Hyperosmolar hyperglycaemic state causing atypical status epilepticus with hippocampal involvement.

Diabetes mellitus may arise abruptly and decompensate suddenly, leading to a hyperglycaemic hyperosmolar state. Coma often ensues, although this usually reverses after the metabolic abnormalities have resolved. Acute symptomatic seizures can also occur in patients who are conscious, although these usually resolve after osmolarity and glycaemia have normalised. We describe an elderly woman who failed to regain vigilance despite prompt treatment; the cause was an unusual non-convulsive status epilepticus arising from the mesial temporal lobe and promoting a progressive and selective hippocampal involvement. During follow-up, her seizures recurred after stopping antiseizure medication and she developed hippocampal sclerosis, although she subsequently became seizure-free with antiseizure medications. Patients who are unresponsive in a hyperglycaemic hyperosmolar state may be having subclinical epileptiform discharges and risk developing permanent brain damage and long-term epilepsy.

An extremely high blood glucose level in a child with hyperglycemic hyperosmolar state and type 1 diabetes.

OBJECTIVES: Hyperglycemic hyperosmolar state (HHS) is one of the most severe acute complications of diabetes mellitus (DM) characterized by severe hyperglycemia and hyperosmolality without significant ketosis and acidosis. What is new? Since HHS in the pediatric population is rare and potentially life-threatening, every reported case is very valuable for raising awareness among healthcare professionals. CASE PRESENTATION: A 7-year-old boy with previously diagnosed Joubert syndrome was admitted due to vomiting, polydipsia and polyuria started several days earlier. He was severely dehydrated, and the initial blood glucose level was 115 mmol/L. Based on clinical manifestations and laboratory results, he was diagnosed with T1DM and HHS. The treatment with intravenous fluid was started and insulin administration began later. He was discharged after 10 days without any complications related to HHS. CONCLUSIONS: Since HHS has a high mortality rate, early recognition, and proper management are necessary for a better outcome.

Clinical characteristics and outcome in patients with combined diabetic ketoacidosis and hyperosmolar hyperglycemic state associated with COVID-19: A retrospective, hospital-based observational case series.

AIM: One of the risk factors for poor outcome with SARS-CoV-2 infection is diabetes mellitus; diabetic ketoacidosis (DKA) and hyperosmolar hyperglycemic state (HHS) are the most serious complications of diabetes mellitus. We aimed to explore the clinical characteristics and outcomes of COVID-19 patients presenting with combined DKA/HHS to our institution. METHODS: A retrospective, hospital based observation case series was performed on patients with SARS-CoV-2 admitted to Intensive Care Unit between 3/20/2020 and 4/20/2020. Inclusion criteria were: (1) Blood Glucose >250 mg/dL; (2) Serum bicarbonate <18 mmol/L; (3) Anion Gap >10; (4) serum pH <7.3; (5) ketonemia or ketonuria; (6) effective/calculated plasma osmolality >304 mOsm/kg and (7) positive SARS-CoV-2 RT-PCR. RESULTS: We reported 6 patients who presented during this period with combined DKA/HHS. Their median age was 50 years, all males, three Hispanic, and three African American. Hispanic patients, had more severe acidosis, and multiple comorbidities, with a higher mortality. The striking feature was that combined DKA/HHS was the initial presentation for COVID-19 for most of the cases. DISCUSSION: Our observational retrospective case series shows that diabetic patients are at risk of developing combined DKA/ HHS associated with COVID-19 and a substantial mortality. To our knowledge, we are first to report the clinical characteristics and outcome in this group of patients.

Diazoxide toxicity in a child with persistent hyperinsulinemic hypoglycemia of infancy: mixed hyperglycemic hyperosmolar coma and ketoacidosis.

