Transient "cerebellar" mutism

The term "cerebellar mutism" refers to a specific disorder in which a complete but transient loss of speech, followed by dysarthria, occurs following resection of intrinsic posterior cranial fossa tumors or cerebellar hemorrhages, or upon trauma. Although it is well known that the lack of long-tract findings and cranial nerve (CN) involvement is the rule, the pathophysiology of cerebellar mutism has not been clearly elucidated. A review of the relevant literature disclosed 93 patients with this condition, the majority of these being in the pediatric age group. The neuropathological findings were as follows: 57 primitive neuroectodermal tumors (PNET), 19 astrocytomas, 10 ependymomas, 5 vascular malformations, 1 metastatic tumor, and 1 traumatic injury. The interval before the onset of mutism ranged from 0 to 168 h (mean 40.9 h). The mutism lasted from 1 to 168 days (mean 37.6 days). Subsequent dysarthria was present in 75 (80%) of the 93 patients. In this article, some specific recent illustrative reports are presented, and the concept of the role of the cerebellum in language and cognition is discussed. With these data as our point of departure, various hypotheses that have been advanced to explain the pathogenesis of this transient speech disorder are analyzed. The findings of the study suggest that the cause of the cerebellar mutism is the ischemia caused by vasospasm, as it usually developed after a latent period.     

Evaluation of drug concentration in tissues after percutaneous administration of non-steroidal antirheumatics]

Forty-two cases of medulloblastoma of posterior fossa in children are presented in this paper. Of the children, 28 were males and 14 females; their age ranged from 1.5 to 12 (mean 7.3) years. The foci found were in vermis (36) and cerebellar hemispheres (6). All of these children presented symptoms and signs of obviously increased intracranial pressure, and 31(77%) of them had the signs of cerebellar functional deficits. CT scan showed severe obstructive hydrocephalus in 39 cases. 42 children with posterior fossa medulloblastoma underwent surgical resection. Three died of postoperative prespiratory and circulatory failure, 39 made good recovery and received craniospinal radiotherapy and adjunct chemotherapy. Up to now, none died. Surgical intervention and operative procedures were emphasized particularly. Radiotherapy and chemotherapy were discussed also.     

Task Switching After Cerebellar Damage.

The authors of this study investigated task switching following cerebellar damage. The study group consisted of 7 children and adolescents (M age = 13.8 years) who underwent surgical removal of a benign posterior fossa tumor. They were tested at a sufficient interval after surgery (M lag = 6.13 years) for restoration of normal cognitive skills and intelligence. Although all showed normal learning of the task compared with control participants, when rapid behavioral changes were required (short preparation time), they exhibited behavioral rigidity manifested by enhanced switching cost. These results are in line with another study on serial reaction time with the same patients (A. Berger et al., in press). They have important implications for our understanding of the cognitive sequelae of early cerebellar damage as well as the involvement of the cerebellum in task switching. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Behavioral approaches to neuropsychological rehabilitation.

Behavioral approaches have been applied to a wide variety of behavioral and cognitive disturbances resulting from brain damage or disease. This article provides a comprehensive and critical review of the literature concerned with behavioral interventions in neuropsychological rehabilitation. The article examines 6 categories of target behavior: inappropriate social behavior, attention and motivation, unawareness of deficits, memory, language and speech, and motor disturbance. The efficacy of behavioral approaches for treatment of the neurologically impaired and implications for the future role of behavioral approaches in neuropsychological rehabilitation are discussed. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Sympathetic response to stimulation of the pontine A5 region in conscious rabbits

