Severe anterograde amnesia with onset in childhood as a result of anoxic encephalopathy.

Our patient (M.S.) had an abrupt onset of amnesia due to a respiratory arrest at the age of 8 years and has been followed by one of us (A.L.R.) for 19 years. A specially designed MRI study indicated that the neuroanatomical localization of his lesion is restricted to the hippocampal formation bilaterally. Comparison of M.S.'s present IQ and academic scores with earlier scores revealed that his literacy skills, certain basic language functions and vocabulary development were arrested by his memory disorder. In contrast, development of mathematical skill was less curtailed, and verbal and nonverbal logical abilities developed to adult levels. Neuropsychological examination at the age of 27 years elicited a pattern of memory deficits similar to those found in a case (H.M.) of known mesial temporal lobe damage in adulthood. The neuropsychological pattern revealed those aspects of cognitive development that do, and those that do not, require intact memory. The limitations to intellectual development imposed by severe amnesia in childhood are not pervasive, but rather, are limited to specific types of abilities.

A new method using neuromagnetic stimulation to measure conduction time within the cauda equina.

Using principles derived from electric field measurements and studies of phrenic nerve in vitro, neuromagnetic stimuli in humans were predicted to excite selective low threshold sites in proximal and distal cauda equina. Physical models, in which induced electric fields were recorded in a segment of human lumbosacral spine immersed in a saline filled tank, supported this prediction. Conclusions from the model were tested and confirmed in normal human subjects. Ipsilateral motor evoked potentials were elicited in lower limb muscles and striated sphincters by magnetic coil (MC) stimulation of both proximal and distal cauda equina. Over proximal cauda equina a vertically oriented MC junction and cranially directed induced current elicited a newly identified compound muscle action potential (CMAP). The F response latency and lack of attenuation when the target muscle was vibrated suggest that the proximal response is a directly elicited M response arising near or at the rootlet exit zone of the conus medullaris. Over distal cauda equina, lumbar roots were optimally excited by a horizontally oriented MC junction, and sacral roots by an approximately vertically oriented MC junction, eliciting CMAPs with similar appearance but shorter latency consistent with the known intrathecal lengths of the lower lumbar and sacral nerve roots. The induced current was usually most effective when directed towards the spinal fluid filled thecal sac. Normal subjects showed stable CMAP onset latencies elicited at proximal and distal cauda equina despite wide variation in amplitude. Thus, cauda equina conduction time can be directly calculated. This new method may improve the detection and classification of peripheral neuropathies affecting lower limbs and striated sphincters.

Reversible increased technetium-99m-HMPAO cerebral cortical activity: a scintigraphic reflection of luxuriant hyperperfusion.

A hemiparetic and aphasic patient, 3 days after acute traumatic transection of the left internal carotid artery requiring life-saving total embolic occlusion, revealed ipsilateral increased peripheral hemispheric 99mTc-HMPAO activity. Ten days postocclusion, HMPAO peripheral cortical flow normalized as hemiparesis and aphasia significantly cleared. The initial lateralized HMPAO hyperactivity pattern may reflect reactive hyperemia, a sign previously identified by contrast angiography and often associated with a better prognosis in evolving CVA. Evanescent peripheral cerebral hyperemia may represent beneficial cortical collateralization of the periinfarct area of a deeper lacunar (white matter) CVA.

Aplasia cutis congenita and arteriovenous fistula. Case report and review.

We describe a child with congenital aplasia cutis congenita of the scalp and an occult giant posterior fossa arteriovenous fistula. Previous case reports of central nervous system malformations associated with aplasia cutis congenita are reviewed. The exact incidence of such malformations is unknown. All patients with aplasia cutis congenita should undergo a neurologic evaluation, and their families should be examined for similar lesions. Early central nervous system imaging and other workup may be required, especially if plastic surgery in the head region is being planned.

Vertebrobasilar occlusion complicating nonpenetrating craniocervical trauma in a child.

We report a juvenile patient who developed vertebrobasilar occlusion following nonpenetrating head and neck trauma, with complete recovery. The patient presented with transient signs of brain-stem dysfunction that were secondary to embolization and/or extension of a thrombus. He was treated with anticoagulants. We have found no other reports of such treatment in juvenile vertebrobasilar occlusion that complicated nonpenetrating head and neck trauma.

Imaging evaluation of children after falls from a height: review of 45 cases.

