Biochemically normal adrenal pheochromocytoma following extensive central necrosis in a child with von Hippel-Lindau (VHL) gene mutation

Pheochromocytomas are rare in children. The diagnosis is usually established from a raised urinary or plasma catecholamine or their metabolites. We present a girl aged 11 years who manifested with a hypertensive crisis secondary to an adrenal tumour but with unexpectedly normal urinary metanephrine and catecholamine results. She improved spontaneously following the crisis and underwent surgery later. The histopathological study confirmed a pheochromocytoma with large central necrosis. Her genetic screening reported a pathogenic von Hippel-Lindau gene mutation. Surveillance scan postsurgery detected no other tumours. Following the catecholamine crisis, an acute infarct occurred, resulting in extensive tumour necrosis and subsequent rapid remission of symptoms and paradoxically normal biochemical markers. Although not unheard of in adults, we believe this is the first reported case of an extensive spontaneous necrosis resulting in a biochemically normal pheochromocytoma in a child.

Abstract 16831: Provoked PFO Shunt And Posterior Circulation Stroke: An Underrecognized Association

Case Presentation: A 50 year old man presented with nausea and weakness. MRI brain showed a small acute infarct in the right pons. CT angiography of the head and neck was unremarkable. No thrombus, vegetation, or inter-atrial communication was seen on transthoracic echocardiogram: LVEF was 55-60% with normal left atrial size. No history of atrial fibrillation, hypertension, diabetes or drug abuse was reported; lower extremity duplex was negative for deep venous thrombosis. TSH was normal. Transesophageal echocardiography showed an aneurysmal atrial septum: agitated saline injection did not demonstrate an inter-atrial communication (figure 1). Repeat saline injection during the same procedure with Valsalva maneuver demonstrated a moderate-sized, provoked right-to-left, patent foramen ovale (PFO) shunt (figure 2). Discussion: Physiologically decreased sympathetic innervation spares posterior cerebral circulation from Valsalva-induced vasoconstriction. The disproportionate increase in posterior cerebral blood flow when venous return/cardiac output increases in the immediate post-strain period explains the association of provoked PFO shunt and paradoxical embolism to posterior circulation. Although the association has been described in literature, it remains underappreciated. Recognition of the association expedited secondary prevention of stroke in this non-elderly patient by circumventing the need to exclude atrial fibrillation on ambulatory rhythm monitoring (3-6 months) before referral for PFO closure.

Combined administration of laminin-221 and prostacyclin agonist enhances endogenous cardiac repair in an acute infarct rat heart

Although endogenous cardiac repair by recruitment of stem cells may serve as a therapeutic approach to healing a damaged heart, how to effectively enhance the migration of stem cells to the damaged heart is unclear. Here, we examined whether the combined administration of prostacyclin agonist (ONO1301), a multiple-cytokine inducer, and stem cell niche laminin-221 (LM221), enhances regeneration through endogenous cardiac repair. We administered ONO1301- and LM221-immersed sheets, LM221-immersed sheets, ONO1301-immersed sheets, and PBS-immersed sheets (control) to an acute infarction rat model. Four weeks later, cardiac function, histology, and cytokine expression were analysed. The combined administration of LM221 and ONO1301 upregulated angiogenic and chemotactic factors in the myocardium after 4 weeks and enhanced the accumulation of ILB4 positive cells, SMA positive cells, and platelet-derived growth factor receptor alpha (PDGFRα) and CD90 double-positive cells, leading to the generation of mature microvascular networks. Interstitial fibrosis reduced and functional recovery was prominent in LM221- and ONO1301-administrated hearts as compared with those in ONO1301-administrated or control hearts. LM221 and ONO1301 combination enhanced recruitment of PDGFRα and CD90 double-positive cells, maturation of vessels, and functional recovery in rat acute myocardial infarction hearts, highlighting a new promising acellular approach for the failed heart.

Acute Ischemic Stroke in a Young Child and Its Association with SARS-CoV-2 Infection

The coronavirus disease 2019 (COVID-19) in children has been shown to have lower morbidity and mortality in children as compared with adults. The neurological complications related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are increasingly reported in children, yet the cerebrovascular complications are rare. We report a case of a toddler who presented with right-sided hemiparesis and motor aphasia, with an antecedent history suggestive of COVID-19 infection. The child tested negative on the nasopharyngeal swab for real-time reverse transcription-polymerase chain reaction (RT-PCR), but the serology for anti-SARS-CoV-2 IgG assay was positive. The neuroimaging showed an acute infarct in the left middle cerebral artery territory. A detailed evaluation for causes of childhood stroke was unrevealing, except for the presence of severe iron deficiency anemia (IDA). The child was diagnosed as acute ischemic stroke (AIS) most probably secondary to mild COVID-19 infection. The objective of this case report is to explain the possibility of AIS after a mild COVID-19 infection, complicated by the underlying severe IDA. Therefore, an association between COVID-19 and stroke in children needs to be emphasized and RT-PCR for SARS-CoV-2 as well as serological assay must be included in the workup of stroke in the young.