OBJECT: This study is a retrospective analysis of clinical data obtained in 28 patients affected by obstructive hydrocephalus who presented with signs of midbrain dysfunction during episodes of shunt malfunction. METHODS: All patients presented with an upward gaze palsy, sometimes associated with other signs of oculomotor dysfunction. In seven cases the ocular signs remained isolated and resolved rapidly after shunt revision. In 21 cases the ocular signs were variably associated with other clinical manifestations such as pyramidal and extrapyramidal deficits, memory disturbances, mutism, or alterations in consciousness. Resolution of these symptoms after shunt revision was usually slow. In four cases a transient paradoxical aggravation was observed at the time of shunt revision. In 11 cases ventriculocistemostomy allowed resolution of the symptoms and withdrawal of the shunt. Simultaneous supratentorial and infratentorial intracranial pressure recordings performed in seven of the patients showed a pressure gradient between the supratentorial and infratentorial compartments, with a higher supratentorial pressure before shunt revision. Inversion of this pressure gradient was observed after shunt revision and resolution of the gradient was observed in one case after third ventriculostomy. In six recent cases, a focal midbrain hyperintensity was evidenced on T2-weighted magnetic resonance imaging sequences at the time of shunt malfunction. This rapidly resolved after the patient underwent third ventriculostomy. CONCLUSIONS: It is probable that in obstructive hydrocephalus, at the time of shunt malfunction, the development of a transtentorial pressure gradient could initially induce a functional impairment of the upper midbrain, inducing upward gaze palsy. The persistence of the gradient could lead to a global dysfunction of the upper midbrain. Third ventriculostomy contributes to equalization of cerebrospinal fluid pressure across the tentorium by restoring free communication between the infratentorial and supratentorial compartments, resulting in resolution of the patient's clinical symptoms.     

Global attentional deviance as a marker of risk for schizophrenia: Specificity and predictive validity.

Evaluated global attentional deficits as possible childhood markers of schizophrenia. Performance deviance was assessed across a continuous performance test, an attention span task, and the Digit Span subtest of the Wechsler Intelligence Scale for Children (WISC). 63 children of schizophrenic parents, 43 children of parents with affective disorders, and 100 children of normal parents were tested when the children were 7–12 yrs old. A subgroup of Ss at risk for schizophrenia displayed extreme global deviance across attentional measures. Only a small number of Ss at risk for affective disorders and normal comparison Ss showed similar extreme attentional deficits. The global attentional deficits measured at these early ages were related to behavioral disturbances (behavior was assessed in 3–6 mo intervals since 1971) in young adulthood for Ss at risk for schizophrenia, but not for Ss in the affective risk group or in the normal comparison group. It is concluded that global attentional deficits have considerable specificity for predicting behavioral problems thought to be precursors of schizophrenia or schizophrenia-related disorders. (54 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Neuropsychological indexes of a partial frontal syndrome in patients with nonfrontal gliomas.

33 brain tumor patients (aged 26–74 yrs) were investigated with quantitative and qualitative neuropsychological assessments, systematic behavioral observations, and recordings of regional cerebral blood (rCBF). Ss with supratentorial highly malignant gliomas showed severe disturbances of attention, lack of control over premotor and executive functions, distractibility, a deficient abstract attitude, a loss of speech initiative, and diminished speech production. Such impairments are associated with functions of the frontal lobe system and may be related to frontal functional cortical changes as mirrored by rCBF. Signs of frontal dysfunction were seen in Ss with nonfrontal tumors and may suggest frontal lobe diaschisis in patients with Grade III–IV astrocytomas. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Future shock and medical research*

The AA. report 9 cases of arteriovenous angiomata in the posterior cranial fossa with chronic evolution: in 8 of these the diagnosis was made before a subarachnoid haemorrhagic episode precipitated the symptomatology. Angiomata in the posterior cranial fossa are detected less often than sopratentorial angiomata, and the possible reasons of this are considered. During the last 11 years in the Neurosurgical Institute of the University of Padua the incidence was 23%, one of the highest reported in literature. The clinical diagnostic criteria of angioma in the posterior cranial fossa with chronic evolution are also discussed. The importance of a fluctuating development of the symptomatology is emphasised with an almost constant association of signs of pyramidal and cerebellar involvement, as well as the differential diagnostic problems with multiple sclerosis. In six of the cases the malformation was so extensive to make it impossible to determine afferences and drainages, so that a surgical operation could not be carried out. This finding seemed rather peculiar, owing to the relatively poor simptomatology, mainly if compared with smaller supratentorial malformations. It appears from the literature that angiomata in the posterior cranial fossa including those found at autopsy are as common as the supratentorial ones; this would suggest that many of these lesions are not diagnosed in life.     

Posterior fossa decompression for Chiari I deformity, including resection of the cerebellar tonsils

This is an analysis of 19 consecutive cases of symptomatic patients with Chiari I deformities, undertaken to evaluate the long-term effect of posterior fossa decompression and duraplasty, assessed by postoperative imaging. Sixteen of the patients had syringomyelia and three had foramen magnum syndromes without a syrinx. Eighteen patients underwent posterior fossa craniectomy, subpial resection of the cerebellar tonsils, and duraplasty. Four patients were 16 years of age or younger. One of the children with syringomyelia had a posterior fossa decompression without resection of the tonsils. In the 15 patients with syringomyelia whose surgery included resection of the tonsils, the syrinx was reduced or resolved in 14. The patient whose syrinx did not change was a child with a lumbosacral lipoma. Three patients had syndromes of the foramen magnum without a syrinx, and of these only a patient with prior chemical and bacterial meningitis caused by a lumboureteral shunt failed to improve dramatically. When our patients are combined with 40 in the literature treated by decompression and duraplasty, 51 of 55 patients had reduction or resolution of the syrinx. Although it does not clearly affect the result, resection of the tonsils can be done safely.     

