Lateral inferior pontine syndrome. Clinico-pathologic study and review of literature

A patient with left-sided deafness, left facial paralysis, sensory loss in the left side of the face and cerebellar signs of acute onset is presented. At necropsy, a circumscribed hemorrhage in the caudal pontine tegmentum and adjacent cerebellum was found. The clinical and pathological findings were those of the lateral inferior pontine syndrome. The pertinent literature is briefly reviewed and the use of topographical nomenclature is emphasized.     

Pure motor hemiplegia with occlusion of the extracranial carotid artery

Two patients with the syndrome of pure motor hemiplegia are presented in whom occlusion of the carotid artery in the neck was demonstrated angiographically. A relationship between extracranial vascular disease and pure motor hemiplegia is suggested and potential implications in the management and treatment of such patients are discussed.     

Dementia and low-pressure hydrocephalus in a patient with pituitary adenoma

A patient is presented in whom global dementia was the initial clinical manifestation of pituitary adenoma in the absence of visual or endocrine symptoms or signs of increased intracranial pressure. The tumor had partially obstructed the flow of cerebrospinal fluid (CSF) resulting in low-pressure hydrocephalus, manifesting as dementia, urinary incontinence and a characteristic motor disorder found on clinical examination. This is the first reported instance, to our knowledge, of pituitary adenoma causing low-pressure hydrocephalus. It is postulated that transventricular absorption of CSF accounted for the normalization of the patient's intraventricular pressure. Clinical and experimental evidence in support of this contention is briefly reviewed.     

Quantitative studies of dorsal root ganglia and neuropathologic observations on spinal cords in familial dysautonomia

Intrauterine development and postnatal maintenance of dorsal root ganglion neurons are abnormal in familial dysautonomia, an autosomal recessive disorder associated with autonomic, motor and sensory deficits. Normally, dorsal root ganglion weight increases with age. This does not occur in the cervical plexus ganglia of dysautonomic patients. Neurons in dorsal root ganglia are found to be markedly diminished in the youngest patients and slow degeneration causes further depletion with age. Quantitative studies on C8 dorsal root ganglia show the normal neuron content to be between 42,500 and 53,600. In 3 patients with familial dysautonomia the range was 4,090-8,590 with the smallest number being in the oldest patient. Lateral root entry zones and Lissauer's tracts are severely depleted of axons. In older patients loss of dorsal column myelinated axons becomes evident and is first seen in lumbar fasciculus gracilis, cervical fasciculus cuneatus and interfascicular fasciculus. Temperature sensation is markedly impaired from infancy in familial dysautonomia. Loss of pain sensation is prominent and worsens with age. Vibration sense diminishes in adolescence and coordination of limb movements becomes poor in older patients. Neuron depletion in dorsal root ganglia and the progressive pattern of cord changes correlate well with these clinical observations.     

Saturday night retinopathy

A case of unilateral ischemic retinopathy leading to permanent blindness in association with unilateral peroneal palsy involving the same side is described. The retinopathy and neuropathy occurred in a setting of heavy alcohol consumption followed by stupor; hence the use of the term “Saturday Night Retinopathy” appears appropriate. The similarity of this syndrome to that observed during face mask anesthesia or anesthesia during neurosurgical procedures utilizing a head rest and inadvertently producing increased orbital pressure, is striking. The spontaneous occurrence of this syndrome outside a hospital setting is unusual. The presence of massive retinal edema indicative of retinal anoxia and the absent ERG signal suggest partial or complete collapse of the ophthalmic artery secondary to orbital pressure as the major underlying mechanism of the retinal ischemia.     