The injury pattern in the child who falls from a height is markedly different from that in the adult, so a different imaging protocol is needed. To help establish such a protocol, the authors reviewed the charts and imaging records of 45 children and infants 12 years of age and younger who had fallen one to six stories. Extremity fractures were the most common injury, occurring in 20 patients. Head injuries (including skull fractures) occurred in 19 patients. Abdominal injuries were present in only one patient. Pneumothoraces were seen in four patients and lung contusions in two patients. Fractures of the pelvis, spine, and os calcis were uncommon. The height of the fall did not enable prediction of either the severity or type of injury. The authors recommend radiography of the cervical spine and chest alone in the initial evaluation of the injured child and greater willingness to obtain computed tomographic (CT) scans of the head in children than in adults. Radiographs of the pelvis, thoracolumbar spine, and lower extremities, as well as CT scans of the head and abdomen, should be obtained on a case-by-case basis, and not according to protocol, as is often the situation in adults.

Pontine calcification following radiotherapy: CT demonstration.

Development of pontine calcifications following radiation therapy for suprasellar tumors is described in two patients, 5 and 9 years old. Post-radiotherapy brain calcifications are rare in the brain stem.

Pediatric AIDS. Neuroradiologic and neurodevelopmental findings.

A group of 23 pediatric patients seropositive for HIV antibody were studied by computed tomography and evaluated neurodevelopmentally. Significant neurodevelopmental delays were found in over 95% of the patients studied. CT findings in six patients were normal and thirteen of 23 (57%) had prominence of the CSF spaces. Less frequent findings included calcifications in the basal ganglia and white matter. Cerebral mass lesions included one case of lymphoma and one case of hemorrhage. The CT findings in the pediatric age group differs from the adult population in that contrast enhancing inflammatory mass lesions are uncommon.

CNS toxoplasmosis in acquired immune deficiency syndrome: a clinical-pathological-radiological review of 12 cases.

From January 1981 to January 1983 acquired immune deficiency syndrome (AIDS) was diagnosed in 90 patients admitted to Kings County Hospital-Downstate Medical Center. CNS involvement occurred in 18 patients of whom 12 had toxoplasmosis confirmed by biopsy or necropsy. Pathological specimens from these 12 patients were notable for a marked diminution or absence of cellular inflammation. Each patient had elevated serological studies for toxoplasma. AIDS presented with symptoms referable to CNS toxoplasma in eight patients. In the remaining four patients, toxoplasma was found late in the course of the illness. CT showed either ring enhancing lesions or solid nodules. The course was uniformly fatal, though patients treated continuously with pyrimethamine and sulfadiazine survived longer.

The management of arterial injuries caused by penetration of zone III of the neck.

Penetrating trauma of the neck has been divided into three anatomic locations. Zone III, the subject of this paper, is defined as the area between the base of the skull and the lower border of the mandible. Management of these injuries remains problematic. Clinical assessment may be misleading, exploration may damage surrounding neurovascular structures, and injuries may go undetected. This has led us and others to advocate mandatory angiography before any surgical exploration. This report reviews 46 patients with Zone III injuries with respect to types of injuries, therapy and outcome. Angiography was normal in 22 patients who were treated conservatively with no complications. The remaining 24 patients sustained 39 arterial injuries diagnosed by contrast studies. Eighteen internal carotid injuries were identified in 16 patients. At operation ligation was performed in four patients and revascularization in two patients. One of the repairs subsequently thrombosed. Ten patients were managed nonoperatively by observation (seven patients) or angiographic embolization (three patients). Catheter embolization of the external carotid or its branches was performed to control bleeding (eight vessels) or close arteriovenous fistulas (two patients). Seven nonbleeding external vessels were successfully managed by observation. Two vertebral artery injuries were diagnosed. One required proximal embolization and distal ligation via occipital craniectomy to control a fistula between the vertebral artery and the jugular vein. Overall mortality was 8.6%. Three of the four deaths were in patients with neurologic deficit on admission. Another died of respiratory arrest. An air embolism resulting in hemiparesis was the only complication of the angiographic studies. We conclude that angiography is essential in Zone III neck wounds. It facilitates triage decisions and, combined with transcatheter embolization, enables the majority of these injuries to be managed without surgical exploration.

Empty sella turcica in intracranial sarcoidosis. Pituitary insufficiency, primary polydipsia, and changing neuroradiologic findings.

A 37-year-old man with visual loss was found to have hypopituitarism and primary polydipsia associated with sarcoidosis. Neuroradiologic studies demonstrated a dramatic evolution of CNS lesions, including a left thalamic infarct, an enhancing suprasellar mass, and ultimately an empty sella turcica. The patient has been clinically stable in spite of these changes. This case is likely to be the first reported of CNS sarcoidosis with an empty sella turcica documented by computed tomography.