED: Pediatric dentists should be educated about dental precautions and consider drug interactions when treating children with autoimmune encephalitis. Various types of non-pharmacological behavior guidance techniques and pharmacological methods of behavior management techniques can aid in the behavioral management of children with psychosis.
UNASSIGNED: Alowi WA, Baghlaf K. Behavioral and Dental Management of a Pediatric Patient Diagnosed with Autoimmune Encephalitis: A Case Report. Int J Clin Pediatr Dent 2023;16(2):416-419.

COVID-19 can affect many organ systems, including the CNS, with symptoms of altered mental status and seizures. We present a case of a 30-year-old man with cerebral palsy who developed seizures after a COVID-19 infection. Admission labs were remarkable for hypernatremia, and elevated creatine kinase, and troponin levels as well as creatinine above baseline. MRI was performed demonstrating a small, evolving acute/subacute abnormality in the midline splenium of the corpus callosum. An EEG showed moderate to severe abnormalities with low-voltage delta waves. The patient was treated with medication and advised to follow up with a neurologist. One month later, no residual CT abnormality corresponding to the previously reported lesion in the midline splenium of the corpus callosum was observed. Although epilepsy is a common finding in patients with cerebral palsy, the complete lack of seizure activity throughout this patient\'s early life, coupled with previously unremarkable brain imaging, further supports our claim that his recent onset of seizures was directly related to COVID-19. This case highlights the possibility of new seizures in patients with pre-existing neurological conditions after COVID-19 infection and emphasizes the need for more research.

Introduction Recent publications have described drug reaction with eosinophilia and systemic symptoms (DRESS) with topiramate. Topiramate has been associated with other severe cutaneous adverse reactions, including Stevens-Johnson syndrome, but a relationship to DRESS has not been established. To determine if there is a causal association between topiramate and DRESS, we conducted a comprehensive review of the data in the Janssen Research & Development Global Safety Database (GSD), signaling databases, and the literature. Methods The primary data were post-marketing reports of DRESS in the Janssen topiramate GSD (cumulative through 1 July 2022), representing >14,000,000 patient-years (PY) exposure. Cases were reviewed, assigned a Registry of Severe Cutaneous Adverse Reaction (RegiSCAR) score, and assessed for overall contribution of topiramate to DRESS based on temporality, concomitant medications, dechallenge/rechallenge, and baseline patient factors. Statistical disproportionality was evaluated in European Medicines Agency\'s EudraVigilance (EV) safety database and the United States Food and Drug Administration Adverse Event Reporting System (FAERS). For EV, the overall disproportionality threshold was the lower limit of the 95% confidence interval (CI) for the reporting odds ratio (ROR025) >1 and N ≥5. The overall threshold for FAERS was the Empirical Bayesian Geometric Mean (EBGM) ≥2, lower bound of the 90% CI (EB05) of >1, and N ≥3. To account for the role of concomitant drugs, Empirical Bayes regression-adjusted arithmetic mean (ERAM) scores were calculated, with a threshold ≥2, a lower bound of the 90% CI (ER05) of >1, and N ≥3. An integrated search of major biomedical literature was performed for reports of topiramate and DRESS. Results There were 17 reports of DRESS in the GSD (reporting rate 0.12/100,000 PY). RegiSCAR scores ranged from -3 to 7 (average -0.4). No cases met full diagnostic criteria and were highly confounded by the presence of other suspect drugs. Disproportionality scores exceeded thresholds for statistical significance in FAERS (N=72, EBGM=2.06, EB05=1.69), but not in EV (N=33, ROR025=0.79). When accounting for co-administered drugs, ERAM was statistically significant for carbamazepine (4.53), lamotrigine (ERAM=6.54), phenytoin (ERAM=2.91), and zonisamide (ERAM=2.25) exceeding disproportionality thresholds, but the score of topiramate was no longer significant (0.25). Conclusion A comprehensive review of all available evidence does not support a causal association between topiramate and DRESS.

There is limited literature on electroconvulsive therapy (ECT) in patients with a severe schizophrenia spectrum illness and concomitant seizure disorder. In addition, it is unclear whether it is safe to perform ECT in a patient with these comorbidities and a history of status epilepticus. This is the case of a 48-year-old patient with a history of schizoaffective disorder, bipolar type, refractory psychosis on clozapine and ECT, and seizure disorder on carbamazepine. She presented to the emergency department with suspected post-ECT delirium four days after her last ECT treatment, was found to be in non-convulsive status epilepticus, and was admitted to the neuroscience intensive care unit. Coma induction was required for seizure control. As she stabilized, her psychosis worsened, and she required psychiatric hospitalization. Multiple factors may have contributed to the development of status epilepticus in this patient. She was on clozapine, which has a time- and dose-dependent risk of seizure that prescribers should be wary of. She had also been prescribed the antiepileptic drug carbamazepine, which induces clozapine and itself, decreasing their effectiveness. Upon the patient\'s discharge, ECT was suspended indefinitely due to concern that it may have led to status epilepticus. However, case reports suggest that intractable seizures following ECT are rare. We found no reports of status epilepticus occurring more than 60 minutes after the completion of ECT. If the benefits of ECT are significant, then it should remain a treatment option for the patient.