ROLE OF AGNILEPA IN PAKSHAGHATA (ISCHEMIC STROKE): A CASE STUDY

Stroke is defined as the rapid onset of focal neurological deficit resulting from diseases of the cerebral vasculature and its contents. Stroke represents the third most common cause of death in the developed nations. The prevalence of stroke in India is approximately 200 per 100,000 persons and 9.94% of total deaths. The present article deals with a diagnosed case of ischemic stroke presenting with left sided hemiplegia Acute infarct in the right temporo-parieto-frontal lobe and right ganglio-capsular region with haemorrhagic transformation. The Ayurvedic diagnosis of vama Pakshaghata was made and managed with shamana aushadhi and agnilepa (bahirparimarjana chikitsa). Two assessments were made before and after treatment using the National Institute of Health Stroke Scale (NIH-SS) and CT scan. Maximum improvement was noticed in the symptoms of Stambha, Ruja, Supti, Shotha, Sparsha hani. Even there was also improvement in left upper and lower extremity functions. At the end of the treatment the patient could be able to walk without support. After treatment CT scan showed considerable recovery. Agnilepa plays a major role in the management of Margavaranajanya pakshaghata (acute ischemic stroke) if associated with saama condition, in acute condition. The drugs used for Agnilepa are having Katu Rasa Pradhana, ruksha, teekshna, lekhana guna, ushna veerya, katu vipaka and kaphavataghna, vedanasthapaka, Shothahara, Stambhahara, Suptihara and helpful in regaining of Sparshana. Agnilepa can be practiced as bahirparimarjana chikitsa in pakshaghata, as many Salvana upanaha are mentioned in the chikitsa.

Bilateral loss of taste from a unilateral thalamic infarct

Loss of sense of taste (hypogeusia) involving a part of the tongue can follow acute stroke. We describe a woman with a small right thalamic acute infarct causing bilateral (mainly left-sided) hypogeusia. Her problem remains sufficiently severe to cause distress and nutritional deficit. The anatomical distribution of her problem—cheiro-oral syndrome with concurrent hypogeusia—suggested involvement of adjacent relevant thalamic fibres. We address key considerations in examining taste in research and in practice and discuss issues to address in people with hypogeusia, including swallow deficits, psychological elements of the poststroke condition and nutrition. Dietetic management should include optimising taste stimuli and nutritional support. Introducing more detailed taste assessments into standard practice would likely improve stroke unit care.

Invasive Rhino-Cerebral Mycormycosis

Mr. Rameshwaran, 51 years who admitted in a private hospital with left upper and lower limb weakness and slowing of speech. He is a known case of Diabetes Mellitus, Systemic Hypertension and CVA. He was an allergic to POSCONAZOLE. On the day of admission, he was conscious and oriented. His BP was 160/90mm of Hg. On examination, he was dehydrated, bilateral pedal edema and right side hemiparesis. Investigations reveals that increased serum urea and creatinine, GFR below 26ml/min, low serum sodium and potassium. MRI shows that large acute infarct. ENT opinion reveals that presence of fungal mass in orbit and secondary to vasculitis. He was treated by corticosteroids, diuretics and anti-platelet. He was advised for physiotherapy, speech therapy, restricted salt and fluids.

Development and Clinical Application of a Deep Learning Model to Identify Acute Infarct on Magnetic Resonance Imaging

BackgroundStroke is a leading cause of death and disability. The ability to quickly identify the presence of acute infarct and quantify the volume on magnetic resonance imaging (MRI) has important treatment implications. MethodsWe developed a machine learning model that used the apparent diffusion coefficient and diffusion weighted imaging series. It was trained on 6,657 MRI studies. All studies were labelled positive or negative for infarct (classification annotation) with 377 having the region of interest outlined (segmentation annotation). The different annotation types facilitated training on more studies while not requiring the extensive time to manually segment every study. We initially validated the model on studies sequestered from the training set. We then tested the model on studies from three clinical scenarios: consecutive stroke team activations for 6-months at the hospital that provided training data, consecutive stroke team activations for 6-months at a hospital that did not provide training data, and an international site. The model results were compared to radiologist ground truth interpretations.ResultsThe model performed better when trained on classification and segmentation annotations (area under the receiver operating curve [AUROC] 0.995 [95% CI, 0.992-0.998] and median Dice coefficient for segmentation overlap of 0.797 [IQR, 0.642-0.861]) compared to segmentation annotations alone (AUROC 0.982 [95% CI, 0.972-0.990] and Dice coefficient 0.776 [IQR, 0.584-0.857]). The model accurately identified infarcts for training hospital stroke team activations (AUROC 0.964 [95% CI, 0.943-0.982], 381 studies), non-training hospital stroke team activations (AUROC 0.981 [95% CI, 0.966-0.993], 247 studies), and at the international site (AUROC 0.998 [95% CI, 0.993-1.000], 171 studies). The model accurately segmented infarcts with Pearson correlation comparing model output and ground truth volumes between 0.968-0.986 for the three scenarios.ConclusionsAcute infarct can be accurately detected and segmented on MRI in real-world clinical scenarios using a machine learning model.