Immunosuppressive measles encephalitis in a patient with a renal transplant.

An inclusion body encephalitis developed in a patient with a renal transplant that was shown by immunofluorescence and electron microscopic studies to be due to measles virus. Measles encephalitis may be an opportunistic infection in children and adults with immunodeficiencies secondary to tumors or to the administration of immunosuppressive drugs.     

Legal aspects of electrotherapy in a catatonic renal transplant patient.

The treatment of a case of catatonic schizophrenia in a renal transplanted patient is presented and demonstrates that electrotherapy can be life-saving and that there are already adequate legal controls which guarantee the patient's rights. Yet zealots are attempting to have legislators pass laws preventing the use of electrotherapy even in voluntary patients. To help prevent these resolutions from being passed, psychiatrists must become more active in the legislative process.     

Deep brain stimulation in a patient on immunosuppressive therapy after renal transplant

We performed thalamic deep brain stimulation (DBS) surgery to treat severe essential tremor in a 36 year-old woman who had undergone cadaveric renal transplant four years earlier. She was receiving chronic immunosuppressive therapy. Post-operative healing was normal and there have been no infections of the DBS hardware. There were no peri-operative complications and no rejection of the transplanted kidney. She remains on the same systemic immunosuppressive agents as pre-operatively: prednisone, cyclosporine, and mycophenolate mofetil (CellCept). DBS surgery may be safely performed in carefully selected patients on systemic immunosuppression after renal transplant.     

Dialysis and renal transplant in a hemophiliac

Hemodialysis was initiated in a mild-moderate hemophiliac at 15 years of age. Hematuria had been a frequent and persisting feature from the age of five years without documented cause. Anemia and proteinuria was first detected at 13 years. A cadaver donor renal transplant was carried out after three months of hemodialysis. Massive intravesical bleeding complicated the immediate post-transplantation period. The allograft rejected after three months and the patient was maintained for eight years on home hemodialysis. A second cadaver donor allograft was carried out at 23 years of age. Again, massive intravesical hemorrhage was a problem post-transplant. The allograft is currently functioning 27 months post-transplant. Factor VIIIc activities have fluctuated between 5% and 40% in the absence of factor infusions.     

Retrograde amnesia in a patient with retrosplenial tumour

Abstract

We describe a patient who developed a pure amnesic syndrome due to anaplastic astrocytoma in the retrosplenial region. A dense retrograde amnesia for personal events characterized the syndrome. Learning of new verbal information was spared, while learning of visual material remained persistently poor. The data confirm previous clinical and activation studies suggesting a specific role of the retrosplenial areas in retrieval processes. The hypothesis is that retrieval of autobiographical events may take advantage of visual imagery and that the retrosplenial cortex may play a specific role in human memory by acting as an interface between memory retrieval and visuospatial processes.     

Development of HTLV-I-associated myelopathy after blood transfusion in a patient with aplastic anemia and a recipient of a renal transplant.

We report the development of rapid progressive HTLV-I-associated myelopathy (HAM) after blood transfusion in two immunosuppressed patients, one of whom had aplastic anemia and the other was the recipient of a renal transplant receiving immunosuppressive chemotherapy. Spastic paraparesis developed 11 or 16 months after transfusion and rapidly progressed to a wheelchair-bound state. The present 2 cases suggest that the coexistent immunosuppression may play an important role in the rapid development of HAM in transfusion-acquired cases.     

Lymphangiolipoma of the thoracic spine in a pediatric patient with Proteus syndrome

Proteus syndrome is a rare hamartomatous disorder involving macrodactyly, hemihypertrophy, and subcutaneous lymphangiomas; fewer than 25 cases have been reported worldwide. We report a case of a thoracic epidural lymphangiolipoma in a 5-year-old boy with Proteus syndrome. Computerized axial tomography (CT) of the thoracic spine revealed a left posterior mediastinal mass that extended into the spinal canal through adjacent neural foramina. No sign of spinal cord compression was observed despite the extensive volume of tumor within the spinal canal. Surgical debulking utilizing a T3-10 laminectomy resulted in gross total resection of the tumor. Microscopic examination of the surgical specimen revealed a lymphangiolipoma. No previous report of spinal cord involvement has been reported in this syndrome. A detailed discussion of the phenotypic features and probable mode of genetic transmission is included.     

Right posterior cortical functions in a tumour patient series

It is standardly believed that the localisation of cognitive function by means of impairments arising from cortical tumour is not possible as the functional defects that result are mild and unspecific. These assumptions were not supported in an investigation of four processes generally sensitive to right posterior cortical lesions, when patients with parieto-occipital lesions were compared with prefrontal ones. In three of the tests loading on the individual processes - Reaching Accuracy, Star Cancellation, Fragmented Letters and Cube Analysis - parieto-occipital impairments were found in the basic groups analysis and this was so in the right-hemisphere group. More critically, in these tests Lesion Behaviour Mapping showed the critical lesion site for the tests to have relatively little overlap with those of the other tests, indicating that the cognitive effects were not widespread and diffuse. In addition, in three of the tests the critical lesion sites fitted localisations arrived from other procedures. Patients with high-grade tumours performed considerably worse than those with low-grade tumours in only two of the tests (Star Cancellation, Cube Analysis) particularly in the right parieto-occipital group. In three (Reaching Accuracy, Star Cancellation, Cube Analysis) there was a deterioration with the operation specifically in the low-grade tumour patients. It is suggested that a tumour patient series may provide converging evidence for the localisation of a function initially obtained by some other procedure.     

