Intra thecal baclofen pump as an ad hoc measure for a case of severe tardive dystonia refractory to multiple lesioning.

Tardive dystonia is an infrequent ailment in patient reliant with chronic antipsychotic medication. The front-line envoy in the treatment of this illness is set into motion with oral agents including baclofen, benzodiazepines, and other antispasmodics. Regardless of an extensive therapy, the patients are not able to control of their spasticity/ dystonia. The authors reported a case of severe tardive dystonia treated with baclofen therapy in a patient frigid to medical therapy and multiple lesioning. Case Report: A 31-year-old female, diagnosed as a case of depressive illness and being managed with neuroleptic medications, who went onto develop tardive dystonia progressively worsening over a 4-year duration. After a comprehensive and meticulous evaluation of her neurological and psychological stratum, globus pallidus interna lesioning was reputed as the best course of action. As intended, staged lesioning was executed bilaterally with a trivial resolution eventually succumbing into recurrence, compelling a repeat lesioning. It was inaptly discouraging to see her crippled with the plight. Determined, not to give upon her, a way out with a baclofen therapy was proposed. A test dose with a 100 mcg of baclofen with an increment up to 150 mcg over a 3-day period demonstrated a promising prospect. On that account, the insertion of the baclofen pump was performed with an outstanding aftermath in her neurological endeavor. Clinical Discussion: Tardive dystonia is believed to be caused by striatal dopamine receptor super-sensitivity persuaded by the dopamine-antagonizing action of antipsychotic drugs. The first line of treatment being oral agents including oral baclofen, benzodiazepines, and antispasmodics. If the patient suffers from an early-onset primary generalized dystonia, then treatment with deep brain stimulation of the globus pallidus interna is the approved and preferred treatment approach. Recurrence of the symptoms despite of multiple lesioning can be overcome by intrathecal baclofen pump infusion as stated by many research. It is not uncommon to face complications in such a procedure, but the benefits outreach the risk, which makes it a choice of treatment. Conclusion: The use of a continuous intrathecal baclofen pump for cases with tardive dystonia refractory to conventional therapy, it has been approved as one of the safest and capable procedures.

Infrared Signatures for Phase Identification in Hafnium Oxide Thin Films.

Phase identification in HfO2-based thin films is a prerequisite to understanding the mechanisms stabilizing the ferroelectric phase in these materials, which hold great promise in next-generation nonvolatile memory and computing technology. While grazing-incidence X-ray diffraction is commonly employed for this purpose, it has difficulty unambiguously differentiating between the ferroelectric phase and other metastable phases that may exist due to similarities in the d-spacings, their low intensities, and the overlapping of reflections. Infrared signatures provide an alternative route. However, their use in phase identification remains limited because phase control has overwhelmingly been accomplished via substituents, thereby convoluting infrared signatures between the substituents and the phase changes that they induce. Herein, we report the infrared optical responses of three undoped hafnium oxide films where annealing conditions have been used to create films consisting primarily of the ferroelectric polar orthorhombic Pca21, antipolar orthorhombic Pbca, and monoclinic P21/c phases, as was confirmed via transmission electron microscopy (TEM), UV-visible optical properties, and electrical property measurements. Vibrational signatures acquired from synchrotron nano-Fourier transform infrared spectroscopy (nano-FTIR) are shown to be capable of differentiating between the phases in a nondestructive, rapid, and nanoscale manner. The utility of nano-FTIR is illustrated for a film exhibiting an antiferroelectric polarization response. In this sample, it is proven that this behavior results from the Pbca phase rather than the often-cited tetragonal phase. By demonstrating that IR spectroscopy can unambiguously distinguish phases in this material, this work establishes a tool needed to isolate the factors dictating the ferroelectric phase stability in HfO2-based materials.

Unusual case of spontaneous bilateral basal ganglia bleed.

