Sudden Death Due to Undiagnosed Ludwig’s Angina: An Autopsy Case Report / 대한법의학회지

Ludwig’s angina is a rapidly progressive and gangrenous cellulitis in the submandibular, sublingual spaces, and neck. It is characterized by an elevated tongue and laryngeal edema, resulting in airway compromise and, rarely, in sudden death. The most common cause is an odontogenic infection of a molar tooth. Infection can spread to the superior mediastinum and buccal area through anatomical structures. Herein, we report the sudden death of a 45-year-old male who was not diagnosed with Ludwig’s angina. He died in the emergency room 20 minutes after loss of consciousness. Intubation was impossible owing to severe laryngeal edema. The C-reactive protein level was 33.81 mg/dL. On autopsy, a diffuse light green abscess of the submandibular space was detected, along with severe edema of the epiglottis, aryepiglottic fold, and vocal cords. Subsequently, we confirmed that the airway obstruction was caused by Ludwig’s angina based on a literature review. In conclusion, this report emphasizes the need to identify the infection source and perform a detailed dissection according to the anatomical structure in the autopsy procedure for Ludwig’s angina.

Validity and Reliability of the Korean-Translated Version of the International Cooperative Ataxia Rating Scale in Cerebellar Ataxia

Objective@#The International Cooperative Ataxia Rating Scale (ICARS) is a semiquantitative clinical scale for ataxia that is widely used in numerous countries. The purpose of this study was to investigate the validity and reliability of the Korean-translated version of the ICARS. @*Methods@#Eighty-eight patients who presented with cerebellar ataxia were enrolled. We investigated the construct validity using exploratory factor analysis (EFA) and confirmatory factor analysis (CFA). We also investigated the internal consistency using Cronbach’s α and intrarater and interrater reliability using intraclass correlation coefficients. @*Results@#The Korean-translated ICARS showed satisfactory construct validity using EFA and CFA. It also revealed good interrater and intrarater reliability and showed acceptable internal consistency. However, subscale 4 for assessing oculomotor disorder showed moderate internal consistency. @*Conclusion@#This is the first report to investigate the validity and reliability of the Korean-translated ICARS. Our results showed excellent construct and convergent validity. The reliability is also acceptable.

A Case of Pathologically Confirmed Corticobasal Degeneration Initially Presenting as Progressive Supranuclear Palsy Syndrome

Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) overlap clinically with parkinsonism or extrapyramidal signs and pathologically with tauopathy. Asymmetric parkinsonism and cortical dysfunctions are classical features of CBD. However, symmetric parkinsonism, frequent falls, and supranuclear gaze palsy are key features of PSP. Despite biochemically classified as 4R tauopathies, tufted astrocytes of PSP and astrocytic plaque of CBD show pathologically important differences. Herein, we report a 68-year-old man with pathologically confirmed CBD. He was clinically suspected to have PSP because of progressive gait disturbances, frequent falls, and vertical saccade limitation. Neurological examination performed at age 71 revealed symmetrical bradykinesia, axial rigidity, and postural instability with worsening of early existing symptoms. Magnetic resonance imaging of the brain taken at age 70 detected midbrain and left frontotemporal atrophy and right middle cerebral artery infarction. Left frontotemporoparietal hypometabolism and asymmetrically decreased fluoro-propyl-carbomethoxy-iodophenyl-tropane uptake in the basal ganglia were observed. The autopsy was performed at the time of his death (at age 72), which revealed severe pallor of the substantia nigra and mildly hypopigmented locus ceruleus.AT8 immunohistochemistry and Gallyas staining revealed tau-positive neuronal and glial inclusions, astrocytic plaques, ballooned neurons, and numerous threads in both gray and white matter. No abnormal inclusions were revealed by beta-amyloid, α-synuclein and TDP-43 immunohistochemistry. In our case, cerebral infarction, periventricular and deep white matter ischemic changes, and midbrain atrophy were likely to produce PSP–CBD overlapping symptoms. However, our patient was finally confirmed to have CBD based on pathological findings such as astrocytic plaques.

Are the Current Guidelines Sufficient to Establish Infection Control Strategies for COVID-19 Related Issues in Hospitals?

