Radial microbrain form of micrencephaly: possible association with carbamazepine.

A premature infant exposed to carbamazepine in utero had a markedly undersized brain on cranial ultrasonogram. Postmortem examination of the brain revealed no evidence of hypoxic-ischemic injury, hemorrhage, infarction, congenital infection, or calcification. The normal cortical gyral pattern, normal residual germinal matrix, and normal cortical lamination suggested the diagnosis of a radial microbrain form of micrencephaly.

Pallidoluysian atrophy: dystonia and basal ganglia functional anatomy.

The clinical correlates of "pure" pallidoluysian atrophy are not well described. A 59-year-old man presented with 20 years of progressive generalized dystonia, dysarthria, gait disorder, supranuclear vertical gaze palsy, and bradykinesia. At autopsy there was severe bilateral atrophy of the external pallidum and subthalamic nucleus with neuronal loss and marked gliosis. This syndrome may epitomize the consequences of "pure" pallidoluysian atrophy. In this case, dystonia appears to occur in the setting of decreased excitation (increased inhibition) of medial pallidal neurons, a pathophysiologic condition common to several hyperkinetic states.

Not 'indifference to pain' but varieties of hereditary sensory and autonomic neuropathy.

Three children, from different kinships, with generalized insensitivity to pain, showed unusual manifestations of congenital, presumably inherited, sensory and autonomic neuropathy. The first child appeared to have a syndrome resembling those previously described as congenital indifference to pain, congenital universal loss of pain sensation from infancy without other apparent neurological deficit. Unlike most types of hereditary sensory and autonomic neuropathies (types I, II, III), but like type IV, she had normal sensory nerve action potentials. Abnormalities of sudomotor function and of somatosensory evoked potentials were demonstrated. A severe decrease in the number of sural nerve A delta fibres and a small reduction in C fibres were demonstrated morphometrically. An abnormality of C fibres was confirmed by a marked reduction in nerve dopamine-beta-hydroxylase activity. The plasma and CSF concentrations of beta endorphins, substance P and several other neuropeptides and hormones were normal. Unequivocal evidence of a neuropathic lesion is provided by this patient; her disorder may be identified as the fifth type of hereditary sensory and autonomic neuropathy. The second patient had a congenital pansensory neuropathy and progressive retinitis pigmentosa. Whether the disorder is inherited and, if so, whether the retinitis pigmentosa results from the same or from a second genetic abnormality, is unclear. The third case has, in addition to what is usually seen in hereditary sensory and autonomic neuropathy, type II, an unusually severe kinaesthetic difficulty in oral food handling. The sural nerves of the second and third patients had fibre composition characteristic of hereditary sensory and autonomic neuropathy, type II, few or no myelinated fibres and reduced numbers of unmyelinated fibres.

Contamination of Pantopaque by glass.

Investigation has shown that when ampules containing Pantopaque are opened, a variable amount of fragments of glass may fall into the Pantopaque. When the Pantopaque is injected into the spinal canal these fragments may also be injected. As yet the results of injecting fragments of glass into the spinal subarachnoid space are unknown. The glass can be removed by passing the Pantopaque through a 0.22 micron Millex Filter.

Clinicopathologic studies of primary cerebral amyloid angiopathy.

Primary cerebrovascular amyloidosis resulting in significant cerebral parenchymal damage was encountered in 23 autopsied cases at the Mayo Clinic over the past 10 years. Patients were 60 to 97 years old and both sexes were equally represented. Large- and medium-sized leptomeningeal and cortical arteries showed the characteristic pattern of medial and intimal involvement, with luminal stenosis. The walls of smaller arteries were often diffusely infiltrated, with fibrinoid degeneration and miliary aneurysm formation. The amyloid nature of the infiltrate was confirmed by electron microscopic examination in all cases. All cases showed varying numbers of perivascular or independent senile plaques in the cerebral cortex. Alzheimer's neurofibrillary tangles were absent or were limited to the hippocampal region in all but two cases. Multiple, small cortical infarcts and hemorrhages were regularly present. Larger hemorrhage was present in nine cases. Of nine patients with terminal massive cerebral hemorrhage, only two were hypertensive. Six patients had had progressive dementia; four had had single episodes of vascular events and seven, multiple episodes; and four had had both dementia and vascular episodes. Primary cerebral amyloid angiopathy should be regarded as an important cause of mental deterioration and fatal cerebrovascular accidents in the elderly.

Effects of anticoagulants in an animal model of septic cerebral embolization.

