Localized hypertrophic neuropathy: a case report and review of the literature.

The case is presented of a 71-year-old man with a 6-year history of symptoms suggestive of carpal tunnel syndrome, which did not improve despite two surgical procedures. On further investigation, a fusiform enlargement of the median nerve was found above the elbow, which was found on biopsy to be localized hypertrophic neuropathy (LHN). This case is the first to be described affecting the median nerve. The literature regarding LHN is reviewed, with discussion of the differential diagnoses and possible etiology of this rare lesion.

Widespread axonal injury in gunshot wounds to the head using amyloid precursor protein as a marker.

In order to determine whether axonal injury (AI) is a factor in cases of penetrating head injury, the brains of 14 patients who died shortly after sustaining a fatal gunshot wound (GSW) to the head were examined, and the presence of AI determined using immunohistochemical staining for amyloid precursor protein (APP). The distribution of AI was mapped throughout the cerebral hemispheres and brain stem. AI was present in all cases in a diffuse distribution distant to the missle track with severe involvement of the brain stem in all cases. There was no axonal APP immunoreactivity in the direct region of the missle track at the point of primary axotomy. The APP-positive AI in these cases is likely to be a mixture of primary and secondary AI as APP immunostaining is unable to distinguish primary AI due to mechanical deformation from AI secondary to hypoxic-ischemic damage.

Malignant melanoma metastatic to a meningioma.

The case presented is that of a 63 year old man with a metastasis to an intracranial meningioma from a malignant melanoma. Although the phenomenon of tumor to tumor metastasis to a meningioma has been previously reported, this is the first case in the literature to date, in which the primary tumor is a malignant melanoma. The criteria for the diagnosis of tumor-to-tumor metastasis and possible reasons for the frequency of metastasis to meningiomas are briefly reviewed.

The neuropathology of melioidosis: two cases and a review of the literature.

Melioidosis is an infectious disease caused by Burkholderia pseudomallei and is hyperendemic in the Top End of the Northern Territory of Australia, as well as being widespread throughout tropical south east Asia. The infection is primarily acquired via the inoculation of compromised surface tissues by contaminated soils and water and it can cause an acute, rapidly fatal illness. Although pneumonia is the commonest manifestation, neurological presentations have been described, most notably encephalomyelitis. This paper presents the neuropathology of 2 fatal cases of neurological melioidosis and reviews the relevant literature.

Patterns of immunocytochemical staining for ferritin and transferrin in the human spinal cord following traumatic injury.

Normally Fe(2+) is strictly controlled within the central nervous system (CNS) because of its potential to react with oxygen and form free radicals.(1,2) Traumatic spinal cord injury (TSCI) leads to cell damage and haemorrhage, both of which may increase the pool of free iron.(3) The aim of this study was to examine the response to TSCI of the iron storage protein ferritin (Ft) and the iron transport protein transferrin (Tf). The study found a significant increase in Ft positive cells compared to controls and a significant correlation between the number of Ft positive cells and the severity of injury. Significantly fewer Tf positive cells were seen in the trauma cases compared to the control and there was no relation with the severity of injury. These observations suggest a disturbance in normal iron metabolism within the spinal cord following injury, with possible implications for free radical mediated secondary damage.

Osler-Weber-Rendu syndrome--pathological manifestations and autopsy considerations.

An 18-year-old university student with Osler-Weber-Rendu disease collapsed in the bathroom. Attempted resuscitation was unsuccessful. Her past history included recurrent epistaxes, mucosal telangiectasias, intracranial arteriovenous malformations with previous hemorrhage, a single pulmonary arteriovenous malformation, recent onset of grand mal seizures, and depression. There was a positive paternal family history. At autopsy the pulmonary arteriovenous malformation was confirmed. In addition, within the brain there were widespread areas of gliosis with hemosiderin deposition and small vascular malformations. No other significant abnormalities were detected and toxicological screening revealed only moderately elevated levels of carbemazepine. Death was attributed to epilepsy associated with glial scarring from previous microhemorrhages. The detection of vascular malformations in the lung or brain at autopsy should prompt careful examination of all tissues for similar lesions. Given the possibility of Osler-Weber-Rendu disease in such cases, fibroblast cultures should be undertaken to enable molecular studies to proceed. The heritable nature of this disorder necessitates accuracy of diagnosis at autopsy; once the diagnosis is confirmed family screening should be recommended.

Fulminant leucocytoclastic brainstem vasculitis in a patient with otherwise indolent systemic lupus erythematosus.

The spectrum of central nervous system (CNS) vascular pathology in systemic lupus erythematosus (SLE) includes small vessel vasculopathy, thromboembolism, perivascular lymphocytic infiltration and, rarely, overt transmural vasculitis. We present the case of a patient, who experienced three CNS relapses over total disease duration of 26 years, with otherwise indolent disease. The first two relapses were suspicious of vasculitis and the last was proven at autopsy. The short duration between final relapse onset and death in this SLE CNS vasculitis case was, to our knowledge, unique. Histopathological investigation demonstrated multiple confluent areas of haemorrhage in the medulla due to an acute small vessel leucocytoclastic vasculitis.

Is postmortem examination useful in pediatric oncology?

Clinicians are often reluctant to request an autopsy for patients who have been extensively investigated during life, because they may believe that little additional information will be obtained. To examine whether this is the case, the autopsy records of pediatric oncology patients at the Adelaide Children's Hospital were reviewed. A total of 63 oncology patients died in the hospital during the period 1982-1991, 28 of whom underwent postmortem examination. Class I findings (those that would have altered management if they had been known during life) were found in 7 cases (25%). Major pathological findings that would not have altered management (class II) were found in 14.3% of cases. The most common class I findings were fungal infections with organisms such as Aspergillus or Candida species. Postmortem examination also revealed treatment-related complications in five patients, and in one case the diagnosis was made only at autopsy. This study confirms that postmortem examination can provide valuable new information even for patients who had been widely investigated in life and in whom the cause of death may appear obvious.