As hospitals cater to elderly and vulnerable patients, a high mortality rate is expected if a coronavirus disease 2019 (COVID-19) outbreak occurs. Consequently, policies to prevent the spread of COVID-19 in hospital settings are essential. This study was conducted to investigate how effectively national and international guidelines provide recommendations for infection control issues in hospitals. After selecting important issues in infection control, we performed a systematic review and analysis of recommendations and guidelines for preventing COVID-19 transmission within medical institutions at national and international levels. We analyzed guidelines from the World Health Organization, Centers for Disease Control and Prevention, European Centre for Disease Prevention and Control, and Korea Disease Control and Prevention Agency. Recent guidelines do not provide specific solutions to infection control issues. Therefore, efforts need to be made to devise consistent advice and guidelines for COVID-19 control.

Clinical Characteristics of Patients with Adrenal Insufficiency and Fever

Background@#Because persistent fever often occurs in adrenal insufficiency, it might be confused with infectious diseases. This study aimed to identify clinical characteristics and risk factors of patients with adrenal insufficiency and fever. @*Methods@#All adult patients (n = 150) admitted to a tertiary care hospital in South Korea and diagnosed with adrenal insufficiency between 1 March 2018, and 30 June 2019, were recruited. Patients were excluded if they had: 1) proven structural problems in the adrenal or pituitary gland; 2) a history of chemotherapy within 6 months prior to the diagnosis of adrenal insufficiency; and 3) other medical conditions that may cause fever. @*Results@#Among the included patients, 45 (30.0%) had fever at the time of the diagnosis of adrenal insufficiency. The mean C-reactive protein level was higher (11.25 ± 8.54 vs. 4.36 ± 7.13 mg/dL) in patients with fever than in those without fever. A higher proportion of patients with fever changed antibiotics (33.3% vs. 1.0%). On multivariate logistic regression analysis, female sex (odds ratio [OR], 0.32) lowered the risk of adrenal insufficiency with fever, while a history of surgery within 6 months (OR, 4.35), general weakness (OR, 7.21), and cough (OR, 17.29) were significantly associated with that. @*Conclusion@#The possibility of adrenal insufficiency should be considered in patients with fever of unknown origin, especially those with risk factors.

Clinical Characteristics of Patients with Adrenal Insufficiency and Fever

Background@#Because persistent fever often occurs in adrenal insufficiency, it might be confused with infectious diseases. This study aimed to identify clinical characteristics and risk factors of patients with adrenal insufficiency and fever. @*Methods@#All adult patients (n = 150) admitted to a tertiary care hospital in South Korea and diagnosed with adrenal insufficiency between 1 March 2018, and 30 June 2019, were recruited. Patients were excluded if they had: 1) proven structural problems in the adrenal or pituitary gland; 2) a history of chemotherapy within 6 months prior to the diagnosis of adrenal insufficiency; and 3) other medical conditions that may cause fever. @*Results@#Among the included patients, 45 (30.0%) had fever at the time of the diagnosis of adrenal insufficiency. The mean C-reactive protein level was higher (11.25 ± 8.54 vs. 4.36 ± 7.13 mg/dL) in patients with fever than in those without fever. A higher proportion of patients with fever changed antibiotics (33.3% vs. 1.0%). On multivariate logistic regression analysis, female sex (odds ratio [OR], 0.32) lowered the risk of adrenal insufficiency with fever, while a history of surgery within 6 months (OR, 4.35), general weakness (OR, 7.21), and cough (OR, 17.29) were significantly associated with that. @*Conclusion@#The possibility of adrenal insufficiency should be considered in patients with fever of unknown origin, especially those with risk factors.

Validation Study of the Official Korean Version of the Movement Disorder Society-Unified Parkinson’s Disease Rating Scale

Background@#and Purpose: The Movement Disorder Society-Sponsored Revision of the Unified Parkinson’s Disease Rating Scale (MDS-UPDRS) is widely used for estimating the symptoms of Parkinson’s disease. Translation and validation of the MDS-UPDRS is necessary for non-English speaking countries and regions. The aim of this study was to validate the Korean version of the MDS-UPDRS. @*Methods@#Altogether, 362 patients in 19 centers were recruited for this study. We translated the MDS-UPDRS to Korean using the translation-back translation method and cognitive pretesting. We performed both confirmatory and exploratory factor analyses to validate the scale.We calculated the comparative fit index (CFI) for confirmatory factor analysis, and used unweighted least squares for exploratory factor analysis. @*Results@#The CFI was higher than 0.90 for all parts of the scale. Exploratory factor analysis also showed that the Korean MDS-UPDRS has the same number of factors in each part as the English version. @*Conclusions@#The Korean MDS-UPDRS has the same overall structure as the English MDSUPDRS. Our translated scale can be designated as the official Korean MDS-UPDRS.

