Long-term cognitive deficits following posterior fossa tumor resection: A neuropsychological and functional neuroimaging follow-up study.

The posterior fossa syndrome (PFS) consists of transient cerebellar mutism, cognitive symptoms, and neurobehavioral abnormalities that typically develop in children following posterior fossa (PF) tumor resection. The pathophysiological substrate of the syndrome remains unclear. We investigated eight children of whom five presented with a variety of clinically relevant non-motor language symptoms associated with cognitive and behavioral disturbances after PF tumor resection. Four children developed transient cerebellar mutism followed by dysarthric speech. Non-motor language symptoms consisted of agrammatism, anomia, impaired verbal fluency, comprehension deficits, and aspontaneous speech. Neurocognitive deficits included executive dysfunctions, concentration deficits, and visuo-spatial disorders. In addition, all children presented with behavioral and affective disturbances. Functional neuroimaging studies during the phase of mutism by means of SPECT showed perfusional deficits in the anatomo-clinically suspected supratentorial areas subserving language dynamics, syntax, naming, executive functioning, affective regulation, and behavior. A significant improvement of frontal perfusional deficits paralleled the clinical remission of mutism. These results add to the view that the PFS might represent a cerebello-cerebral diaschisis phenomenon, reflecting the metabolic impact of the cerebellar lesion on supratentorial cognitive and affective functions. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Optic chiasm astrocytomas of childhood. 1. Long-term follow-up

There are many in vivo animal models for studying airway mucus secretion and hypersecretion, each with advantages and disadvantages. Use of a particular test system will depend upon the aspect of secretion to be modelled. Airway hypersecretory diseases exhibit chronic mucus hypersecretion, of which the clinical impact is predominantly in the distal airways. The majority of documented test preparations study acute secretion, invariably using tracheal preparations, but have been invaluable in elucidating the normal physiology of airway mucus secretion. Chronic models of the hypersecretory state in the distal airways have been developed, but are predominantly histologic in nature (for example quantification of increased goblet cell number). There are few investigations of mucus hypersecretion. Examination of the 'antisecretory' potential of pharmaceutical compounds has been investigated predominantly in chronic histologic models with the drug being given 'prophylactically' rather than 'therapeutically'. Refinement of chronic hypersecretory models should lead to elucidation of the connection between airway irritation, inflammation, MUC gene expression, mucous cell hyperplasia/metaplasia, airway hypersecretion and bronchial hypersecretory disease.     

Epidural hematomas in the posterior cranial fossa

A review of 89 cases of posterior fossa epidural hematoma (PFEDH) is presented. The mortality rate was 17.9%. In 44 cases (49.4%) there were associated intracranial hematomas. In 30 cases the hematoma was localized within the boundary of the foramen magnum and the transverse and sigmoid sinuses ("pure" PFEDH). In 59 cases the hematoma extended beyond the sinuses to the occipital area ("mixed" PFEDH). In the pure PFEDHs, the bleeder could be identified in only six cases and in five cases the source was a bleeding transverse sinus. The bleeders disclosed in the mixed PFEDHs were a torn transverse sinus in 28 cases, a meningeal artery in three cases, and a bony fracture in three cases. The possibility of a PFEDH should be kept in mind when evaluating patients who have suffered an occipital blow resulting in a frontal or temporal hematoma. In our series, patients with the pure PFEDHs with no associated intracranial hematomas had the best prognoses. Nine patients developed a PFEDH after surgery for a supratentorial hematoma. In 14 cases the PFEDH was treated at the subacute or chronic stage. All but one patient survived with a good recovery. Children generally had better prognoses.     

Hemangioblastoma of the posterior fossa. The role of multimodality treatment

The authors made a review of a series of patients with hemangioblastomas of the posterior fossa treated between 1973 and 1993. A total of 32 patients were analyzed with 24 patients receiving resection, 8 patients receiving radiosurgery and 2 patients receiving conventional radiotherapy. The mortality in the patients with a resection was considered acceptable with 2 deaths (8%) and with a morbidity of 3 patients (12.5%). A review of the literature suggests that conventional radiotherapy with high doses (45-60 Gy) may have a role in the post-operative control of hemangioblastomas and in some cases could be employed even before the resection in order to facilitate the surgery. The radiosurgical treatment is regarded like adjuvant. Poor results were obtained with radiosurgery in large tumors where low doses (less than 20 Gy) were used. Because of the rarity and complexity of these tumors, mainly when associated with von Hippel-Lindau disease, a multicenter study could be useful with the assessment of the optimal utilization and combination of these treatment modalities.     