The effect of anticoagulation on lesions caused by cerebral emboli of different types was studied in 57 dogs. The resultant arterial and parenchymal lesions were assessed by pathologic and angiographic studies. Embolization with emboli that caused little or no inflammatory response in the artery (12 dogs) was not associated with hemorrhagic infarcts or with subdural or subarachnoid hemorrhage; furthermore, treatment with anticoagulants (9 dogs) did not change the character of the lesions. Embolization with emboli that caused arteritis, that is, bacterial contamination or presence of lead chromate in the embolus (21 dogs), was associated with hemorrhagic infarcts, focal subarachnoid hemorrhage, and increased incidence of acute subdural hemorrhage. Treatment with anticoagulants (16 dogs) was associated with a further increase in the incidence of subdural hemorrhage.

Case report. Computer assisted tomography in syringomyelia.

A case of cervicothoracic syringomyelia shown on computer assisted tomography is presented. Surgical verification was obtained, and the extent of the cord cavitation was also demonstrated.

Pineal tumors. A 53-year experience.

Thirty-four patients with pathologically confirmed primary pineal tumors were evaluated at the Mayo Clinic from 1923 through 1976. All tumors were of germ cell or pineal cell origin. Most patients were adolescent boys with subacute increased intracranial pressure and Parinaud's syndrome. Hypothalamic symptoms were surprisingly infrequent. Direct surgical intervention was associated with high mortality, but has been more successful in recent years.

Cerebral arterial lesions resulting from inflammatory emboli.

In order to study the effects of septic embolism on the brain, silicone rubber emboli of various types were injected into the carotid arteries of 35 dogs. Pathologic and angiographic studies were performed to assess the resultant arterial and parenchymal lesions. Pure silicone rubber emboli (14 dogs) produced occasional intra-arterial thrombosis but no arteritis. Sterile and bacterially contaminated emboli containing a lead-chromate pigment (similar to those used in previous studies of septic embolism) (11 dogs) and pure silicone rubber emboli with transversely oriented canals (10 dogs), after brief placement in a bacterial suspension, were associated with intense inflammatory arteritis. This was accompanied by focal meningitis, subarachnoid hemorrhage, thrombosis, and cerebritis of the underlying cortex. The findings resembled those found in mycotic aneurysm. Aneurysmal dilatation was observed in one postmortem angiogram. In previous models of mycotic aneurysm, the inflammation attributed to bacterial contamination was probably due to the lead-chromate pigment used.

Intraventricular hemorrhages in full-term and premature infants.

Intraventricular hemorrhages were the most common neuropathologic findings in both full-term and premature infants in a retrospective study of neonatal deaths. In general, the intraventricular hemorrhages of full-term infants were less frequent, originated from veins of the choroid plexus, were small, and rarely caused death. Intraventricular hemorrhages of premature infants usually originated in the germinal matrix and were large enough to be fatal. Intraventricular hemorrhages were most frequently found when death occurred between 24 and 72 hours of age.

Combined nuclear and supranuclear defects in ocular motility. A clinicopathologic study.

A 65-year-old man suffered a midbrain infarct after coronary artery bypass surgery. He was left with a severe neuro-ophthalmologic deficit consisting of paralysis of upward and downward vertical gaze, weakness of adduction of the left eye, a dilated fixed left pupil, and partial right Horner's syndrome. He died 31 months after the episode. Postmortem examination disclosed an infarct involving parts of both oculomotor nuclei as well as supranuclear structures thought to be involved in the mediation of vertical eye movements.

Sudden death from stroke.

Sudden death is defined as any death that occurs less than 24 hours after the onset of first symptoms. Strokes account for 10 to 20% of all sudden deaths. The records of all residents of Rochester, Minn., who had their first stroke during the period 1955 through 1969 were analyzed. Among 255 deaths caused by the first stroke, 52 were sudden. Twenty-six of the deaths were due to primary intracerebral hemorrhage, and 20 to primary subarachnoid hemorrhage. Only two of the sudden deaths were caused by infarction: one by pontine and cerebellar infarct and the second by a cortical infarct, which resulted in death from status epilepticus. Among the nine patients who died within 2 hours of the onset of symptoms, six had primary subarachnoid hemorrhage. Hypertension was noted in 23 of the 26 patients (88%) who died of primary intracerebral hemorrhage; 8 patients with primary intracerebral hemorrhage were on long-term oral anticoagulant therapy, and all 8 were hypertensive.

