Fatal head injury in children younger than 2 years in New York City and an overview of the shaken baby syndrome.

Context: Shaken baby syndrome is a controversial topic in forensic pathology. Some forensic pathologists state that shaking alone is insufficient to explain death and that an impact must have occurred even if there is no impact site on the head.

Objective: To examine a large cohort of fatal, pediatric head injuries for patterns of specific autopsy findings and circumstances that would support or dispute pure shaking as the cause of death.

Hyperextension and rotation of head causing internal carotid artery laceration with basilar subarachnoid hematoma.

Hyperextension of the head can cause injury to the vessels at the base of the brain. These lacerations are believed to be caused by stretching of the vessels due to the abrupt movement of the head and rotational acceleration of the brain within the cranium, and they usually occur in the intracranial portions of the vessels, producing a subarachnoid hemorrhage. This is the case of a 35-year-old man who received a blow to the face that forcefully hyperextended and rotated his head to the left.

Mortality in cerebral palsy (CP): the importance of the cause of CP on the manner of death.

Cerebral palsy (CP) is a nonprogressive motor deficit present or sustained in the perinatal period. We reviewed the files of the Office of Chief Medical Examiner of the City of New York for the 1997-2001 interval seeking those with any mention of cerebral palsy. There were 26 such cases, including 18 natural deaths, three accidents, two homicides, two therapeutic complications, and one death classified as undetermined.

Spontaneous cerebellar hemorrhage in children.

Spontaneous cerebellar hemorrhages are a rare but often fatal occurrence in children. Although there are several predisposing factors such as blood dyscrasias or astrocytomas, the most common cause of cerebellar hemorrhage in an otherwise healthy child is the rupture of a vascular malformation. We reviewed the files of the Office of Chief Medical Examiner of the City of New York and found four such instances over a period of less than two years.

West Nile encephalitis: the neuropathology of four fatalities.

West Nile virus was identified by immunohistochemistry (IHC) and polymerase chain reaction (PCR) as the etiologic agent in four encephalitis fatalities in New York City in the late summer of 1999. Fever and profound muscle weakness were the predominant symptoms. Autopsy disclosed encephalitis in two instances and meningoencephalitis in the remaining two.

The pathology of human West Nile Virus infection.

West Nile Virus (WNV) was identified by immunohistochemistry (IHC) and polymerase chain reaction (PCR) as the etiologic agent in 4 encephalitis fatalities in New York City in the late summer of 1999. The fatalities occurred in persons with a mean age of 81.5 years, each of whom had underlying medical problems.

Neurofibromatosis and other disorders among children with CNS tumors and their families.

We conducted a population-based case-control study with 338 patients, less than 15 years of age, diagnosed with a primary tumor of the central nervous system from January 1968 through December 1977 in 53 New York State counties. The study also included 676 controls selected from the birth certificate files of the New York State Department of Health. We collected information on neurofibromatosis and congenital anomalies in study subjects, their siblings and parents by telephone interview with the mother of each case and control.

An epidemiologic case-control study of central nervous system tumors in children and parental occupational exposures.

A population-based case-control study was conducted with 338 patients less than 15 years of age who were diagnosed with a primary tumor of the central nervous system from January 1968 through December 1977 in 53 contiguous New York counties. The study also included 676 controls who were selected from the birth certificate files of the New York State Department of Health. Parental occupational exposures at the time of each child's birth and at the time of tumor diagnosis were derived from maternal interviews.

A clinicopathological study of 323 patients with oligodendrogliomas.

All patients with oligodendrogliomas (554) from the repository of the Armed Forces Institute of Pathology were retrospectively analyzed. The pathological diagnosis was confirmed in 323 patients and each case was graded according to a previously established grading system. The clinical features of these 323 verified cases of oligodendroglioma are presented, analyzed, and compared with findings in previous studies.

Myoadenylate deaminase deficiency and malignant hyperthermia susceptibility: is there a relationship?