Radiological case of the month. Cystic meningioma of the posterior fossa

This short report serves as a warning to the unwary of possible "pseudoclusters" of infection with Aspergillus fumigatus as shown by the typing system, random amplification of polymorphic DNA (RAPD). This was demonstrated by typing 10 epidemiologically distinct isolates of A fumigatus using two different preparations of Taq DNA polymerase. One of the enzymes did not discriminate between the isolates, giving the false impression that a cluster of infection had occurred. Enzyme source is thus a key variable when using RAPD to distinguish between isolates of A fumigatus.     

Core neurocognitive functions in children treated for posterior fossa tumors.

Identifying cognitive deficits associated with pediatric brain tumors and their treatment is important in delineating the mechanisms of intellectual decline often associated with these diseases. The authors evaluated sustained attention, information processing speed, working memory, and IQ in 64 patients with posterior fossa tumors, including those treated with either: (a) surgery and cranial radiation (n = 32), and (b) surgery without radiation (n = 32). Ten patients treated for non-CNS solid tumors were included as a comparison group. The authors also examined the impact of relevant demographic and medical variables on neurocognitive outcome. The authors found that neither age at, nor time since, diagnosis predicted cognitive outcome in this sample. Further, sustained attention and working memory were largely intact and there were no differences between groups. Patients treated with cranial radiation demonstrated lowered short-form IQ and slow information processing speed: Patients treated with cranial radiation and who experienced postsurgical complications demonstrated the poorest performance. The authors consider information processing speed to be an excellent candidate mechanism in understanding the impact of cranial radiation on intellectual outcome. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Diagnostic problems in epidural hematoma of the posterior cranial fossa

Posttraumatic posterior cranial fossa haematoma is a rare occurrence. In our material it accounted for 1.5% of all intracranial haematomas. Due to its infrequency and diagnostic difficulties these patients are referred with delay to neurosurgeons and often die with signs of brain stem lesion. The reported case was a 25-year-old man with this haematoma and considerable diagnostic difficulties were encountered despite the application of modern imaging techniques. These difficulties hamper the qualification of the patient for operation which is the method of choice in these cases.     

Sexual function in patients with disseminated sclerosis. A 5-year follow-up study

Sexual dysfunction is known to occur in multiple sclerosis (MS). The purpose of the study is to describe the change in sexual function and symptoms in a five year follow-up study. Forty-nine patients (27 females, 22 males) with definite MS were interviewed and examined. The number of patients with sexual dysfunction increased significantly (p = 0.004) and involved females and males equally. Males usually had one or two symptoms, while females frequently had two or more symptoms. It is concluded that the risk of sexual dysfunction increases over time. Further studies concerning treatment possibilities are needed.     

Tentorial and posterior cranial fossa meningiomas: operative results and long-term follow-up: experience with twenty-six cases

In spite of significant recent advancements in imaging technology, monitoring [12] and surgical techniques [1,5,7,18,21,24], complete and safe removal of tentorial and posterior fossa meningiomas remains a formidable challenge for most neurosurgeons [6,8,9,14,15,17]. This paper reviews our experience with 28 patients with tentorial and posterior fossa meningiomas.     

Recurrent ameloblastoma of the jaws. A follow-up study

Familial hyperproinsulinaemia is a rare genetic disorder characterized by point mutations in the insulin gene which impair the conversion of proinsulin to insulin. We report here three members of a two-generation Caucasian family in whom this syndrome was identified by unexplained hyperinsulinism associated with normal glucose tolerance and normal insulin sensitivity. Plasma insulin immunoreactivity showed a reduced affinity for the insulin receptor and eluted mainly, on Biogel chromatography, at the position of proinsulin. Analysis of the PCR-amplified insulin gene by restriction enzyme mapping revealed a new recognition site for the enzyme Nla III, indicating a Arg65 to His mutation. Sequence analysis of exon 3 confirmed this mutation in one allele of the gene. CONCLUSION: This study reports a two-generation European-Caucasian family with hyperproinsulinaemia due to a substitution of His for Arg at position 65 in proinsulin, the seventh now identified worldwide and the second from Europe. The mutation generated a new restriction site on the insulin gene suggesting the usefulness of restriction enzyme mapping as a screening procedure.     

Disposition of presumed coronary patients from an emergency room. A follow-up study

All patients with presumed coronary problems seen at the Chaim Sheba Medical Center during a one-year period were followed up. The fate of those who were not hospitalized and the factors contributing to the two types of erroneous decisions, ie, refusing hospitalization to those needing it and unnecessary hospitalization of others, were evaluated. Approximately 50% of the patients were not admitted. Myocardial infarctions were later diagnosed in 6% of these patients. Another 8% were eventually categorized as other cardiac emergencies. Ten percent of all patients subsequently diagnosed as having myocardial infarctions were not admitted. On the other hand, 56% of the patients whose cases were later not considered to have been emergencies were hospitalized unnecessarily. Previous hospitalization for cardiac disease played a major role in making an error of both types. Other factors influencing the physician's decision regarding the patients' disposition included their age, sex, ethnic origin, and the findings from the emergency room electrocardiogram.     

