Ten patients have been described showing inability to stop breathing on command, spontaneous respiration and voluntary respiratory stimulation being unaffected. This abnormality not previously described in the literature, we feel should be named respiratory inhibitory apraxia (R.I.A.). The anatomical organization of respiration is briefly reviewed. R.I.A. is often associated with other forms of apraxia or motor impersistence. It is thought that the urinary and bowel incontinence present in some of these cases might also represent a form of inhibitory apraxia. Information is presented which supports the view that respiratory inhibitory apraxia is due to a minor hemisphere lesion, usually deepseated. Our one autopsied case showed a lesion in the descending motor pathways in the internal capsule, in middle cerebral artery branch territory, disconnecting the voluntary respiratory inhibitory center in the cortex in anterior and middle cerebral cortical branch territories.

A computerized analysis of 128 patients admitted with acute head injury and who underwent angiography is shown. Patients were divided into groups according to: age, sex, type of accident, state of consciousness and presence of localizing signs on admission, types of cerebral lesions on angiography, and discharge condition.

There is a preponderance of young males in this series of patients, related mainly to MVA. A total of 71% of the patients had abnormal angiograms, but the incidence of normal and abnormal results did not correlate significantly with any of the chosen parameters.

The same parameters were also analysed to assess their value as a prognostic index for the patient. The conclusion was drawn that the angiogram per se has no significant value as a prognostic tool, and that state of consciousness on admission is the best single index for prognosis.

The clinical features of the Chiari Malformation in seven adult patients are presented. It is suggested that the clinical syndromes associated with this malformation, in adults, can be classified as (a) compression of structures at the level of the foramen magnum (with or without radiologically demonstrable associated bony anomaly at the cranio-vertebral junction) (b) increased intracranial pressure or obstructive hydrocephalus and (c) intramedullary cervical cord syndrome. The usefulness of tomography, and demonstration of the vertebro-basilar circulation in the neuro-radiologic investigation of these patients is emphasized. The surgical procedures performed in the management of these patients are outlined.

Median eminence and ventromedial hypothalamus have in the past been the principal foci of research in neuroendocrine and neurovisceral control mechanisms. The present report provides an overview of work involving the dorsomedial hypothalamic nucleus (DMN). This structure is located dorsal to the ventromedial hypothalamic nucleus (VMN) and extends anteroposteriorly from the plane of the largest cross section of the VMN to the plane of the dorsal premammillary nucleus. Fibers from the DMN pass with the periventricular system and the dorsal longitudinal fasciculus of Schütz and have been traced to the midbrain tegmentum and reticular formation. Intrahypothalamic connections involve intensive networks between DMN, lateral hypothalamic nucleus (LHN) and VMN. Regarding neurotransmitters, recent studies indicate that the DMN receives noradrenergic innervation along two pathways, a dorsal and a ventral one. Monoamine-containing systems approach the DMN from the lateral hypothalamus and the bulk of these fibers are carried in the medium forebrain bundle from their cells of origin in the brain stem. Studies of the vascular supply indicate that both VMN and DMN receive their blood supply from the internal carotid artery. It has been recently demonstrated that the DMN is involved in the control of food intake and possibly water intake as well. Discrete lesions in the DMN have caused hypophagia and hypodipsia, and implantation of epinephrine and norepinephrine in this area has initiated eating. Many years ago, electrical stimulation of this area was reported to cause eating. Although DMN lesions cause hypodipsia, they do not result in the reduced water/food intake ratios that are so characteristic of the VMN syndrome. DMN lesions are also followed by reduced spontaneous activity (running wheel), but this reduced activity is not accompanied by increased weight gain and accretion of adipose tissue, the latter being consistently observed in the VMN rat. Rather, carcass fat remains normal in the DMN rat and carcass protein is either normal or slightly increased. Many of the aforementioned changes in weanling rats with DMN lesions, however, are not matched by similar alterations in the intermediary metabolism of carbohydrate and lipid. Possibly this is due to a “resetting” of a central autonomic control system that makes it possible for the DMN rat to adapt more efficiently to a reduced influx of substrate, i.e. the consistent hypophagia. From a review of the literature it appears that the DMN and their circuitry are involved in only a few neuroendocrine, i.e. hypothalamohypophyseal control mechanisms. Both lesion and cervical stimulation experiments suggest an involvement of the DMN in the control of LTH. Circumstantial evidence points to the DMN as a possible formation and/or storage site of growth hormone inhibiting factor (GIF). Although DMN rats show reduced ponderal and linear growth, they have been found to have normal or elevated plasma growth hormone (GH) levels. Both lesion and stimulation studies have yielded the impression that the DMN is not involved in thyroid, i.e., thyrotropin stimulating hormone releasing factor (TSHRF) control. Electrical stimulation of the DMN has been reported to result in a positive correlation between adrenal blood flow and adrenal corticoid release in hypophysectomized dogs. This has been interpreted as a coordinated response at the level of a “dorsomedial sympathetic vasodilator relay” rather than a “true” neuroendocrine effect via corticotropin releasing factor (CRF). Experiments that failed to demonstrate a relationship between the DMN and the tonic and cyclic control of luteinizing hormone releasing factor (LHRF) are discussed. The data reviewed indicate the existence in the dorsomedial hypothalamus of an area that exerts a profound influence on many aspects of neurovisceral and some neuroendocrine control systems.

