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Prospective study of nesidioblastosis in newborns and infants: hypoglycemic seizures, epileptogenesis and the significance of the C-peptide suppression test in pancreatectomy.

The long-term follow-up of chronic hyperinsulinemic seizures, epileptogenesis and other neurological complications in five patients who were treated with conservative therapy followed by pancreatectomy during the neonatal period and infancy, who were confirmed to have diffuse nesidioblastosis are described. The reaction pattern of the C-peptide (CPR) suppression test and its relation to the final extent of pancreatectomy was examined in four patients. The chronological change in electro-encephalography (EEG) and its epileptogenesis was also examined in each patient during hyperinsulinemic hypoglycemia, and during normoglycemia in a long-term post-pancreatectomy follow-up. All patients demonstrated several types of hypoglycemic seizures, ranging from apnea, erratic seizures, evolving to generalized/unilateral tonic-clonic or tonic seizures, myoclonic seizures and EEG abnormalities. Four of five patients still suffered from epilepsy at the age of 4-22 years. The reaction pattern of the CPR suppression test showed dichotomy, with a hyper-reactive pattern in two patients who required total pancreatectomy to control hypoglycemia, and a suppression pattern in two other patients treated with 90-95% pancreatectomy. Neonatal onset and subsequent myoclonic seizures were ominous signs of epileptogenesis to various types of intractable epilepsy and other neurological sequelae. A prompt diagnosis and pancreatectomy of a sufficient extent at the first operation are essential. The CPR suppression test may be useful for a prompt diagnosis and selection of the extent of pancreatectomy.

Blood Chemical Analysis↗

Management of hyperprolactinaemic amenorrhoea.

Results of treatment of 52 patients with amenorrhoea associated with hyperprolactinaemia are presented. All patients had a detailed radiological examination of the pituitary fossa, including lateral tomography in every patient and air encephalography in those in whom a pituitary tumour was suspected. There were 17 patients with untreated pituitary tumours, 5 patients with previously treated pituitary tumours and persisting hyperprolactinaemia, and 30 patients with normal pituitary radiology. Patients with pituitary tumours were treated either by transsphenoidal or transfrontal surgical extirpation of the tumour, followed, if necessary, by external irradiation and/or bromocriptine, Four patients were treated with external irradiation as primary therapy, and three patients who did not wish to conceive were treated with bromocriptine as primary therapy. Patients with normal radiological appearances were treated with bromocriptine as primary treatment. Ovulatory menstrual cycles developed in 42 patients and there were 19 pregnancies. Those ovulating but not conceiving had adequate nonendocrine factors to account for the disparity. Failure of response was seen in 10 patients and was due to inadequate fall of prolactin in response to surgery (2 patients), external irradiation (3 patients) and bromocriptine (1 patient), and gonadotrophin deficiency which developed after surgery in 3 patients but was present pre-operatively in 1. The relative merits of treatment by surgery, external irradiation and bromocriptine are discussed and a policy of treatment outlined.

Adult↗

Whiplash: chronic organic brain syndrome without hydrocephalus ex vacuo.

Eleven patients who had sustained whiplash injuries to the neck were studied by pneumo-encephalography, by a variety of psychological tests, and by psychiatric evaluation with a view to assessing whether organic CNS changes might, in some cases, be a consequence of such injuries. Patients with traumatic encephalopathy, and age- and sex-matched groups of patients served as control groups. Pneumoencephalography showed normal findings in the whiplash group as a whole, the width of the ventricular system being significantly smaller than that of patients with traumatic encephalopathy. Psychological tests and psychiatric evaluation indicated that organic brain pathology was present in the whiplash patients, although to a lesser extent than in patients with traumatic encephalopathy.

Adult↗

Echo-encephalographic studies in patients with space-occupying lesions in the posterior fossa.

Echo-encephalographic studies were performed on 57 patients treated for space-occupying lesions in the posterior fossa. Preoperative echo-ventriculography (EVG) on 36 patients showed that 32 (89%) had dilatation of the third ventricle. Three of the four patients with normal preoperative ventricle width had cerebello-pontine angle tumors. Papilledema was lacking in many cases with considerable ventricular dilatation on EVG. Postoperative examinations on 49 patients showed in most cases a fairly rapid normalization of ventricular size often already during the first 1-4 weeks after operation. Postoperative EVG monitoring of the ventricular system was particularly useful for detection of shunt dysfunctions and recurrence of tumors. Computerized tomography have to some extent replaced echo-encephalography, but it is concluded that EVG still offers some definite advantages for certain application in the management of patients with posterior fossa lesions.

Adolescent↗

The empty sella and pituitary adenomas. A theory on the causal relationship.