BACKGROUND: Persistent hyperinsulinemic hypoglycemia of infancy (PHHI), also known as congenital hyperinsulinism, has been known to go into spontaneous remission, with patients developing diabetes in later life. A temporary phase of hyperglycemia is, however, rarely reported. CASE PRESENTATION: We describe a 16-month-old child, a known case of diazoxide responsive PHHI, presenting with mixed hyperglycemic hyperosmolar coma and ketoacidosis with rhabdomyolysis while on diazoxide treatment. The patient required temporary cessation of diazoxide and initiation of insulin infusion, followed by a relapse of hypoglycemia again necessitating diazoxide therapy. CONCLUSIONS: Hyperosmolar coma with ketoacidosis is a rare side-effect of diazoxide therapy, documented even in patients with persistent hyperinsulinemic hypoglycemia of infancy.

Clinical Features and Prognosis of Type 2 Myocardial Infarction in Acutely Decompensated Diabetic Patients.

BACKGROUND: After the introduction of the universal definition of myocardial infarction, the incidence and diagnosis of type 2 myocardial infarction have risen dramatically, yet there are no clear guidelines on clinical management. Diabetic patients are at high risk for developing type 2 myocardial infarction when admitted in a decompensated state, and they are also at high risk for future cardiovascular events. METHODS: We performed a retrospective analysis of 1058 patients admitted with diabetic ketoacidosis or hyperosmolar hyperglycemic state between 2011 and 2016. Patients were included if they had cardiac troponin I measured within 24 hours of admission, were older than 18 years of age, and had no evidence of acute coronary syndrome on admission. Baseline characteristics, admission laboratory test results, major adverse cardiovascular events, cardiac stress testing, and coronary angiography data up to 1 year after admission were reviewed. Patients were categorized into 2 groups: those with and those without type 2 myocardial infarction. The study had 2 endpoints: mortality and major adverse cardiac events (MACE) at 1 year and an abnormal result on stress test or coronary angiography at 1 year. RESULTS: Of the 845 patients who met the inclusion criteria, 133 patients (15%) had type 2 myocardial infarction on admission. Patients with type 2 myocardial infarction were at a significantly higher risk for mortality and MACE at 1 year than those without. Patients with type 2 myocardial infarction were also at higher risk for developing an abnormal result on stress test or coronary angiography within 1 year of admission as compared with those without type 2 myocardial infarction (40% vs 24%; odds ratio 2; P = .0699). CONCLUSION: Acutely decompensated diabetic patients with type 2 myocardial infarction are at increased risk for death and MACE. These patients may also be at risk for undiagnosed coronary artery disease.

Retrospective evaluation of hyperosmolar hyperglycemia in 66 dogs (1993-2008).

OBJECTIVES: To clinically characterize a large group of dogs with the hyperosmolar hyperglycemic state (HHS) and to determine whether 2 HHS subgroups, dogs with hyperosmolar ketonuric (HK) diabetes mellitus (DM) and dogs with hyperosmolar nonketonuric (HNK) DM were clinically different from one another. DESIGN: Retrospective study. Records of 1,250 diabetic dogs that were examined between January 1993 and July 2008 were reviewed in order to identify dogs with HHS. Inclusion required a calculated serum osmolality ≥325 mOsm/kg, with or without ketonuria. SETTING: University teaching hospital. ANIMALS: Sixty-six dogs with HHS including 34 dogs with HK, 25 dogs with HNK, and 7 dogs with unclassified HHS. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: HHS was diagnosed in 5% of dogs with DM. HK and HNK dogs were similar to one another in regard to most historical, physical examination, and clinicopathologic variables as well as outcome. Sixty-two percent of dogs with HHS survived to discharge from the hospital. Poor outcome of HHS dogs was associated with abnormal mental status (P = 0.03) and a low venous pH (P = 0.045). Dogs with HK were significantly more likely to have acute pancreatitis (P = 0.046), higher body temperature (P = 0.006), higher WBC count (P = 0.01), and a shorter duration of clinical signs (P = 0.02) compared to dogs with HNK. Dogs with HNK had significantly higher BUN and creatinine concentrations (P = 0.0002 and P = 0.008, respectively) and higher calculated osmolality (P = 0.001) compared to dogs with HK. CONCLUSIONS: HHS is a rare condition in which poor outcome is associated with abnormal mental status and low venous pH. Among dogs with HHS, the subgroup of dogs with HK has significantly more acute pancreatitis, shorter duration of clinical signs, and higher body temperature and WBC count compared to dogs with HNK, whereas dogs with HNK have more azotemia and higher calculated osmolality compared to dogs with HK.