A five-year-old boy succumbed 24 days following an unusual farm accident in which considerable manure was deposited in multiple lacerations.Death was due to an extensive meningo-encephalomyelitis caused by a nematode that is ordinarily saprophagous.

The superior temporal gyri were measured in 33 infants and in 33 adults. In the adults, most right superior temporal gyri were larger. This asymmetry was not found in infants, a difference which suggests greater growth of the right superior temporal gyri in the population from which our sample was taken. The asymmetry may be related to the functional asymmetry found by Penfield: some of his patients reported re-experiencing of past sensory experiences with electrical stimulation of the temporal lobe. This response was more frequently evoked from the right hemisphere.

A 45-year old man was referred to the Johns Hopkins Hospital with a seven-year history of repeated episodes of light-headedness, increasing irritability and forgetfulness. Examinations revealed a right superior, incongruous quadrantanopsia. EEG showed an abnormality in the left temporal lobe, and a cerebral angiogram outlined an avascular mass in the left cerebral hemisphere. At operation, the patient was found to have a giant dermoid cyst involving the left frontal, temporal and parietal lobes. Over the last 30 years we have encountered only 6 cases of intracranial epidermoids and 3 cases of intracranial dermoids. These cases are cited, and a discussion of the embryology, histology, clinical characteristics, and treatment of these lesions with a review of the literature is undertaken.

Three elderly women with marked progressive voice tremor, without other neurological symptoms, and negative family histories were investigated.

All had a 4-5 Hz respiratory tremor in expiration and, to a lesser degree, in inspiration; and all had vocal tremulousness synchronous with their respiratory irregularity. Articulation of phonemes was normal. In two cases the neurological examination was otherwise normal; in the third case there was a minimal 7½ Hz tremor in the left thumb and index finger.

Simultaneous speech and vocal air pressure recordings, as well as cinematographic studies of the vocal apparatus and diaphragm were carried out.

It is suggested that these cases represent primarily an action tremor of respiration, that they belong in the spectrum of essential tremor, and hence may be amenable to treatment with propranolol.

The kindling effect is a relatively permanent alteration in brain function which results from repeated electrical or chemical stimulation and culminates in the appearance of electrographic and behavioral convulsions whenever the original stimulus is reapplied. The effect results from tetanic activation in the anterior cortex, limbic system or associated areas of the adult mammalian brain, and the lasting alterations are transynaptic and quite widespread. They are based in part on synaptic facilitation, and they are accompanied by specific alterations in normal behavior. In these and other respects, kindling is analogous to normal learning. It is possible that the stored component (engram) of kindling involves the same physiological mechanism as the engram of normal long term memory. Morphological study of identified synapses has not provided conclusive evidence for an anatomical substrate of kindling, but physiological experiments demonstrate a lasting potentiation of the excitatory postsynaptic potential.

The field potentials recorded simultaneously at various depth of the rabbit’s visual cortex and superior colliculus were analysed following light ON and light OFF. The collicular ON and OFF potentials exhibited three slow components superimposed by fast rhythmic oscillations. Only the first slow component reversed its polarity with penetration from surface negative to positive in depth. The cortical ON and OFF responses similarly contained three slow waves which all reversed their polarity with electrode penetration: from surface positive to negative in deeper layers. The most striking difference between ON and OFF cortical responses is the absence of fast rhythmic oscillations in the cortical ON response.

