1/90. Cranio-cerebral erosion: delayed diagnosis and treatment. Cranio-cerebral erosion is a well-known complication of calvarian fracture with underlying dural tear and cerebral injury in infancy and early childhood. The anatomy, pathogenesis and natural evolution of these lesions remain obscure. The common clinical symptoms are seizures, focal neurological deficits, impairment of consciousness and a soft subgaleal mass. Three patients of cranio-cerebral erosion who underwent delayed surgery in their adult lives are presented to illustrate the common and uncommon features, and their long-term outcome is discussed. ( info) |
| OBJECTIVE: To investigate the incidence and characteristics of patients with structural central nervous system (CNS) lesions and cerebrospinal fluid oligoclonal IgG bands. DESIGN: A retrospective study. METHOD: The medical records of patients with cerebrospinal fluid oligoclonal IgG bands were evaluated for the presence of structural CNS lesions, their location and cause, and for clinical characteristics. SETTING: cerebrospinal fluid oligoclonal IgG bands were examined in the Neuroimmunology Laboratory, Hadassah University Hospital, Jerusalem, israel. patients: Two hundred seventy of 570 patients with positive cerebrospinal fluid oligoclonal IgG bands were available for analysis. Twenty patients had structural CNS lesions. RESULTS: Twenty (7.5%) of the 270 patients had structural CNS lesions: 3 patients had spinal arteriovenous malformation; 5 patients had tumors; 9 patients had compressive cervical myelopathy. Traumatic leukomalacia, arnold-chiari malformation type 1, and CNS hemosiderosis were present in 1 patient each. In 2 patients (1 patient with recurrent meningioma and 1 patient with posttraumatic encephalomalacia) the presence of a structural CNS lesion was followed by the development of multiple sclerosis. In all 3 patients with spinal arteriovenous malformation, oligoclonal IgG identification prolonged the time to diagnosis and therapy, which varied from a few weeks to 3 years. CONCLUSIONS: Structural CNS lesions, responsible for the neurological disorder, were present in 20 patients (7.5%) with cerebrospinal fluid oligoclonal IgG bands. The mechanism underlying oligoclonal IgG presence in spinal arteriovenous malformation and the coexistence of multiple sclerosis and structural CNS lesions is unknown, but may be related to recurrent tissue damage with repeated presentation of CNS antigens to the immune system. ( info) |
3/90. Acquisition and transfer of new verbal information in amnesia: retrieval and neuroanatomical constraints. Four experiments examined new associative learning in amnesia by contrasting the performance of 2 amnesic participants-1 (C.C.) with basal forebrain damage and the other (R.H.) with medial temporal lobe damage--and 3 controls. Both amnesic individuals were severely impaired on explicit memory measures but showed intact perceptual priming. On the new associations measures, only C.C., not R.H., exhibited learning by producing correct targets (HIJACKER) in the absence of perceptual cues for them (e.g., STAFF shot ???). When the perceptual cue (e.g., medicine cured _I_C_P) was provided, both C.C. and R.H. showed learning. Transfer to information containing conceptually related targets (e.g., TERRORIST or BELCH) was reliably observed only in C.C. This finding was replicated with further reduction in perceptual overlap across original (lightning torched JUNGLE) and transfer (lightning burned wilderness) sentences. Together, these findings delineate the role of experimental conditions, severity of amnesia, and different neuroanatomical structures in mediating new verbal learning in amnesia. ( info) |
4/90. Hamartomatous gastric polyposis in a patient with tuberous sclerosis. A 42-year-old female diagnosed with tuberous sclerosis was found to have multiple polyps in the fundus of stomach. On histologic examination, the lesions were hamartomatous polyps. In tuberous sclerosis, many lesions occur in multiple organs and there are several reports about the frequent association of hamartomatous polyps of the colon. However, gastric manifestation of tuberous sclerosis has not been established probably due to its asymptomatic nature. This is the first report of multiple gastric hamartomatous polyposis in patient with tuberous sclerosis. ( info) |
5/90. Spontaneous thrombosis of deep cerebral veins: a complication of arteriovenous malformation. An uncommon type of stroke in children is presented. An intracranial arteriovenous malformation in a 13-year-old boy spontaneously occluded about 22 months after surgical intervention. precipitating factors, such as bacterial infections, could not be demonstrated in this patient, who had been attending school since the time of the craniotomy. The histological features of venous encephalomalacia in the galenic territory are contrasted with hemorrhagic encephalomalacia as seen after arterial occlusions: in the former, hemorrhages are more widespread and edema is more pronounced. ( info) |