Suppression of acute and chronic experimental allergic encephalomyelitis in strain 13 guinea pigs: A clinical and pathological study

Adult inbred Strain 13 guinea pigs develop an acute, fatal form of experimental allergic encephalomyelitis (EAE) about 2 weeks after a single injection of isologous spinal cord in complete Freund's adjuvant (CFA), but similarly injected juveniles develop a delayed, rarely fatal chronic form. Thirty-seven sensitised adult Strain 13 animals were separated into 2 groups. One group was permitted to develop acute EAE. The other group was injected intramuscularly with 1 mg of guinea pig or bovine myelin basic protein (MBP) in incomplete Freund's adjuvant (IFA) on day 2, 7 or 10 post-inoculation (PI) followed by 0.2 mg in IFA every third day for a total of 10 doses. Animals in the unsuppressed group succumbed to acute EAE 13–16 days post-sensitisation. No animal in the suppressed group died during this period. Animals treated with MBP beginning 2 days PI showed no clinical signs, but mild clinical manifestations occurred in animals suppressed from days 7 and 10 PI. These signs remitted by 21 days post-sensitisation. One suppressed animal (out of 21) died during the fourth week postsensitisation. The other 20 suppressed animals appeared clinically normal towards the end of the course of MBP injections and remained so for the 6 months of study. Morphological examination revealed that CNS lesions occurred in all animals. In animals suppressed with MBP beginning on day 2 PI, lesions consisted only of a few meningeal inflammatory cells. Animals given MBP beginning on day 7 or 10 PI and sampled 1–2 weeks later, had lesions which could not be distinguished from those occurring in the non-suppressed acute EAE group. In time, the suppressed animals developed lesions which were typical of chronic EAE with remyelination as a predominant feature. Preliminary experiments on the suppression of chronic EAE in 5 juvenile Strain 13 guinea pigs have revealed that 3 MBP-injected animals failed to develop clinical disease over a 28-week period of study although lesions typical of chronic EAE were present. Simultaneously, 2 non-suppressed juvenile animals developed clinical signs by 12 weeks. These were associated with both acute inflammation and demyelination superimposed upon regions of chronic demyelinative activity.     

Ultrastructural characteristics of the brain and blood-brain barrier in experimental seizures

During experimental seizures, the blood-brain barrier (BBB) is broken; tracer substances such as I¹³¹-albumin, Evans blue and horseradish peroxidase (HRP) geographically locate the barrier breakdown primarily in the diencephalon. Using rats, we have induced seizures with electroshocks and demonstrated the breakdown of the BBB with Evans blue and HRP. We have shown that (1) the BBB breakdown is proportional to the number of electroconvulsant shocks (ES) given; (2) the mechanism of increased barrier permeability is primarily by micropinocytosis in the cerebral capillaries, arterioles, and, to a lesser extent, venules; and (3) the stimulus for micropinocytosis and hence BBB breakdown is associated with the abrupt rise in systemic blood pressure and cerebral vasodilatation that accompanies each ES. If the systolic hypertension is abolished via cervical cordotomy, there is little to no breakdown in the BBB.     

Sex differences in dopaminergic and cholinergic activity and function in the nigro-striatal system of the rat

Possible sex differences in the balance between dopaminergic and cholinergic activity in the rat striatum were investigated. Female rat show a greater vulnerability to neuroleptic-induced catalepsy compared to male rats. This vulnerability to neuroleptics has a human counterpart in that women show an increased frequency of neuroleptic-induced extrapyramidal syndrome (EPS) compared to men. In humans, EPS can be alleviated by increasing dopaminergic activity or by decreasing cholinergic activity. Conversely, EPS can be precipitated by decreasing dopaminergic activity with neuroleptics, and pre-existing EPS can be made worse with cholinergic agonists. Thus, the presence of EPS in humans or catalepsy in animals, as a result of neuroleptic agents, appears to be related to low dopaminergic and/or high cholinergic activity. In the present studies of sex differences it was found that: (1) cycling female rats had fewer striatal dopamine receptors than did male rats. Estradiol treatment significantly reduced the number in males and also reduced the number, although not significantly in ovariectomized (OVX) rats; (2) cycling female rats had lower apomorphine-induced stereotypy during phases with high estrogen levels as compared to phases with low estrogen levels. Estrogen treatment of male or OVX rats resulted in attenuation of induced stereotypy compared to untreated male or OVX rats; (3) female rats had a significantly higher affinity of striatal cholinergic receptors of 3H-QNB than did males and also had higher choline acetyltransfease activity than male rats. The data from these experiments provide the first direct evidence that estrogen plays a role in shifting the balance toward cholinergic predominance in the striatum, a state that favors the development of cataleposy in female rats and EPS in women.     