Hypertension is the most prevalent determinant condition embarking on the development of spontaneous intracerebral hemorrhage. Usually, the presentation is a unilateral hematoma. Spontaneous bilateral intracerebral hemorrhage is an outstandingly infrequent context, and not a lot of cases have been reported till date. Ensuing hypertension, trauma inflicted on the brain case is another common cause that out turn into the sequelae of bilateral intracerebral hemorrhage. Lately, a few cases of bilateral basal ganglia bleed have been revealed, as a repercussion of COVID-19 infection. Globally, <40 such cases have been reported. A 39-year-old man presented with complaints of acute onset of weakness of right half of the body. Additionally, facial deviation was noticed by his family members. He then sought medical help from a local healthcare center where CT scan of head was advised. Unexpectedly, the scan demonstrated bilateral intracerebral hemorrhage. He was then managed conservatively with oral medications and rehabilitation. The course of his hospital stay was uneventful and was eventually discharged after 9 days. He then presented to our institution for further evaluation. Thereupon, he had slurring of speech but was able to walk with minimal support. Simultaneous Bilateral Basal Ganglia Hemorrhage (SBBGH) is an exceptionally rare ailment. This genre of cerebrovascular accident embraces a comprehensive span of morbidity and mortality. In an acute setting, CT scan of head is the most relevant imaging modality. Nonetheless, MRI is the gold standard for definitive diagnosis and should be performed urgently to further typify and delineate the lesion.

Surgical Management among Patients with Spontaneous Supratentorial Intracerebral Haemorrhage Admitted in a Tertiary Care Centre: A Descriptive Cross-sectional Study.

Introduction: Spontaneous intracerebral haemorrhage is the second most common form of stroke and the most deadly one. An understanding of changing trends in the epidemiology of intracerebral haemorrhage prevalence, its risk factors, current practice in management, case fatality, and long-term outcome is essential to measure the effectiveness of stroke prevention and various treatment efforts. The objective of this study was to find out the prevalence of surgical management among patients with spontaneous supratentorial intracerebral haemorrhage in a tertiary centre. Methods: A descriptive cross-sectional study was conducted in the Department of Neurosurgery from January 2017 to December 2019. Ethical approval was obtained from the Institutional Review Committee (Reference number: 06/2020/IRC-ANIAS). A convenience sampling method was used. Data of the patients were retrieved from online medical records. Point estimate and 95% Confidence Interval were calculated. Results: Among 221 patients with spontaneous supratentorial intracerebral haemorrhage, 115 (52.04%) (45.45-58.63, 95% Confidence Interval) underwent surgical management. In-hospital mortality was seen in 23 (20%) and survivors at 3 months were 78 (67.82%) patients. Conclusions: The prevalence of surgical management among spontaneous supratentorial intracerebral haemorrhages was higher than in other studies done in a similar setting. Keywords: intracerebral haemorrhage; mortality; surgical procedure.

Venture of a Tertiary Care Neurosurgical Center in Course of COVID-19 Lockdown without RT-PCR.

"I will not permit considerations of age, disease or disability, creed, ethnic origin, gender, nationality, political affiliation, race, sexual orientation, social standing or any other factor to intervene between my duty and my patient." Obliged by the aforementioned oath, no medical practitioner shall sit in a moral judgment on any patient but will treat their illness to the best of their ability whatever the circumstances. A clear concord was yet to be authorized after the World Health Organization (WHO) declared the global pandemic of severe acute respiratory syndrome coronavirus 2infection. As a diagnostic modality, WHO recommended real-time reverse transcription-polymerase chain reaction (RT-PCR) as a reliable test; however, its availability in a deprived nation like ours became a major restraining factor. Despite an asset of having high specificity, RT-PCR for coronavirus disease 2019has its own liability of having low sensitivity. Henceforth, as time passed by, the validity of the rapid diagnostic tests was put into question. In later months, a few centers around our periphery started conducting RT-PCR, but the time taken to obtain the result was long-drawn-out process and the patient who needed urgent neurosurgical intervention at Annapurna Neurological Institute and Allied Sciences had to wait. We would like to share our expedition through peaks and valleys of managing 215 patients during the vicious circle of lockdown and global pandemic.

Ganglioglioma Arising from the Septum Pellucidum.

A 24-year-old gentleman presented to us with complaints of occasional headache for 2 years. Magnetic resonance imaging showed enhancing supra sellar mass with nonenhancing cystic components, extending superiorly up to the body of bilateral lateral ventricle, laterally displacing septum pellucidum, and compressing the third ventricle with obstructive hydrocephalus. Hormone profile depicted adrenocorticotropic hormone <5.00 pg/mL, growth hormone 1.32 ng/mL, insulin-like growth factor-1 <3.0 ng/mL. The patient underwent surgical resection. Histological finding was suggestive of ganglioglioma World Health Organization grade I. Customized immunohistochemistry panel was advised and revealed positive CD 56, NSE, and GFAP immunohistochemical stains. Gangliogliomas are less frequent neoplastic lesions confined to only a handful of case reports and studies. Accounting less than 2% of intracranial neoplasms, these lesions primarily affect those in the first 3 decades of their life. As ubiquitous it is in nature, we hereby present a case of ganglioglioma in a young adult male arising from septum pellucidum.