Stroke in renal transplant recipients

Cerebrovascular events are the most common neurological complications seen in renal transplant recipients. Cerebral infarction and transient ischemic attacks are the most common events and may occur years after transplantation. Recipients older than 40 years at the time of transplantation and those with diabetes mellitus are at greater risk. No instances of aneurysmal subarachnoid hemorrhage occurred among 31 patients with polycystic kidney disease who had undergone transplantation.     

Fatal adenovirus meningoencephalitis in a bone marrow transplant patient

We describe a bone marrow transplant patient with fatal subacute adenovirus meningoencephalitis, the first such patient reported. Neuropathological examination revealed unique, bilaterally symmetrical degeneration in the inferomedial temporal cortex, amygdaloid nuclei, hippocampi, hypothalamus, and some brainstem nuclei. Viral intranuclear inclusions were noted in these areas by light microscopy and confirmed by electron microscopy. Identification was authenticated by viral culture and the isolation of adenovirus from cerebral cortical tissues, and further confirmed by immunofluorescence and serological methods.     

Treatment of tardive dyskinesia with levetiracetam in a transplant patient

Objective –  To describe successful treatment of tardive dyskinesia with levetiracetam.
Background –  Tardive dyskinesia is a late-onset movement disorder caused by exposure to dopamine receptor blocking agents, most commonly neuroleptics. Metoclopramide is frequently used to treat gastrointestinal dysmotility. It has antidopaminergic properties, and is estimated to be responsible for two-thirds of drug-related movement disorders.
Design/methods –  Case report.
Results –  A 68-year-old woman presented with a history of intestinal transplantation (12 years ago; short gut syndrome related to bowel resection for rectal carcinoma) and renal transplantation (1 year ago; diabetes). She developed involuntary movements with stereotypic oro-buccal-lingual dyskinesias and right-sided choreiform movements. Her Abnormal Involuntary Movement Scale score (AIMS) score was 27. She has been treated with metoclopramide for gastrointestinal dysmotility for more than 10 years and was diagnosed with tardive dyskinesia. Treatment with levetiracetam 250 mg orally b.i.d. led to a significant improvement of abnormal movements within a week. Her AIMS score decreased to 8.
Discussion –  Tardive dyskinesia may be quite disabling and options include withdrawal of offending medication, or use of tetrabenazine or reserpine. Several reports also suggested improvement of tardive movement disorders with levetiracetam. In our patient, levetiracetam relieved symptoms of tardive dyskinesia and allowed continuous use of metoclopramide. Larger studies are needed to confirm its efficacy.     

Rosette-forming glioneuronal tumour of the lateral ventricle in a patient with neurofibromatosis 1

We report a 33-year-old man with neurofibromatosis type 1 (NF-1) with a rosette-forming glioneuronal tumour (RGNT) in the lateral ventricle. The patient had been treated with radiotherapy and chemotherapy for a typical juvenile pilocytic astrocytoma of the hypothalamus seven years prior. MRI revealed a contrast-enhancing mass in the anterior horn of the left lateral ventricle. Histological examination demonstrated two distinct regions characterised by a rosette-forming neurocytic component and an astrocytic predominant component. Immunohistochemical studies showed glial fibrillary acidic protein and S-100 positivity in the astrocytic component and neuron-specific enolase was positive in the neurocytic cells. Although RGNT has been reported at other sites within the central nervous system, this report documents a previously undescribed lateral ventricular location of a rare RGNT in an asymptomatic patient with NF-1.     

Antithrombin III in renal transplant recipients

Antithrombin III (AT III) is increased in situations where there is increased platelet turnover. Plasma AT III levels measured in 39 renal transplant recipients were significantly higher than in 20 healthy subjects (p less than 0.001) and 20 patient controls (p less than 0.025). AT III levels were significantly correlated with the patients' platelet counts (r = 0.4, p less than 0.02). Transplant patients with less than 1 year follow up had significantly higher AT III levels than patients with more than 1 year follow up (p less than 0.01). All 5 patients with transplant rejection in the study had elevated plasma AT III levels. The data suggest that elevation of plasma AT III may be related to graft rejection.     

Neurologic problems in renal transplant recipients

Renal transplant recipients are at an increased risk of developing certain neurologic problems. These problems differ from those encountered in recipients of other organs. Development of atherosclerosis is accelerated in renal transplant patients and results in an increased incidence of thromboembolic events. Immunosuppressive therapy predisposes to infection with opportunistic organisms, including reactivation of latent viruses and also is associated with an increased incidence of de novo neoplasia. The transplantation procedure may be complicated by a neuropathy and occasionally by distal spinal cord infarction. Some immunosuppressive agents have a direct adverse effect on the nervous system, particularly when toxic levels accumulate in the body. Uremia prior to, and if present after transplantation, has a toxic effect on the nervous system as well. The reasons for these problems are discussed. Awareness of these special problems in renal transplant patients will facilitate their prevention and diagnosis. A recommended diagnostic approach has been outlined. After the etiology has been established, treatment can be tailored to the individual patient.     

Gadolinium encephalopathy in a patient with renal failure

Gadolinium chelates are extensively used in MRI studies. Neurotoxicity due to gadolinium chelates is minimal and uncommon. A 57-year-old woman in renal failure developed a subacute encephalopathy after inadvertent repetitive gadolinium contrast administration. An unusual MRI appearance with CSF hyperintensity due to gadolinium diffusion into the CSF is also shown.