Unit activity of limbic system neurons: Effects of morphine, diazepam and neuroleptic agents

The effects of morphine, diazepam and three neuroleptic agents (chloropromazine, perphenazine and haloperiodol) on neuronal firing rats were studied in the limbic system of immobilized cats. Parietal craniotomy was carried out under 1.5--4.0% halothane. Extracellular potentials from single cells in the cingulate gyrus, septum and lateral hypothalamic areas were recorded using glass-coated, platinum-iridium microelectrodes. In general, intravenous adminstration of morphine sulfate augmented the spontaneous firing rates of most of the neurons studied. In contrast, diazepam produced a marked attenuation of both spontaneous and morphine augmented firing rates, whereas the neuroleptic agents had no significant or consistent effects on the morphine augmented firing rates of neurons in these limbic areas. These data indicate that the limbic system may play an important role in the behavioral excitement in cats induced by morphine administration and also the depressant effect of the tranquilizer diazepam. In contrast, the inability of the neuroleptic agents to antagonize the morphine augmented neuronal firing rates suggest these agents may act outside the limbic areas studied here.     

Effeminate behavior present in boys from childhood: Ten additional years of follow-up

This is a 10-year further follow-up of 16 boys with early effeminate behavior, a group of cases first reported in 1966. The average age at this follow-up was 22 years, and the average number of years elapsed since first seen was 14 years. Twelve of the 16 cases developed some form of deviant behavior—homosexuality in 10, transvestism in 1, and transsexualism in 1. Two of the cases were heterosexually oriented, and in the remaining two, no decision could be reached as to their sexual orientation.     

Efficacy and safety of induced seizures (EST) in man

A review of the EST process finds its efficacy and safety in the treatment of psychotic depressive states and mania to be well-documented. In acute schizophrenia, EST is symptomatic and its usefulness is equivalent to psychotropic drugs. In chronic schizophrenia, the effects of EST and other therapies are equally poor; but the principal studies are faulted by inadequate numbers of EST treatments. In all other conditions, including the use in children and adolescents, the data are insecure and additional studies are needed.The principal risks of EST have been reduced by improved treatment methods. Fracture, panic, spontaneous seizures, and death are no longer prominent. Persistent deficits in memory and the psychologic stigma of having had “shock therapy” are the principal costs of therapy today. Suggestions to reduce these risks are made.For depression, mania, and acute schizophrenia, EST has a risk/benefit ratio equal to or better than other available treatments. Further study of the relative efficacy and safety of these treatments are needed; as are controlled studies for other conditions where its use has been recommended.     

Laterality effects in response to offsets of tonal stimuli

In two experiments employing quite different procedures it was found that the offsets of tones presented in the right ear elicit significantly later responses than do the offsets of tones presented in the left ear. However, no ear differences were observed for responses to the onsets of tones in either experiments, attentional explanations of the results seem unlikely. The results may reflect an echoic memory for right-ear stimuli which is of longer duration than that for left-ear stimuli.     

Life events, psychological distress and the Type A coronary-prone behavior pattern

The relationship between life events and psychological distress was examined in Type A and Type B coronary-prone college students. The results indicated that Type A's report the occurrence of more life events than Type B's. In addition, examination of the perceived characteristics of the life events revealed different patterns for A's vs B's. Life events seen as undesirable, unexpected, and ambiguous in terms of perceived control were positively correlated with increased distress for Type A's. Type B's, on the other hand, were less distressed the more the event was perceived to be beyond their control. The results are discussed in terms of the differences in the Type A/B personality styles.     

Chronic relapsing experimental allergic encephalomyelitis. Correlation of circulating lymphocyte fluctuations with disease activity in suppressed and unsuppressed animals.