Remote subarachnoid haemorrhage in the posterior fossa following supratentorial surgery. Clinical observation of 6 cases

Haemorrhage in regions remote from the site of following intracranial operations is rare, but they do occur. We performed supratentorial craniotomy on 639 patients between the time of introduction of computed tomography (CT) for clinical use in 1983 and June 1992; subarachnoid haemorrhage (SAH) in the posterior fossa occurred postoperatively in six of these cases. These included four patients with tumours in the sellar region, one with an arteriovenous malformation (AVM) and one who underwent superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis. The ages of the six patients ranged from 17-72 years. Haemorrhage occurred on the day of operation in one case and was detected on CT examination on the day following surgery in the remaining five cases. Of three patients with disturbance of consciousness, two underwent suboccipital craniectomy for reduction of intracranial pressure, while one received barbiturate therapy and later underwent cerebrospinal fluid (CSF) shunt surgery. No special treatment was necessary for the remaining three patients with less serious lesions. Five of the six patients ultimately recovered their pre-operative neurological status apart from the primary diseases. Factors inducing such haemorrhages seem likely to include displacement of the cerebellum by reduced CSF pressure during and after operations, and stretching and tearing of the veins and venules in the sulci of the tentorial surface of the cerebellum. Consideration should therefore be given to the maintenance of an appropriate CSF pressure during operation; this is particularly important in elderly patients and those with an atrophied cerebral cortex.     

Recurrent intermetatarsal neuroma. A follow-up study

Sixteen patients were evaluated following excision of recurrent intermetatarsal neuroma. Each underwent removal of a segment of nerve through an incision on the plantar aspect. The nerve stump was buried in the flexor digitorum brevis muscle. At the time of follow-up examinations, six patients were asymptomatic, seven experienced mild pain after extended activity, one suffered mild consistent pain with activity, and two suffered pain that limited activities.     

Endodermal cysts of the posterior fossa. Report of three cases and review of the literature

The pathogenesis of endodermal cysts of the posterior fossa is still incompletely understood. The authors reviewed three new cases and those reported in the literature to clarify the clinical, pathological, radiological, and surgical characteristics of these lesions. A total of 49 cases were reviewed. Details on demographic profiles, clinical characteristics, histopathological and radiological features, and surgical methods were collected. These cysts have a predominance in male patients (61%) and can occur at any age (birth-77 years). In patients with posterior fossa endodermal cysts there is a bimodal age distribution and headache is the most frequent complaint. On immunohistopathological examination, endodermal cysts were reactive for epithelial membrane antigen and for keratin immunostains whenever the latter were tested. The cysts were reactive for carcinoembryonic antigen in nine of 11 cases. Endodermal cysts were located anterior to the brainstem in 51% of cases and in the fourth ventricle in 21% of cases. They frequently appeared hypodense on computerized tomography scans, and in five cases, the lesion was missed. The cyst's appearance on magnetic resonance imaging is variable. Resection was complete in 19 cases and partial in 11; marsupialization was achieved in two cases. Three recurrences have been reported. Total excision with preservation of neurological function should be the goal. Cranial base approaches are helpful for surgical access in selected examples of these lesions.     

Multiple osteocartilaginous exostosis. A follow-up study

In order to evaluate the results of voice and speech rehabilitation after total laryngectomy some acoustic parameters (fundamental frequency, waveform perturbation) were examined in 18 total laryngectomy patients. Eight of these subjects had previously been surgically rehabilitated with a tracheal-esophageal phonatory valve while 10 had been submitted to esophageal speech rehabilitation. Analysis of results has shown that tracheal-esophageal voices are more likely to provide a stable fundamental frequency; there is also a tendency toward more clearly defined harmonics; jitter and shimmer are more similar to the values of normal subjects compared with those observed in esophageal speech. Such results seem to depend on a more regular vibration pattern in the pharyngeal-esophageal segment, due to the more efficient expiratory flow in tracheal-esophageal speech. Moreover we were able to demonstrate a correlation between the objective parameters evaluated and the subjective score on speech acceptability.     