In 44 consecutive patients with sellar volume larger than 1100 mm3, computer tomography showed that 20 had an empty or partly empty sella. None had radiological evidence of a suprasellar tumor. 10 of the 20 patients had experienced episodes with acute neurological symptoms presumably reflecting a pituitary apoplexy. It is suggested that an unknown proportion of intrasellar adenomas may disappear as a result of an infarction, which may comprise the entire adenoma or part of it--leaving an empty or partly empty sella as diagnosed by computer tomography. Air encephalography will demonstrate the empty sella only if the diaphragmatic aperture is large enough to allow cisternal herniation. The infarction may present clinically with no, slight or severe acute neurological symptoms. Late consequences of a pituitary adenoma infarction may be rhinorrhea or hydrocephalus.

Adenoma↗

Correlations between echo-encephalographic and computer tomographic measures of third and lateral ventricle size in children and adults.

Accuracy of echo-encephalography (EVG) was assessed by comparison with computer-tomography (CT) in a material of 49 children aged 2 weeks to 15.5 years and 158 adults aged 17 to 83 years. Third ventricle width could be measured on EVG in all the patients, and the correlations with CT values were excellent both in children and adults (r = 0.89 and r = 0.94, respectively). Lateral ventricle echoes could be recorded in 43 children (87.7%) and in 95 adults (60.1%). There were very good correlations between lateral ventricle size estimated as LVI (lateral ventricle index on EVG) and BI (body index on CT) both in children and adults (r = 0.84 and r = 0.86, respectively). In adults, a fairly large proportion (46.3%) of the patients where lateral ventricle echoes could be recorded, apparently had essentially normal ventricle systems, although there was evidence that these echoes could be recorded more easily from widened ventricles.

Adolescent↗

Callosal agenesis followed postnatally after prenatal diagnosis.

Callosal agenesis is a congenital brain anomaly caused by embryonal hypogenesis of the corpus callosum. Concerning the neurological prognosis, epilepsy and motor disturbance are noted in some cases, while many cases are asymptomatic and the prognosis is good. We report a fetus tentatively diagnosed with hydrocephaly on prenatal echo-encephalography, which was performed without adequate explanation to and understanding of the parents. The parents had not expected an abnormality before the screening, and were subsequently not psychologically prepared for the discovery of the congenital brain anomaly on imaging. Moreover, they received no guidance on how to deal with any possible abnormalities. The pregnant mother was referred to our hospital. Prenatal MRI was performed after informed consent was obtained, and the fetus was diagnosed with callosal agenesis. The patient was followed for 5 years, and neurological development was normal. However, the parents have remained anxious while raising the child. Thus, the prenatal diagnosis of callosal agenesis in this case caused unnecessary mental burden to the parents. Here, we report the course of the case, and discuss the way prenatal ultrasonography should be used as a prenatal screening method, and the importance of counseling before the test.

Agenesis of Corpus Callosum↗

Evaluating cost-effectiveness of diagnostic equipment: the brain scanner case.

An approach to evaluating the cost-effectiveness of high-technology diagnostic equipment has been devised, using the introduction of computerised axial tomography (CAT) as a model. With the advent of CAT scanning, angiography and air encephalography have a reduced, though important, role in investigating intracranial disease, and the efficient use of conventional equipment requires the centralisation of neuroradiological services, which would result in major cash savings. In contrast, the pattern of demand for CAT scanning, in addition to the acknowledged clinical efficiency of the scanner and its unique role in the head-injured patient, ephasies the need for improved access to scanners. In the interest of the patients the pattern of service must change.

Brain↗

True glucose content of lumbar and ventricular cerebrospinal fluid.

Glucose levels in 154 random specimens of lumbar cerebrospinal fluid, normal as regards cell and protein content, were estimated by the glucose-oxidase method and a mean value of 60.5 mg./100 ml. (S.D. 7.3) was obtained. In 33 fasting patients subject to air encephalography the mean cerebrospinal fluid (C.S.F.) glucose level was 56.5 mg., and mean blood glucose level 65.5 mg. with an average C.S.F. glucose: blood glucose ratio of 0.85.

Blood Glucose↗

A case of high-pressure intracerebral pouch.

A case is presented of a 13-year-old boy with a long-standing high-pressure pouch in the right parietal lobe containing cerebrospinal fluid. The clinical presentation was of epilepsy. Isotopic studies suggested that the pouch was filling along the subarachnoid space at the back of the corpus callosum in the posterior part of the interhemispheric fissure. There was a dilated sulcus at this point, which filled freely on air encephalography. Pressure studies suggested the likelihood of a venous pulsation filling mechanism with a valvular action. Electrocorticography, excision of an epileptic focus in the wall of the cavity and opening of the ventricular wall into the pouch have produced a good clinical result. Computerized tomography after operation has confirmed correction of the high-pressure state.

Adolescent↗

Symptomatic hypopituitarism revealing a primary empty sella turcica.

A 64-year-old nulliparous woman presented with clinical signs of thyroid and adrenocortical insufficiency. Subsequent hormonal investigations demonstrated a failure of all anterior pituitary functions. Pneumotomo-encephalography revealed a large arachnoid herniation, leading to the diagnosis of primary empty sella turcica syndrome with secondary panhypopituitarism. This unusual observation emphasizes the necessity of ruling out an empty sella turcica syndrome in patients with pituitary insufficiency.