Deanna M. Barch: Award for Distinguished Scientific Early Career Contributions to Psychology.

Announces Deanna M. Barch as a recipient of the Award for Distinguished Scientific Early Career Contributions to Psychology for 2002. A biographical statement is included, along with major works and contributions for the field. Barch received this award for research on the relationship between clinical, cognitive, and neurobiological disturbances in schizophrenia. Using behavioral methods, functional neuroimaging, and computational modeling, her work has taken ideas from basic research on the function of prefrontal cortex and used these to dissect the specific contribution that prefrontal deficits make to cognitive abnormalities in attention, memory, and language in schizophrenia and their expression as clinical symptoms. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Interleukin-1 inhibits Leydig cell steroidogenesis without affecting steroidogenic acute regulatory protein messenger ribonucleic acid or protein levels

This study compared levels of referential communication disturbance in speech samples from 41 stable schizophrenia outpatients, 46 parents of patients, and 23 nonpsychiatric control participants in affectively positive versus affectively negative conditions. The speech of the patients and parents showed elevated frequencies of reference failures in the affectively positive condition compared with control participants: the speech of the patients became more disordered in the affectively negative condition, whereas the speech of the parents did not. These results support the idea that referential communication disturbances reflect vulnerability, as well as overt illness, but that affective reactivity of these disturbances is associated mainly with the manifest illness. These findings are consistent with biological, cognitive, and psychological theories about the processes underlying stress responsiveness of schizophrenic symptoms more generally.     

Psychological assessment of sexually abused children for legal evaluation and expert witness testimony.

This article presents the theory and practice behind the psychological assessment of sexually abused children using the posttraumatic stress disorder subcategories for abused children. Measurement of cognitive disturbances including intrusive recollections of the trauma, affective disturbances by simultaneously experienced high arousal and high avoidance symptoms, and observable behavioral changes are described, and practical approaches including case examples are presented. Included in this article is a discussion about the different standards of legal proof that psychologists must meet in the various kinds of child sexual abuse cases that they might be called upon to consult. Psychologists need to know basic child development and have the assessment skills to measure it as well as specialized training in the field of child abuse to avoid the perils of becoming overinvolved in these emotional cases. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Linguistic focus affects eye movements during reading

OBJECTIVE: To clarify the diagnostic significance of selective mutism (elective mutism in DSM-III-R). METHOD: Fifty children with selective mutism were evaluated systematically by means of semistructured clinical interviews and rating scales to obtain detailed diagnostic information. RESULTS: All 50 children met DSM-III-R criteria for social phobia or avoidant disorder and 24 (48%) had additional anxiety disorders. Clinical measures of anxiety and behavioral symptoms supported the presence of anxiety disorders as a characteristic of selectivity mute children. Only one case each of oppositional defiant disorder and attention-deficit hyperactivity disorder was found. CONCLUSIONS: Persistent selective mutism typically presents in the context of anxiety disorders.     

Differential accumulation and tissue distribution of mosquito hexamerins during metamorphosis

We report on 30 cases where we have used cortical stimulation mapping to define the areas representing sensorimotor, language and speech functions under local anesthesia to facilitate resection of space-occupying lesions near these areas. Under the simplistic concept that Broca's area lies in the frontal operculum (inferior frontal gyrus) and that Wernicke's area is located in the posterior perisylvian area (superior temporal, angular and supramarginal gyri), we found language and speech function to be represented outside these areas in up to 4 stimulation sites of 15 patients. The results of cortical stimulation mapping were therefore essential to decide on the optimal access route to the lesions that were located subcortically and on the optimal resection plane in gliomas. After the limits of these areas and of the lesions had been established with stimulation mapping and with intrasurgical microscopic smear preparations, respectively, lesions were safely removed under continuous monitoring of sensorimotor, language and speech function. Immediately after surgery we encountered language and speech deficits in 9 patients (30%), which resolved completely in 5 and incompletely in 4 instances. Thus, language functions were normal in 26 patients (87%) at the end of the follow-up period. It is concluded that use of this technique allows safe and extensive resection of lesions that would otherwise have been considered hazardous to remove or inexcisable.     