Biotin-Thiamine-Responsive Basal Ganglia Disease is an extremely rare autosomal recessive neurometabolic disorder characterized by recurrent waxing and waning episodes of subacute encephalopathy and seizures. High dose biotin and thiamine administration has been shown to improve symptoms within days, and the symptoms may reappear rapidly if supplementation is discontinued. Here we present a case of a 20-year-old male with classical clinical and imaging findings of Biotin-Thiamine-Responsive Basal Ganglia Disease, with a 12-year delay in diagnosis, finally diagnosed after presenting at our institution based on imaging and subsequent reexamination of exome sequencing. In this report, we review the classic imaging findings in this disease and examine why making the diagnosis can be extremely challenging due to its wide differential. Both clinically and radiographically, this condition demonstrates significant overlap with a vast array of disease entities, ranging from viral or autoimmune encephalitis to metabolic disorders. Finally, we discuss the various negative prognostic predictors described in the literature, several of which were observed in this patient\'s clinical course.

The neurobiological underpinnings of functional seizure (FS) development and maintenance represent an active research area. Recent work has focused on hardware (brain structure) and software (brain function and connectivity). However, understanding whether FS are an adaptive consequence of changes in brain structure, function, and/or connectivity is important for identifying a causative mechanism and for FS treatment and prevention. Further, investigation must also uncover what causes these structural and functional phenomena. Pioneering work in the field of psychoneuroimmunology has established a strong, consistent link between psychopathology, immune dysfunction, and brain structure/function. Based on this and recent FS biomarker findings, we propose a new etiologic model of FS pathophysiology. We hypothesize that early-life stressors cause neuroinflammatory and neuroendocrine changes that prime the brain for later FS development following secondary trauma (e.g., traumatic brain injury or psychological trauma). This framework coalesces existing knowledge regarding brain aberrations underlying FS and established neurobiological theories on the pathophysiology of underlying psychiatric disorders. We also propose brain temperature mapping as a way of indirectly visualizing neuroinflammation in patients with FS, particularly in emotion regulation, fear processing, and sensory-motor integration circuits. We offer a foundation on which future research can be built, with clear recommendations for future studies.

Episodic memory impairment and hippocampal pathology are hallmark features of both temporal lobe epilepsy (TLE) and amnestic mild cognitive impairment (aMCI). Pattern separation (PS), which enables the distinction between similar but unique experiences, is thought to contribute to successful encoding and retrieval of episodic memories. Impaired PS has been proposed as a potential mechanism underling episodic memory impairment in aMCI, but this association is less established in TLE. In this study, we examined behavioral PS in patients with TLE and explored whether profiles of performance in TLE are similar to aMCI.
Patients with TLE, aMCI, and age-matched, healthy controls (HCs) completed a modified recognition task that relies on PS for the discrimination of highly similar lure items, the Mnemonic Similarity Task (MST). Group differences were evaluated and relationships between clinical characteristics, California Verbal Learning Test-Second Edition scores, and MST performance were tested in the TLE group.
Patients with TLE and aMCI demonstrated poorer PS performance relative to the HCs, but performance did not differ between the two patient groups. Neither the side of seizure focus nor having hippocampal sclerosis affected performance in TLE. However, TLE patients with clinically defined memory impairment showed the poorest performance.
Memory performance on a task that relies on PS was disrupted to a similar extent in TLE and aMCI. The MST could provide a clinically useful tool for measuring hippocampus-dependent memory impairments in TLE and other neurological disorders associated with hippocampal damage.

Background: Long QT syndactyly syndrome (long QT syndrome type 8), also known as Timothy Syndrome (TS) was first described in 1994 with still <50 case reported in the literature. The full spectrum of the syndrome is not yet known. Results: Here we report a girl who presented with new onset refractory seizures and an undiagnosed cause of intermittent abdominal distention. She also had syndactyly of her fingers and toes and was found to have prolonged QT. Upon further investigations she was found to have a de novo pathogenic variant in CACNA1C, along with Segmental Ileal Dilatation (SID), and subsequently diagnosed with Timothy syndrome. Conclusion: To our knowledge, the association of Timothy Syndrome with Segmental Ileal Dilatation, was not described before.