A 56 year old woman developed symptoms of lumbar nerve root compression caused by a granuloma arising in the ligamentum flavum. The histological features of the lesion are discussed and the clinical and radiological findings of the patient are described.

The light and electron microscopic features of a pituitary adenoma composed of adrenocorticotrophic hormone (ACTH) and melanocyte stimulating hormone (MSH) cells with perivascular amyloid deposition is reported. Histochemical and fine structural data indicate that this material is APUDamyloid and is present in the extra-cellular perivascular spaces. It is suggested that the differences in fine structure and of distribution of the amyloid in pituitary adenomas is dependent upon the cell of origin.

Skin punch biopsies and huffy coats of white blood cells were examined electron microscopically in patients suffering from a variety of storage diseases. No specific abnormalities could be detected in Gaucher’s disease and adrenoleucodystrophy. While characteristic deposits were found in cutaneous nerves in globoid and metachromatic leucodystrophy, this method was deemed inferior to sural nerve biopsy. In gangliosidoses, on the other hand, pathognomonic membranous cytoplasmic bodies were common in axons of cutaneous nerves, and in generalized gangliosidoses marked vacuolation of many other cells was prominent. Specific deposits were found in various cells in skin punch biopsies and in lymphocytes of children suffering from ceroid lipofuscinoses, and in lymphocytes of their parents. This constitutes the easiest diagnostic laboratory procedure in such cases.

Two approaches to the study of the kindling phenomenon were discussed: 1) an attempt to identify the pattern of neural activity required to produce the changes underlying kindling and 2) an investigation into the nature of those changes. Three experiments were reported that used the neocortical transcallosal system as a monosynaptic model system in which to study possible synaptic mechanisms of the kindling effect. Experiment I showed an increase in the transcallosal evoked potential following neocortical kindling. Experiment II showed an increase in the strength of the transcallosal evoked cell discharge following neocortical kindling. Experiment III reported the results of an histological examination of neocortical tissue in kindled and non-kindled animals using the Golgi-Cox technique. Spine density, spine dimension and branching were measured for pyramidal cell apical dendrites. No differences were found between primary and secondary (contralateral) foci or between kindled and non-kindled animals.

This is a report of the first recorded instance of ventricular obstruction at the foramen of Monro by an ectatic carotid artery. The clinical course, investigations and treatment are described.

Secondary epileptogenesis was induced in the hippocampal cortex of the paralyzed bullfrog by means of localized, unilateral, intermittent electrical stimulation (kindling). Stimuli were designed to yield a brief but definite after-discharge. In control animals a progressive increase in after-discharge duration occurred at the 1° (stimulated) site and then at the 2° site (contralateral hippocampus). Spontaneous epileptiform potentials (SEP's) occurred between stimuli, eventually independently on both sides.

Cycloheximide (50 mg/kg) caused 88-99% reduction in protein synthesis, measured by 14C-leucine incorporation into brain protein. Cycloheximide-treated animals revealed no evidence of progressive prolongation of after-discharge duration when subjected to the kindling procedure (p = 0.1205xl0-7). SEP's were reduced in the cycloheximide-treated animals, and confined to 1° hemisphere (p=0.6xl0-10).

Since cycloheximide did not disturb normal electrogenesis or disrupt the after-discharges, this experiment distinguishes processes dependent upon electrical events from those requiring macromolecular synthesis. Protein synthesis seems critical to the emergence of spontaneous and autonomous epileptiform behavior of neural aggregates.

A 63 year old female with the ataxic form of Creutzfeldt-Jakob disease (CJD) is presented. In addition to amyloid plaques which were not associated with Alzheimer’s neurofibrillary tangles, rare profiles similar to those reported in Scrapie were also seen. To our knowledge, these profiles have never been observed in CJD and their presence in this condition adds a further morphologic similarity between the human and animal forms of subacute spongiform “viral” encephalopathies.

This is a single case report of an ovarian teratoma. It is a unique case of a primitive neuroepithelial tumor with many similarities to a medulloblastoma arising in an ovarian teratoma, and the second report of fetal cerebellum occurring in a teratoma of the ovary.