Empty Sella Syndrome↗

A head-holding device for computed tomography.

The authors describe a chin holder, modified from a commercially available encephalography chair, which significantly decreases head movement and resultant motion artifacts during computed tomography.

Humans↗

Enlarged cisternae magnae and posterior fossa cysts simulating Dandy-Walker syndrome on computed tomography.

Computed tomography was performed on 5 children with posterior fossa cystic abnormalities. On an axial tomogram, an enlarged cisterna magna could not be distinguished from a Dandy-Walker variant, nor could an encysted fourth ventricle be distinguished from a subarachnoid cyst. Supplementary air encephalography or sagittal reconstruction of tomograms is necessary to define posterior fossa cysts accurately.

Adolescent↗

Brain images of pain.

Combined magneto- and encephalography proves the sequential involvement of multiple cortical structures in pain processing. Bilateral activity in secondary somatosensory cortices reflects the sensory-discriminative component and is reduced in states of unconsciousness. Later activity in the posterior cingulum reflects the emotional-aversive component, which is blocked by narcoanalgesics.

Brain↗

No specific thalamic influence on cell composition, sugar or electrolytes of peripheral blood.

Cellular components, blood sugar, and serum electrolyte concentrations of chloride, sodium and potassium were analyzed serially in 20 patients who underwent either stereotactic thalamotomy, frontal internal capsulotomy or subcaudate tractotomy. No significant changes were seen during the interventions either after electrical stimulation or coagulation of the target area, but 3 h after the operation the neutrophile granulocyte count was significantly raised. This reaction reached a peak by the morning of the first posoperative day. Other cellular elements, blood sugar, and serum electrolytes remained unchanged. We believe that the leukocytosis is not a specific effect of the thalamic lesions, but a nonspecific stress reaction, similar to that seen after air encephalography.

Adult↗

Cerebrospinal fluid spectrophotometry and computerized transverse axial tomography (EMI scanning) in cerebrovascular diseases. Comparative study between two actual diagnostic methods in cerebrovascular disorders.

51 patients with cerebrovascular diseases were examined by CSF spectrophotometry (CSF-SPE) and computerized transverse axial tomography (CTAT: EMI scanning), most cases being followed by several examinations. Specific diagnoses were detected in 98 (CSF-SPE) and 75% (CTAT) of the cases, generally by only one examination. In cases examined angiographically or by isotope encephalography, the corresponding figures were 50 and 0%. The combination of CSF-SPE and CTAT examination obviously means a breakthrough in diagnosis of cerebrovascular diseases which is naturally a prerequisite for the more rational therapy of these common and frequently disabling disorders.

Angiography↗

Development of a liposarcoma out of the capsules of a hygroma.

We treated a patient who had a very rare finding: the development of a malignoma out of the capsules of the subdural hygroma covering the right occipitotemporal brain. Lumbar encephalography and arteriography confirmed the fact that surgery was necessary. A subdural-auricular shunt was performed and revised 2 years later. During the second operation, the external and internal capsules were removed in addition to the shunt. 3 years later the hygroma and large capsules were removed again and a ventricular-subdural drainage performed. 1 year later once more a subdural-auricular shunt was performed. 10 months later a tumour was found to be a pseudomucinous liposarcoma with large hyperchromatic nuclei and oval cells with large vacuoles of neutral fat. Renewed growth of the tumour led to the child's death. The histological and macroscopic findings of the capsules suggest the development of a malignoma within the last 3 years.

Brain Neoplasms↗

Characterisation of the pro opiocortin family of peptides in human cerebrospinal fluid.

Chromatography under acid dissociating conditions in conjunction with radioimmunoassay has been employed to investigate the nature of peptides related to opiocortin in human cerebrospinal fluid. Samples of cerebrospinal fluid (CSF) were collected for chromatography from 15 patients prior to air encephalography. 2 patients had pituitary dependent Cushing's disease, 3 non-endocrine neurological disease and 10 non-ACTH related pituitary disease. The column fractions were assayed for N- and C-terminal beta-lipotropin, N-terminal ACTH and gamma-MSH immunoreactivity. Elution profiles obtained from chromatography on Sephadex G-50 demonstrated peaks of immunoreactivity corresponding to the elution positions of synthetic human beta-endorphin, highly purified beta-lipotropin and highly purified gamma-lipotropin in all CSF samples. A peak of a large molecular weight material with N and C terminal beta-lipotropin immunoreactivity was also detected. Chromatography of CSF on Sephadex G-75 showed this large molecular weight peak to be comprised of peptides eluting in the positions of a 31K molecular weight marker with beta-lipotropin and ACTH immunoreactivity and a 16K molecular weight marker with gamma-MSH immunoreactivity. This suggests the presence of the common precursor to ACTH and LPH in the CSF.

Chromatography, Gel↗