The Clinical Presentations and Prognosis of Non-Ketotic Hyperglycemia Induced Seizure

BACKGROUND: Seizure can be triggered by the non-ketotic hyperglycemia (NKH). Recently we analysed 18 cases of NKH induced seizure to identify the causes for NKH, seizure types, prognosis, and the differences of clinical presentation between the patient with chronic brain structural lesion (CBSL) and the patient without. METHODS: Eighteen patients with NKH induced seizure were selected from the database. Data regarding brain images, clinical symptoms, co-morbid illnesses, blood laboratories, and prognosis were collected. Patients were divided into two groups according to the presence of CBSL. RESULTS: The patients with CBSL showed more generalized tonic-clonic seizure (GTCS) than without. Focal seizures in this group appeared to be originated from the pre-existing lesion in many situations. The poor compliance to anti-diabetic treatment and physical stresses were most common causes for NKH. One year seizure remission without anti-epileptic drug treatment was achieved in 17 of 18 patients. CONCLUSIONS: The patients with CBSL might have more GTCS than without. The impairment of inhibitory mechanism surrounding the focal irritative zone might be one of plausible explanation for this phenomenon. The prognosis was favorable. Further large studies are required.

Validity and Reliability Study of the Korean Tinetti Mobility Test for Parkinson's Disease

OBJECTIVE: Postural instability and gait disturbance are the cardinal symptoms associated with falling among patients with Parkinson’s disease (PD). The Tinetti mobility test (TMT) is a well-established measurement tool used to predict falls among elderly people. However, the TMT has not been established or widely used among PD patients in Korea. The purpose of this study was to evaluate the reliability and validity of the Korean version of the TMT for PD patients. METHODS: Twenty-four patients diagnosed with PD were enrolled in this study. For the interrater reliability test, thirteen clinicians scored the TMT after watching a video clip. We also used the test-retest method to determine intrarater reliability. For concurrent validation, the unified Parkinson’s disease rating scale, Hoehn and Yahr staging, Berg Balance Scale, Timed-Up and Go test, 10-m walk test, and gait analysis by three-dimensional motion capture were also used. We analyzed receiver operating characteristic curve to predict falling. RESULTS: The interrater reliability and intrarater reliability of the Korean Tinetti balance scale were 0.97 and 0.98, respectively. The interrater reliability and intra-rater reliability of the Korean Tinetti gait scale were 0.94 and 0.96, respectively. The Korean TMT scores were significantly correlated with the other clinical scales and three-dimensional motion capture. The cutoff values for predicting falling were 14 points (balance subscale) and 10 points (gait subscale). CONCLUSION: We found that the Korean version of the TMT showed excellent validity and reliability for gait and balance and had high sensitivity and specificity for predicting falls among patients with PD.

Family Functioning and Communication in Spouses of Patients with Parkinsonism / 가정의학회지

BACKGROUND: Patients with parkinsonism exhibit motor symptoms, cognitive impairment, and neuropsychiatric changes, and these symptoms increase caregiver burden. Family dynamics can be influenced by the presence of comorbidities, which is especially important in diseases causing caregiver burden. We investigated the effects of spousal parkinsonism on family functioning and communication. METHODS: Couples without parkinsonism, who visited hospital-based family practices, were recruited by 28 family physicians from 22 hospitals between April 2009 and June 2011; patients with parkinsonism and their spouses were recruited from a single institution. The participants completed questionnaires on demographic characteristics, lifestyle factors, family functioning (the Korean version of the Family Adaptation and Cohesion Evaluation Scale [FACES] III), and family communication (the Family Communication Scale of the FACES-IV). We compared family functioning and communication between spouses of the patients with and without parkinsonism. RESULTS: The mean family adaptability and cohesion scores of the spouses of the patients with parkinsonism were 23.09±6.48 and 32.40±8.43, respectively, whereas those of the control group were 23.84±5.88 and 34.89±7.59, respectively. Family functioning and family communication were significantly different between the spouses of individuals with and without parkinsonism. After adjusting for age, sex, income, and cardiovascular disease in the logistic regression analysis, family functioning was found to significantly deteriorate in the spouses of patients with parkinsonism but not the control group. Family communication decreased significantly in spouses of patients with parkinsonism. CONCLUSION: Family functioning and family communication significantly deteriorated in spouses of patients with parkinsonism.