Etiological profile of epilepsia partialis continua among adults in a tertiary care hospital.

BACKGROUND: Epilepsia partialis continua (EPC), is a subtype of status epilepticus, have a varied spectrum of etiology and the out-come depends on the etiology. AIMS AND OBJECTIVES: The present study is aimed to analyze the clinical characteristics and outcome. MATERIALS AND METHODS: This is a prospective analysis of 17 patients admitted to our center between August 2010 and April 2012. EPC was defined as regular or irregular clonic muscular twitches affecting a limited part of the body, occurring for a minimum of 1 h, and recurring at intervals of no more than 10 s. The data collected included etiology, radiological findings, electroencephalogram (EEG) abnormalities, associated comorbid conditions, and outcome. RESULTS: The mean age at presentation was 44.26 ± 13.77 years and the mean duration was 2.7 ± 1.5 days. There were ten patients with diabetic non-ketotic hyperosmolar state and one patient each of oligodendroglioma, varicella zoster vasculitis, central nervous demyelination, ischemic stroke, post traumatic seizure, arteriovenous malformation, and in one patient no cause could be established. Imaging showed abnormality only in five patients and EEG was abnormal in four patients. The EPC was controlled by one antiepileptic drug (AED) in eight patients, with two AEDs in seven patients and two patients required three AEDs. CONCLUSION: EPC is a rare type of focal motor status epilepticus. Treatment of the underlying cause in addition to controlling EPC is essential to achieve the good outcomes.

Coma hiperosmolar / Hyperosmolar coma

El coma hiperosmolar, hiperglicémico, no cetoacidótico, o más conocido como hiperosmolar, es un grave trastorno metabólico que puede verse en determinados pacientes diabéticos cuando, a expensas, sobre todo de una hiperglicemia severa, mayor de 500 mg/dL, y en menor grado, de una hipernatremia absoluta. Se origina por el aumento de la osmolaridad plasmática, estableciéndose con un cuadro de deshidratación intensa, tanto intracelular como extracelular, con toma de la conciencia (que puede llegar hasta el coma). En el coma hiperosmolar se destaca la escasa existencia o la ausencia total de la cetoacidosis acompañante. Es característica su elevada mortalidad, cuando el referido coma no es diagnosticado a tiempo o no se aplican las medidas terapéuticas adecuadas(AU)

Hyperosmolar, hyperglycemic, non-ketoacidotic coma, or better known as hyperosmolar, is a serious metabolic disorder that can be seen in certain diabetic patients when, at the expense, especially of severe hyperglycemia, greater than 500 mg / dL, and to a lesser degree , of an absolute hypernatremia. It originates from the increase in plasma osmolarity, establishing itself with a picture of intense dehydration, both intracellular and extracellular, with awareness (which can lead to coma). In hyperosmolar coma, the scarce existence or total absence of the accompanying ketoacidosis stands out. Its high mortality is characteristic, when the referred coma is not diagnosed in time or the appropriate therapeutic measures are not applied(AU)

Progressive vacuolating glycine leukoencephalopathy with pulmonary hypertension.

To report two unrelated patients with a new phenotype of nonketotic hyperglycinemia associated with idiopathic pulmonary hypertension. Clinical findings included rapidly progressive neurological deterioration with onset in the first year of life characterized by developmental regression without seizures or electroencephalogram abnormalities during follow-up. Both patients died before the age of 18 months. Glycine cleavage system deficiency was confirmed by enzymatic studies in frozen liver. Molecular analysis in the related genes showed no pathogenic mutation. Radiological and pathological findings were consistent with progressive vacuolating encephalopathy. Our patients with biochemical and enzymatic parameters consistent with atypical nonketotic hyperglycinemia. The clinical and radiological evolution, as progressive vacuolating leukoencephalopathy and the association with pulmonary hypertension constitute a previously unrecognized variant.