Early internal capsule infarction following globus pallidus internus lesioning for cervical dystonia.

INTRODUCTION AND IMPORTANCE: Ischemia associated with lesioning for the treatment of cervical dystonia is a very rare phenomenon. We reported a case of early internal capsule infarction following GPi lesioning for cervical dystonia. CASE PRESENTATION: A 56-year-old man with one year history of progressive onset of cervical dystonia which was unresponsive to medical therapy. He was planned for bilateral globus pallidus internus pallidotomy. After completion of the procedure on one side and the successful completion of stimulation and test lesion on another side, the patient suddenly developed dysarthria and one sided weakness. Post-operative magnetic resonance imaging revealed acute infarction in the posterior end of the internal capsule. The patient was managed conservatively and underwent physiotherapy and rehabilitation care. He recovered gradually with modified ranking scale 3 on discharge. CLINICAL DISCUSSION: Globus pallidus internus lesioning or ablation is one of the surgical treatment for dystonia and movement disorder. Though considered as a safe technique, various risk are associated with the procedure. Ischemia is one of the associated risk but is a very rare phenomenon. Though the patient did not have any signs of pre-operative ischemia, the occlusion of one of the perforators to internal capsule during ablation may be the cause of intra-operative ischemia. CONCLUSION: Ischemia associated with lesioning in the treatment of cervical dystonia is a very rare phenomenon. Though, ischemia related with radiofrequency ablation is very rare and sporadic, one has to be very cautious during GPi pallidotomy to prevent occlusion of perforators to internal capsule.

Adult-onset Still's Disease: A Case Report.

Adult-onset Still's Disease is a rare auto inflammatory disorder of unknown etiology characterized mainly by high spiking fever, arthritis, evanescent rash and lymphadenopathy. It is a form of systemic onset juvenile rheumatoid arthritis that is encountered in adults, typically between 15-25 years and 36-45years. We here describe a 28 years lady with fever, arthritis of multiple large joints, lymphadenopathy and rash, with negative Rheumatoid factor and evidence of past infection with Ebstein-Barr virus and Parvovirus B19. History, examination findings and investigations showed several features consistent with adult-onset Still's disease along with high ferritin level. After exclusion of probable other diagnosis and use of Yamaguchi criteria, she was diagnosed with adult-onset Still's disease. All the major and minor criteria of Yamaguchi for diagnosis were met. Her disease responded well with steroid, she achieved remission and is currently under maintenance therapy. Keywords: Adult-onset stills disease; arthritis; ferritin; fever.

A child with hypertension and ambiguous genitalia - an uncommon variant of congenital adrenal hyperplasia: a case report.

BACKGROUND: Deficiency in 11ß-hydroxylase as a cause of congenital adrenal hyperplasia is uncommon. It should be considered in the differential diagnosis of hypertension with virilization in any prepubescent child. CASE PRESENTATION: A 12-year-old Asian boy from eastern Nepal presented with pain in his abdomen and hypertension. He was raised as a male but had absent testicles since birth and had precocious puberty. Plasma testosterone, follicle-stimulating hormone, and luteinizing hormone were below baseline level. Basal 17-hydroxyprogesterone was elevated. Magnetic resonance imaging of his pelvis showed presence of Müllerian structures and karyotyping revealed 46,XX genotype. A clinical diagnosis of 11ß-hydroxylase deficiency was made in view of hypertension with severe virilization in a 46,XX individual. Our patient's legal guardian was unwilling for our patient to change gender and because our patient is underage, the condition was well explained to his parents. He was managed with steroids and antihypertensive drugs. He was on regular follow-up; after 2 years there was no hypertension but he developed true puberty with functional ovaries. He was prescribed leuprolide (gonadotropin-releasing hormone analogue), letrozole (aromatase inhibitor), and a continuation of antihypertensive drugs. CONCLUSIONS: This case highlights the importance of a thorough physical examination of the external genitalia at birth and appropriate referral, and addresses issues in the management of such a disorder. Ethical issues pertaining to consent and who is entitled to give it should be clear so that the affected individual will have optimal psychological development and quality of life.