Groups of juvenile Strain 13 guinea pigs sensitized for chronic relapsing experimental allergic encephalomyelitis (EAE) with isogeneic central nervous system (CNS) tissue in complete Freund's adjuvant (CFA) were either left to develop late-onset chronic EAE (unsuppressed), or given a series of injections of bovine myelin basic protein (MBP) in incomplete Freund's adjuvant (IFA) to suppress the disease. All unsuppressed animals developed disease and all suppressed animals remained healthy over a 27-month period of study. some unsuppressed and suppressed animals were rechallenged with CNS tissue in CFA 12 or 26 months post-inoculation (PI). Unsuppressed animals all became sick 2-4 weeks after rechallenge, while rechallenged, suppressed animals were protected, indicating that the suppression was permanent. Pathologic findings in the CNS complemented the clinical changes. Circulating lymphocyte studies were performed on animals from all groups. Early (active, high-affinity rosetting) T cell levels in unsuppressed animals showed significant decreases during exacerbations (P less than 0.01) and normal values during remissions. After rechallenge, circulating early T cells decreased in unsuppressed animals with the development of signs. In suppressed animals, early T cells showed significant elevations during, and for a short time after, the period of suppressive injections, and normal values afterwards. These levels did not change significantly after rechallenge. Late (total, 24 hour rosetting) T cell and B cell values showed minor fluctuations only which did not correlate with disease activity. These results indicate that chronic relapsing EAE can be successfully suppressed with MBP in IFA, that this suppression is permanent and that the immunologic findings presented correlate well with the clinical and pathologic facets of the disease. the findings are presented in terms of their relevance to multiple sclerosis.     

Deficits in retrieval of category exemplars in alcoholic korsakoff patients

Reports that Korsakoff patients generate category exemplars with normal efficiency in a 30 sec period would seem to be inconsistent with the operation of a generalized retrieval factor in the Korsakoff amnesia. A replication of such studies was conducted extending the number of categories sampled to six, the time period to 5 min, and adding a written condition. Results indicate that Korsakoffs generate exemplars at a slower rate, even in the first 30 sec, and terminate production earlier than normal controls. Both groups demonstrate the same retrieval strategy, generating exemplars of decreasing “popularity”. The possible mechanism underlying the slower rate and early termination are discussed.     

Biochemical abnormalities of muscle ribosomes during attacks of hyperkalemic periodic paralysis

Three familial cases (a mother and 2 sons) with hyperkalemic periodic paralysis are presented. Myotonia (orbicularis oculi and hands) was noted all 3 cases. Exposure to cold produced weakness in the 2 older patients. An oral KCl loading test induced paroxysmal weakness in all 3 cases. Ribosomes isolated in 2 cases from biopsied muscle during the induced attacks showed definite increases of protein synthesis in a group of rather heavy muscle polyribosomes. Electron microscopy revealed in both cases scattered small membrane-bound vacuoles, subsarcolemmal aggregates of myotubules, glycogen particles and occasional filamentous bodies.     

Clinical effects of amphetamine and l-DOPA on sexuality and aggression

The effects on sexuality and aggresion noted in a group of hospitalized amphetamine-abusers are presented. Intensified sexual feelings were most frequently noted, but were not a consistent finding in all cases. The same inconsistently increased sexual tension was directly observed in a subgroup of these individuals to whom amphetamine was administered on a research ward. Instances of assaultive behavior are described.Cases of hypersexuality and irritability noted in psychiatric patients to whom l-DOPA was administered are also presented. These observations suggest that baseline status is an important determinant of these effects in the cases in which they are noted.     

Allergic potential and emotional precipitants of asthma in children.

The relationship between certain psychological and allergic variables in childhood asthma was studied. The prediction was that across patients of equal illness severity, an inverse relationship would be observed between measures of the involvement of emotional symptom precipitants and of allergic factors. This relationship failed to emerge at a statistically significant level when the index of emotional precipitants included only such things as worry, anger, upset, excitement, etc. and excluded emotionally toned “respiratory behaviors” such as crying or laughing. When crying was included in the index of emotional precipitants, the prediction was clearly supported. These findings were discussed in terms of their bearing on what is meant by the involvement of psychological factors in asthma, and a broader conceptual scheme was suggested. A clinical implication of the findings and certain methodological considerations was also discussed.