Fluconazole in the treatment of pulmonary zygomycosis.

Pulmonary zygomycosis is an aggressive, often terminal infection that may be found in patients who are immunocompromised as a result of cytotoxic chemotherapy. Conventional treatment is by surgical debridement augmented with high-dose intravenous amphotericin B, but even with such treatment the course is usually fulminant with a high mortality rate. Recent work has suggested that the new antifungal triazole, fluconazole, may be of benefit in treating zygomycete infection. The case of a 15-year-old boy who developed pulmonary zygomycosis while on chemotherapy for acute lymphoblastic leukaemia, and who survived for 11 months with oral fluconazole therapy alone, is supportive of this proposal.

Unexpected childhood death due to a rare complication of ventriculoperitoneal shunting.

A 10-year-old boy with Arnold-Chiari malformation, spina bifida, and a ventriculoperitoneal shunt for hydrocephalus died unexpectedly, having appeared to be only mildly unwell with fever on the night before death. At autopsy, the shunt was partially obstructed with an associated enterococcal meningitis. The tip of the shunt was located within the transverse colon, which was embedded in a mass of fibrous adhesions resulting from previous abdominal surgery. Blood cultures were sterile. Intestinal perforation is a rare complication of ventriculoperitoneal shunting that may be associated with the development of meningitis and unexpected death. The autopsy assessment of children with such indwelling devices requires examination of the functional state of the shunt, full septic workup, and determination of the precise location of the tip of the catheter within the peritoneal cavity.

Crossbow suicide: mechanisms of injury and neuropathologic findings.

Crossbow injuries are rarely reported events in modern times. Two cases of death due to self-inflicted crossbow injuries to the head are reported in 2 men aged 18 and 27 years, respectively. Despite relatively low velocity and concussive force, the sharpness and propulsion force of crossbow bolts may be sufficient to enable penetration of the skull at short range. Due to the relatively low concussive force of the crossbow bolt, however, death may not be instantaneous but may occur from intraparenchymal cerebral damage sometime thereafter. Detailed neuropathologic evaluation of such cases may therefore demonstrate "red cell" hypoxic injury, as well as axonal injury, not limited to the region of the missile tract, but widely distributed, even to the point of extensive brain stem involvement. These changes may result from primary mechanical deformation at the time of injury, from secondary hypoxic damage, or from a combination of both factors. Immunohistochemical staining of brains for amyloid precursor protein to delineate more clearly the pattern of axonal damage may assist in determining the extent of injury in such cases.

Microfractures in coxarthrosis.

We examined the subchondral bone architecture of the femoral head in relation to trabecular microfracture. Three groups of femoral head specimens were studied. Twenty-eight specimens taken during hip replacement had grade III or IV arthrosis (70 +/- 8 years). From autopsy, 40 femoral heads were obtained, 18 in a group greater than 50 years of age (72 +/- 10 years) and 22 in a group less than 50 years of age (25 +/- 11 years). None of these 40 heads had worse than grade II arthrosis. Coronal slices of the femoral heads were macerated and examined under a dissecting microscope to count trabecular microfractures. For bone histomorphometry, blocks were taken from the subchondral principal compressive and tensile trabeculae. The bone volume, trabecular thickness, and marrow space were quantified. In the subchondral principal compressive region, the arthrotic group had more bone volume, thicker trabeculae, similar trabecular space, and trabecular microfractures when compared with the group greater than 50 years old. In the tensile region, there were no differences except for decreased trabecular microfracture number in the arthrotic group. With the thinnest trabeculae in the compressive region occurring in the greater than 50 years old group, the trabeculae of the younger age group have thinned with age, but with the onset of arthrosis, the thinning is overtaken by pathologic thickening of trabeculae.

Trabecular microfractures. Nature and distribution in the proximal femur.

The nature and distribution of healing trabecular microfractures (TMF) of femoral heads were studied in 50 autopsy specimens, 30 patients with osteoarthritis (OA) of the hip and 45 patients with subcapital fractures of the femoral neck (SFFN). Macerated coronal slices were examined with a dissecting microscope, and the TMFs were identified where there was callus formation, either nodular or smooth. The number of TMFs did not differ significantly in the SFFN and age-matched control groups. The number of TMFs in the OA group was lower than in SFFN and age-matched control groups. The numbers of nodular TMFs in the control and SFFN groups did not differ significantly but were fewer in the OA group. Smooth TMFs were present in similar numbers in OA, control, and SFFN groups. The ratio of the nodular to smooth TMFs regressed with age. The greater proportion of nodular TMFs in older patients was indicative of an early stage of woven bone callus formation. In all three groups (excluding controls younger than 50 years of age), TMFs tended to appear some distance from the midpoint of the trabeculae. The number of TMFs in trabeculae parallel and perpendicular to the direction of principal stress was similar in all three groups.

Lethal manifestations of neurofibromatosis type 1 in childhood.

Neurofibromatosis type 1 (NF-1), also known as peripheral neurofibromatosis or von Recklinghausen's disease, is a common inherited neurocutaneous syndrome. Complications of the disease are numerous and may be fatal, but because the frequency of potentially lethal complications increases with age, deaths from the disease are often thought to occur only in adults. Examination of autopsy records at the Adelaide Children's Hospital for the period 1952 to 1991 revealed four cases of children with NF-1. These cases illustrate a number of interesting pathologic features including a proliferative glial infiltrate involving the subarachnoid space, two primary gliomas within the same patient, gliomatosis cerebri, and sudden death due to massive intratumoral hemorrhage.