A novel mutation of CACNA1A gene in episodic ataxia type 2 family in Korea

Episodic ataxia type 2 (EA-2) is a rare disorder presenting with paroxysmal vertigo and cerebellar dysfunction. EA-2 is known to be caused by mutations of the CACNA1A gene on chromosome 19q13. We examined a family of EA-2 with a novel mutation of the CACNA1A gene showing characteristic ocular symptoms. A-36-year woman visited our hospital with paroxysmal vertigo. When she experienced vertigo attack, she also suffered from gait disturbance, dysarthria, and ataxia. She complained that she could not ride in a car or a train that moved fast, because she could not visually follow the moving objects. Her mother, grandmother, and uncle also complained of similar symptoms. Video nystagmographic findings showed loss of optokinetic nystagmus. We found a novel missense mutation, R279C (c.835C>T), on exon 6 in the CACNAIA gene. This is the first report of a family with new mutation of EA-2 in Korea.

A Survey of the Use of Antiepileptic Drugs in Stroke Patients

BACKGROUND: Seizures occur in 2-20% of stroke patients. Recent studies have reported that post-stroke seizures are associated with poorer functional outcomesand higher mortality. However there are no official guidelines on how to use antiepileptic drugs (AEDs) in stroke-related seizures. In this study we surveyed neurologists and neurosurgeons and compared the responses of subgroups categorized by department, specialty and workplace discrimination using a questionnaire containing questions concerning the present tendency to use AEDs in stroke patients. METHODS: 256 neurologists and neurosurgeons participated in the survey. The research instrument was a questionnaire comprising 9 parts and 30 questions. The questions concerned stroke mechanism, the prophylactic use of AEDs, and the choice of AED in early and late onset post-stroke seizures. RESULTS: Tendencies to use prophylactic AEDs in stroke differed depending on specialty and workplace(neurologist vs. neurosurgeon; 17.8% vs. 83.1%, p<0.001, hospital vs. university staff; 46.2% vs. 28.4%, p=0.05). The most commonly used prophylactic AEDs were valproic acid (75%) and levetiracetam (60%). Carbamazepine was the most commonly used AED and phenytoin and phenobarbital were still used in all subgroups to treat post-stroke seizures. CONCLUSIONS: There are significant differences between neurologists (17.8%) and neurosurgeons (83.1%) in the use of prophylactic AEDs after stroke. Valproic acid and levetiracetam are considered first-line prophylactic AEDs by neurosurgeon. Phenytoin and phenobarbital are still used in post-stroke seizure although they have been reported to have an adverse influence on motor recovery. We suggest that proper guidelines should be established for the use of AEDs in stroke-related seizures.

Relapsing Herpes Simplex Encephalitis

Herpes simplex encephalitis (HSE) is the most common type of viral encephalitis and a fourteen day administration of acyclovir is well-known as the treatment of choice for HSE. Occasionally HSE relapses, but rarely with acyclovir treatment. We report a case of relapsing HSE after treatment with acyclovir for 14 days. Our case suggests that patients with progressive high signal intensities in diffusion-weighted brain MRIs might need longer antiviral therapy over 14 days for preventing the relapse of herpes simplex encephalitis.

A Case of Ipsilateral Hemiageusia Following Right Middle Cerebral Artery Infarction

Taste disorder is not rare in patients with acute stroke is not usually evaluated. There is also controversy about central gustatory pathways. We report one case of ipsilateral hemiaguesia induced by a right middle cerebral artery infarction. A right-handed 70-year-old woman presented with headache. She had a history of hypertension and diabetes mellitus. The systemic examination revealed no abnormalities. Neurologic examination revealed left side hemispatial neglect and right side hemiageusia. Brain MRI showed high signal intensity in the right middle cerebral artery territory including the temporal lobe and parietal lobe with sparing insular cortex on T2 and diffusion weighted image (DWI). This case is suggestive of the existence of an uncrossed ascending gustatory pathway, explaining the ipsilateral hemiageusia above the midbrain lesion.

Transient Upbeat Nystagmus Due to Unilateral Pontine Infarction

Upbeat nystagmus has been described in bilateral lesions of the medulla, the ventral tegmentum, the anterior cerebellar vermis, the adjacent brachium conjunctivum, and the midbrain. Imbalance of the vertical vestibulo- ocular reflex (VOR) favoring the downward VOR activity would therefore result in upbeat nystagmus. We report a patient with transient upbeat nystagmus due to unilateral pontine infarction that may have disrupted bilateral upward VOR pathways running in the ventral tegmental tracts.