Suicide of a diabetic by inducing hyperglycemic coma.

Deaths due to hyperglycemic and hyperosmolar coma in diabetics are usually disease-related. In the exceptional case reported here there was evidence for an intentional ingestion of a sugar solution, the person was a diabetic and known to be depressive suggesting a suicidal intention. The autopsy findings were inconspicuous and only further laboratory findings led to the final diagnosis.

Medical examiner cases associated with previously undiagnosed diabetes mellitus, North Carolina, 1994.

BACKGROUND: Approximately half of the 16 million Americans with diabetes mellitus (DM) are unaware they have the disease; the epidemiology of death from previously undiagnosed DM is unclear. We report medical examiner (ME) cases of death from DM in North Carolina in 1994 to determine the number and characteristics of these cases. METHODS: Deaths from DM ICD-9 were identified and reviewed. RESULTS: Of the 42 cases ascertained from ME records, 6 had previously undiagnosed DM, 5 had diabetes ketoacidosis, and 1 had nonketotic hyperosmolar hyperglycemia. Mean patient age at death for all cases was 42 years (range, 35 years to 57 years). Four patients felt bad at least 24 hours before death, indicating that they are not technically "sudden deaths." CONCLUSIONS: We provide a limited epidemiologic perspective of the phenomenon of death from undiagnosed DM. We suggest further investigation of mortality from previously undiagnosed diabetes.

Clinical characteristics and outcome of hyperglycaemic emergencies in Johannesburg Africans.

In this prospective analysis we investigated the clinical characteristics of black South African diabetic patients admitted to hospital with hyperglycaemic emergencies. The study cases were selected from the medical admissions to an urbanized, Johannesburg academic hospital over a period of 12 months. Only patients with severe diabetic ketoacidosis (DKA) or hyperosmolar non-ketotic hyperglycaemia (HNKH) as defined in the text were included. Over the study period, we identified 58 patients with severe DKA (M: 32, F: 26) and 24 with HNKH (M: 14, F:10). Thirty-two of the patients with DKA (55.2%) were classified as having non-insulin dependent (Type 2) diabetes mellitus (NIDDM). Compared to the 26 subjects with insulin-dependent (Type 1) diabetes mellitus (IDDM), the NIDDM patients were older (51.7 vs 27.7 years) and had a significantly higher body mass index (BMI) (29.4 vs 23.5 kg m(-2), p = 0.002), and glucose levels 47.5 vs 34 mmol l(-1) p = 0.004). Mortality from DKA was 6.8 % and from HNKH 16.6%. Infection was the leading precipitating factor for both DKA and HNKH, followed by first presentation and noncompliance. We conclude that the majority of urban African patients admitted to hospital with DKA have NIDDM. Mortality from DKA among the black Africans in Johannesburg is low and comparable to the mortality in western Europe.

Neurophysiological and anatomical correlations in neonatal nonketotic hyperglycinemia.

Electroencephalographic (EEG) and brainstem auditory evoked response (BAER) findings have not been previously described and correlated with the pathological findings in an autopsied case of neonatal nonketotic hyperglycinemia (NKH). A 38 week gestation male infant presented within two hours of age with stimulus-evoked myoclonus and seizures in the context of progressive coma. Electrographic studies demonstrated cortical myoclonus and electrical seizures exquisitely localized to the midline region as well as a suppression-burst background disturbance. These vertex spike discharges were elicited after tactile stimulation. Prolonged intra-axial latencies for waves III and V were recorded on the BAER on the second day of life. Spongy leukodystrophy was noted on gross and microscopic examination of the brain involving all myelinated tracts especially in the reticular activating system, cerebellar peduncles and optic tracts. Neuropathological confirmation of brainstem involvement emphasizes the role of the nonspecific diffuse somatosensory projection system in the generation of myoclonus and stimulus-evoked seizures in the comatose patient with NKH.