Correlation of glucose consumption and tumor cell density in astrocytomas. A stereotactic PET study

To determine histological correlates of the variability of glucose consumption in astrocytomas, the authors performed positron emission tomography (PET) with 18F-2-fluoro-2-deoxy-D-glucose (FDG) and matched the PET scans three-dimensionally with computerized tomography scans obtained in a stereotactic frame before biopsy. Ten patients with astrocytomas of World Health Organization Grade 2 or 3 were studied; patients with glioblastomas, oligodendrogliomas, or oligoastrocytomas were excluded from the study to avoid any confounding effects of different cell types and necroses. In samples of pure tumor, glucose consumption correlated significantly with cell density, but not with nuclear polymorphism. It is concluded that tumor cell density is a major determinant of glucose consumption in astrocytomas. The use of PET with FDG may help to locate the highest cell density and thus improve the diagnostic yield of stereotactic biopsy.     

Pneumocephalus as a complication of posterior fossa surgery in the sitting position

A case of pneumocephalus complicating posterior fossa surgery in the sitting position is presented. Rapid diagnosis was obtained by computerised tomography. This condition should be suspected whenever a patient demonstrates delayed recovery or neurological deficit after craniotomy and nitrous oxide anaesthesia. The literature on the subject is reviewed.     

Neuropsychological functioning among the nonpsychotic relatives of schizophrenic patients: A 4-year follow-up study.

In a prior study of 54 relatives of patients with schizophrenia and 72 control participants, 3 neuropsychological functions met the criteria for risk indicators of the schizophrenia genotype: executive functioning, memory, and auditory attention. In an assessment of the stability of these findings, the sample was reexamined 4 years after the initial assessment. Three test scores were found to differ between groups (Immediate Verbal Memory, Delayed Verbal Memory, and Dichotic Listening Digits Detected) or to show a significant Group?×?Gender interaction (immediate and delayed verbal and visual memories). None of the test scores showed Group?×?Time interactions, suggesting that the discriminating power of the tests was stable over time. Evidence for deficits in working memory and rule learning on the object alternation test was also found. These results support the idea that neuropsychological dysfunction among relatives of patients with schizophrenia is a stable trait caused by the familial predisposition to schizophrenia. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Value of oncologic follow-up of patients operated for colorectal cancer. A prospective study of 1000 patients

OBJECTIVE: The purpose of our prospective study was to evaluate the contribution of follow-up in resected colorectal cancer. METHODS: One-thousand patients with colorectal adenocarcinoma who underwent radial resection from January 1975 to January 1990 were prospectively divided into two groups: the first group (n = 442, 42%) entered a 5-year follow-up protocol and the second group (n = 558, 56%) was free to make unscheduled visits in case of symptoms. RESULTS: Recurrence was found in 31.5% of the patients in the first group compared with 33% in the second group. Chorioembryonic antigen was the most accurate test detecting recurrence: 77% of the cases (97% for hepatic metastasis). Surgical resection of recurrent tumors was performed in 37% of the group 1 patients (curative resection in 15%) and in 9% (curative resection in 1.5%) of the group 2 patients (p < 0.001). 5-year survival after recurrence in group 1 was 11.5% versus 1% in group 2 (p < 0.01). CONCLUSION: These results emphasize the rational for a follow-up program in patients undergoing surgery for colorectal cancer.     

Excretory azoospermia: contribution of exploratory and curative surgery. Apropos of a follow-up of 33 patients

Thirty-three patients with azoospermia of apparently excretory origin underwent surgery for epididymis-deferens anastomosis and/or epididymal sperm puncture. Pathology examinations of the epididymal fluid and biopsies of the testicles or epididymis were performed at surgery. Based on the clinical presentation, sperm results and per-operative findings, patients were divided into six groups by etiology: idiopathic azoospermia (n = 5), post-infectious azoospermia (n = 15), agenesia of the excretory (n = 6) or secretory (n = 3) ducts, vasectomy (n = 2), and obstruction of the ejaculatory ducts (n = 2). Peroperative identification of spermatozoa at epididymal puncture or biopsy was statistically more frequent in patients with agenesis of the excretory ducts than in patients with post-infectious or idiopathic azoospermia. Biopsies of the testicle led to the diagnosis of secretory azoospermia in 3 cases and revealed a functional parenchyma in all the other groups of patients. Epididymis-deferens anastomosis was performed in 45% of the cases and was successful in 13%. Rate of fertility with the intracytoplasmic sperm injection was 33%; there was no difficulty in using fresh or frozen sperm. Clinical pregnancy was continued to term with frozen sperm. This study confirms that testicular function is preserved in excretory azoospermia. With or without epididymis-deferens anastomosis, epididymal spermatozoa can generally be preserved for later use. Couples should however be counselled on the delays to contraception which may vary from months to years.