Epilepsia partialis continua. A review of 32 cases.

Clinical, EEG, and autopsy findings were studied in 32 patients with epilepsia partialis continua. The seizures occurred at different ages and with diverse diseases of the brain. Facial and distal limb muscles were preferentially involved. Often, seizures suggested multifocal origin within a limited cerebral region, with low-amplitude, irregular, asynchronous contractions that could resemble other movement disorders. Twitching varied in rate, rhythm, intensity, and territorial extent continued sometimes for decades and was poorly responsive to therapy. Focal EEG abnormalities commonly consisted of discrete spikes, sharp waves, or slow-wave activity. Autopsy findings in eight patients showed consistent involvement of the motor cortex or closely adjacent areas. Clinical, EEG, and pathologic data favor a cortical origin of epilepsia partialis continua.

Acute cerebral dysfunction in diabetic ketoacidosis: survivial followed by panhypopituitarism.

We report a unique case of a 15-yr-old diabetic patient who survived ketoacidosis complicated by acute severe encephalopathy, hypopituitarism, and optic atropy. We reviewed our 25-yr experience with fatal diabetic ketoacidosis; three additional cases resembled the "cerebral edema" syndrome. We observed that (1) cerebral edema is not often documented in this syndrome, (2) the cause of the acute neuronal disturbance in these patients may be hypoxic, with cerebral edema a secondary development, and (3) development of encephalopathy was probably unrelated to overvigorour correction of acidosis and hyperglycemia in our cases.

Controlled study of CCNU and radiation therapy in malignant astrocytoma.

The authors report 63 patients with biopsy-proved malignant (Grades 3 and 4) astrocytomas who were randomly placed in one of three treatment schedules within 2 weeks of surgery. One group (22 patients) received radiation therapy alone; the second group (22 patients) received 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU) orally at intervals of 8 weeks; and the third group (19 patients) received combined radiation and drug therapy. Patients who received radiation therapy, with or without the drug, had a significantly longer survival than did those who received the drug alone. There was no difference in survival between the two groups who received radiation. The nitrosourea derivative CCNU does not seem to be an effective agent in the therapy of primary malignant brain tumors.

Alpha-coma. Electroencephalographic, clinical, pathologic, and etiologic correlations.

"Alpha-coma" denotes the conjunction of clinical coma with an electroencephalographic pattern resembling that of normal wakefulness and predominantly consisting of alpha activity. Clinical, EEG, and pathologic data from 13 patients with this syndrome were reviewed. The patients were divided into two groups, based on the pathogenesis of their conditions. The first group consisted of eight patients with brain stem strokes, and the second group consisted of five patients with diffuse hypoxic encephalopathy resulting from cardiac or pulmonary arrest. There were some differences between the EEGs of the two groups. In the first group, the alpha pattern was located more posteriorly, showed more variability and reactivity, and was more persistent in sequential recordings. In the second group, the alpha activity was transient and showed little reactivity. In both groups, this pattern indicated a poor prognosis for survival.

A distinctive clinical EEG profile in herpes simplex encephalitis.

Two patients with herpes simplex encephalitis had clinical courses consisting of an acute febrile illness with early onset of seizures and rapidly progressive neurologic signs evolving to coma and death within 3 weeks. The electroencephalograms of both patients were similar and showed widespread, periodic, stereotyped sharp-and-slow-wave complexes occurring bilaterally over both hemispheres, as well as transient episodes of electrographic seizure activity occurring unilaterally, with suppression of the periodic activity on that side. Although the electroencephalographic findings are not pathognomonic, when associated with an acute encephalitic process, they would suggest the diagnosis of herpes simplex encephalitis.

The familial occurrence of glioma.

In a family with four children, an intracranial neoplasm developed in three of the siblings at or before the age of 12 years. Two of the tumors were hitologically verified gliomas and the third was diagnosed on ventriculography but did not have microscopic confirmation. One of the three siblings also had a presacral lipoma, and the fourth sibling developed a mediastinal cystic hydroma. There was no known history of brain tumors in any other family members, nor was there any evidence suggestive of neurofibromatosis. A careful examination of this family and an extensive review of other published cases of similar familial constellations of brain tumors reveal no definite pattern which would suggest a specific mode of inheritance. Careful documentation of familial aggregation of brain tumors is important in order to explore the genetic, environmental, demographic, and clinical features (such as associated extracranial tumors) that could serve to identify groups at high risk for the familial occurrence of brain tumors.