6/90. arthrogryposis and multicystic encephalopathy after acute fetal distress in the end stage of gestation. The natural history of the rare association "multicystic encephalopathy-arthrogryposis" was traced in a fetus carefully followed after artificial insemination. The fetus exhibited normal viability and brain morphology up to the 32nd week. At 36 weeks, active movements diminished and at 37 weeks, hydramnios and signs of fetal distress led to cesarean section. The infant presented with severe arthrogryposis of the limbs and spine, but not with the other elements of a long-lasting akinesia. US showed multicystic encephalopathy. Both the clinical and the neuropathological findings established that multicystic encephalopathy was neither the cause nor the sequential consequence of the fetal akinesia, but the result of a recent diffuse, acute malacic process that also involved the anterior horn cells. Acute fetal distress, responsible for major ischemic damage to CNS but compatible with fetal survival, remains an obscure condition which allows for the development of severe arthrogryposis in a few weeks. ( info) |
7/90. Obstructive hydrocephalus caused by intraventricular collapse of malacotic brain. Case report. The authors present the case of a 68-year-old man who experienced acute obstructive hydrocephalus after having suffered an infarction in the occipital lobe. Histopathological and serial neuroimaging examinations revealed that portions of a large disintegrating occipital infarct had entered the lateral ventricle and obstructed the passage of cerebrospinal fluid (CSF). Ventricular drainage was performed for 2 weeks until the patient's hydrocephalus resolved. The CSF initially contained a high concentration of protein (1070 mg/dl), a high leukocyte count of 115 cells/mm3, and a rich fibrinous exudate. Findings in the present case indicate that collapse of a periventricular ischemic lesion into the ventricles may sometimes occur not only after cerebral hemorrhage but also after cerebral infarction. ( info) |
8/90. Subacute necrotizing encephalomyelopathy. Clinical, ultrastructural, biochemical and therapeutic studies in an infant. Subacute necrotizing encephalomyelopathy (SNE) has been observed in an infant with regressing psychomotor development. The concentrations of alanine, pyruvate and lactate were increased in the serum and blood as well as in the cerebrospinal fluid. pyruvate carboxylase activity was reduced in the liver tissue. An inhibitor of thiamine-pyrophosphate-ATP-phosphotransferase was present in the urine. thiamine treatment was followed by a decrease of serum alanine and blood pyruvate and lactate, but there was no clinical improvement during a period of 17 months. Ultrastructural investigations revealed high glycogen levels in liver tissue and skeletal muscle. These findings contrast with decreased gluconeogenesis, which is suggested by the diminished pyruvate carboxylase activity. Therefore it is concluded that reduced hepatic pyruvate carboxylase activity is not the primary cause of SNE. ( info) |
9/90. Multiple cystic and focal encephalomalacia in infancy and childhood with brain stem damage. Two cases are described in which damage to the brain stem was associated with extensive necrosis of the cerebral hemisphere. In the first case--a monochorionic twin--there was clear evidence that injury of an ischaemic or hypoxic type had occurred during fetal life and some evidence that an inadequate share of the placental circulation was an important aetiological factor. In the second case death occurred 4 yr after an asphyxial episode at birth. The lesions in the hemispheres and brain stem were extensive, although less than in the first example. The lesions are discussed in the context of our knowledge of the anatomy and physiology of the developing nervous system. Although they cannot as yet be fitted into the concepts of "critical periods" and "vulnerable periods" of development, this is perhaps because observations on human cases are scanty in comparison with the extensive animal studies which have been reported. The lesions are contrasted and compared with those seen in animals. ( info) |
10/90. diffusion-weighted MR imaging of Carmofur-induced leukoencephalopathy. Carmofur (1-hexylcarbamyl-5-fluorouracil), a derivative of 5-fluorouracil (5-FU), has been widely used in japan as a postoperative adjuvant chemotherapy agent for colorectal and breast cancer. Periventricular hyperintensity on T2-weighted MR images in carmofur-induced leukoencephalopathy confront the physician with a broad range of differential diagnoses. We describe two cases of carmofur-induced leukoencephalopathy in which diffusion-weighted MR imaging revealed periventricular hyperintensity. We compared their findings with those of age-related periventricular hyperintensity in five patients and found discrepancies in signal intensity of periventricular areas. Our results suggest that diffusion-weighted MR imaging may be useful to differentiate carmofur-induced leukoencephalopathy from age-related periventricular hyperintensity. ( info) |