Muscle biopsies from 35 patients referred for possible malignant hyperthermia were subjected to contracture testing with halothane, caffeine, and the combined agents, histopathological and fiber-type-distribution analysis, and quantitative assay of three major muscle enzymes: adenylate deaminase, adenylate kinase, and creatine kinase. Adenylate kinase and creatine kinase were in the normal range in all biopsies and each averaged 92% of expected normal value when corrected for their fiber-type distribution. Of the 14 cases with a positive halothane test, 2 had primary myoadenylate deaminase deficiency, and 5 others had low levels of this enzyme (less than one-third normal).

Spinal metastases. A rare mode of presentation of brain tumors.

An analysis of more than 18,000 primary central nervous system (CNS) tumors revealed only 18 cases (0.01%) in which dropped spinal metastases had caused the presenting symptoms. This group included 11 males and 7 females in whom there was no history of surgical intervention or irradiation.

Levels of adenylate deaminase, adenylate kinase, and creatine kinase in frozen human muscle biopsy specimens relative to type 1/type 2 fiber distribution: evidence for a carrier state of myoadenylate deaminase deficiency.

Myoadenylate deaminase deficiency is believed to reflect a genetic deficiency of skeletal muscle, but its pattern of inheritance has not been established. We examined, histochemically and by quantitative biochemical assay, muscle biopsy specimens from 3 putative carriers of this disorder. Adenylate kinase and creatine kinase were assayed in parallel with adenylate deaminase in order to establish enzyme activity ratios and the variation of each enzyme with fiber-type distribution.

Interstitial 5'-nucleotidase stain for frozen biopsy specimens of skeletal muscle. A useful adjunct in the diagnosis of polymyositis.

We applied a simple lead salt-based stain for interstitial and vascular 5'-nucleotidase to 150 muscle biopsy specimens. No reaction was obtained with 2'- or 3'-adenosine monophosphate, indicating that the stain was specific, and distinct from phosphatases. Staining was not inhibited by alpha, beta-methylene adenosine 5'-diphosphate, but was prevented by formaldehyde fixation or by brief immersion in octoxynol 9 (Triton X-100).

Grading of oligodendrogliomas.

The purpose of this study was to determine the effectiveness of histologic grading of oligodendrogliomas in a retrospective study. 323 cases with adequate clinical information and confirmatory histological material were acquired from the files of the AFIP. A grading system was devised using the following criteria: endothelial proliferation, necrosis, nuclear/cytoplasmic ratio, cell density and pleomorphism.

The course of alcoholic-nutritional peripheral neuropathy.

63 patients with alcoholic-nutritional peripheral neuropathy were given neurologic, electrophysiologic and nutritional examinations. 24 of these patients were reexamined later in the course of their disease, after from 2 to 72 months (mean 33). Alcoholic-nutritional neuropathy appeared and worsened after bouts of heavy alcohol intake and malnutrition.

Diffuse meningeal rhabdomyosarcoma.

Pure rhabdomyosarcomas are extremely rare as primary tumors in the cranial vault. In this instance, a 25-year-old man had symptoms and signs of chronically increased intracranial pressure. A biopsy of the meninges of his posterior fossa was performed revealing a spindle-cell sarcoma.

Stain for skeletal muscle adenylate deaminase. An effective tetrazolium stain for frozen biopsy specimens.

Procedures for staining frozen sections of human muscle biopsy specimens for adenylate deaminase are outlined. The preeminent role of this procedure is as a survey stain for myoadenylate deaminase deficiency, a new and common muscle enzyme deficiency, an example of which is illustrated. Discrimination of type I and II muscle fibers with this stain is based on color differences rather than density differences.

The carcinomatous neuromyopathy of oat cell lung cancer.

A prospective controlled clinical-neurophysiological-pathological study of 71 patients with oat cell carcinoma of the lung revealed no increased incidence of peripheral neuropathy at the initial stages of illness. All patients developed neuropathy by the time they had lost 15% of their body weight, but the neuropathy was less severe than in 20 age-matched alcoholic patients with an equal degree of weight loss. The weight loss and peripheral neuropathy progressed with atrophy of type II (adenosine triphosphatase-positive) muscle fibers out of proportion to the patient's loss of body weight.