Incomplete Kawasaki disease with development of fatal coronary artery thrombosis in a 13-year-old male.

Kawasaki disease (KD) is among one of the most common causes of vasculitis in children. Since KD was first described in 1967, there have been several reports of patients who did not meet the full diagnostic criteria for KD but who ultimately developed significant coronary artery lesions. Children with incomplete KD are at similar risk of developing coronary artery abnormalities to those with complete Kawasaki. A previously healthy 13-year-old Asian male was seen at a clinic for fever, pharyngitis, and conjunctivitis. He was given antibiotics for a presumed streptococcal pharyngitis. Two weeks later, the decedent complained of chest pain, collapsed, and was transported by Emergency Medical Services to a nearby hospital where he was pronounced deceased on arrival. A complete autopsy was done by the local medical examiner. Histologically, all three coronary arteries showed varying degrees of severe transmural lymphoplasmacytic inflammation, marked vascular smooth muscle intimal proliferation, focal destruction of muscular and elastic layers, and luminal stenosis. Some vessels had recent thrombi. We present an example of incomplete KD in an older child and reiterate the importance of obtaining relevant medical history in sudden death cases that come to the Medical Examiner Office, especially in the pediatric age group.

A Case of Severe Cardiac Sarcoidosis with Minimal Pulmonary Involvement: A Case Report with Literature Review.

Sarcoidosis is a granulomatous disease of unknown etiology. Although sarcoidosis is a systemic disease, there appears to be a predilection for involvement of certain organs. The pulmonary system is the most commonly affected system among all racial groups. Cardiac and respiratory complications are the leading causes of death due to sarcoidosis and in certain patient populations about half of these deaths are attributed to cardiac sarcoidosis. There are few autopsy case reports of cardiac sarcoidosis with minimal respiratory involvement making this case report relevant to the importance of the recognition and awareness of this entity. Acad Forensic Pathol. 2018 8(2): 407-415.

A Case of Previously Unsuspected Huntington Disease Diagnosed at Autopsy.

Huntington disease (HD) is a neurodegenerative disorder with a worldwide prevalence of four to ten per 100 000. It is characterized by choreiform movements, behavioral/psychiatric disturbances, and eventual cognitive decline. Symptoms usually present between 30 and 50 years of age and the diagnosis is based on the combination of clinical symptoms, family history, and genetic testing. A variation of HD, juvenile Huntington disease (JHD), presents earlier, with more severe symptoms and with a worse prognosis. Symptoms are different in JHD, with personality changes and learning difficulties being the predominant presenting features. Seizures are common in JHD, and chorea is uncommon; movement disorders at presentation of JHD are predominantly nonchoreiform. The inheritance pattern for both HD and JHD is autosomal dominant and the disease is caused by an elongation of the CAG repeat in the huntingtin gene. There are many published case reports of Huntington disease that were confirmed at autopsy, but to our knowledge, there are no reports in the literature where the diagnosis of Huntington disease was first made at autopsy. We present a case of a 28-year-old African-American male who was in a state of neglect due to a lifetime of abuse, cognitive difficulties, and seizures, whose cause of death was pneumonia. The gross autopsy findings included bilateral caudate nucleus atrophy and lateral ventricular dilation. Microscopically, severe bilateral neuronal loss and gliosis of the caudate and putamen nuclei were seen. Genetic testing for the number of CAG repeats confirmed the diagnosis and was consistent with JHD.