Affective reactivity of speech in schizophrenia patients and their nonschizophrenic relatives.

This study compared levels of referential communication disturbance in speech samples from 41 stable schizophrenia outpatients, 46 parents of patients, and 23 nonpsychiatric control participants in affectively positive versus affectively negative conditions. The speech of the patients and parents showed elevated frequencies of reference failures in the affectively positive condition compared with control participants; the speech of the patients became more disordered in the affectively negative condition, whereas the speech of the parents did not. These results support the idea that referential communication disturbances reflect vulnerability, as well as overt illness, but that affective reactivity of these disturbances is associated mainly with the manifest illness. These findings are consistent with biological, cognitive, and psychological theories about the processes underlying stress responsiveness of schizophrenic symptoms more generally. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Extralobar pulmonary sequestration presenting as a mediastinal malignancy

OBJECTIVE: We intended to characterize the CT patterns of hemorrhage associated with ruptured posterior inferior cerebellar artery (PICA) aneurysms. MATERIALS AND METHODS: CT scans of 44 cases of angiographically confirmed ruptured saccular PICA aneurysms (4) aneurysms at the junction of the vertebral artery and the PICA and three distal PICA aneurysms) were retrospectively reviewed. All scans had been obtained within 2 days of the subarachnoid hemorrhage (SAH) (day 0 [less than 24 hr], 35 patients; day 1, eight patients; day 2, one patient). Presence or absence of hemorrhage in specific subarachnoid, intraventricular, and intraparenchymal locations was noted, as were the presence and degree of hydrocephalus. RESULTS: Posterior fossa SAH was present in 95% of cases. Isolated posterior fossa SAH was present in 30% of cases, but in no case was isolated supratentorial SAH present. Supratentorial SAH was present in 70% of cases. SAH involving the sylvian fissure or the interhemispheric region was present in 25% and 23% of cases, respectively. SAH along the convexity was present in 2% of cases. Intraventricular hemorrhage (IVH) with or without associated SAH was seen in 95% of cases, whereas isolated IVH was seen in 5% of cases. Hydrocephalus was present in 95% of cases and was moderate to marked in 70%. Both IVH and hydrocephalus were present in 93% of cases. CONCLUSION: Ruptured PICA aneurysms almost always coexist with hydrocephalus and IVH, as seen in 93% of cases, and almost never coexist with SAH along the convexity. The most common pattern of hemorrhage associated with such aneurysms includes IVH and posterior fossa hemorrhage. Extensive supratentorial SAH, in conjunction with posterior fossa SAH, is a common finding in patients with ruptured PICA aneurysms. SAH isolated to the posterior fossa is present in a sizeable minority of cases.     

Cerebellum in attention-deficit hyperactivity disorder: a morphometric MRI study

Clinical, neuroanatomic, neurobehavioral, and functional brain-imaging studies suggest a role for the cerebellum in cognitive functions, including attention. However, the cerebellum has not been systematically studied in attention-deficit hyperactivity disorder (ADHD). We quantified the cerebellar and vermal volumes, and the midsagittal areas of three vermal regions, from MRIs of 46 right-handed boys with ADHD and 47 matched healthy controls. Vermal volume was significantly less in the boys with ADHD. This reduction involved mainly the posterior inferior lobe (lobules VIII to X) but not the posterior superior lobe (lobules VI to VII). These results remained significant even after adjustment for brain volume and IQ. A cerebello-thalamo-prefrontal circuit dysfunction may subserve the motor control, inhibition, and executive function deficits encountered in ADHD.     

Adult medulloblastoma: an analysis of survival and prognostic factors

PURPOSE: This analysis aimed to review the experience in the management of adult medulloblastoma at the University of California, San Francisco, and to identify important prognostic factors for survival and posterior fossa control. PATIENTS AND METHODS: We performed a retrospective review of 34 adult patients, age > or = 15, with cerebellar medulloblastoma treated with radiotherapy at the University of California, San Francisco from 1970 to 1994. All patients underwent a surgical procedure (complete resection in 17, subtotal resection in 10, and biopsy alone in seven), followed by craniospinal irradiation. Most patients treated after 1979 also received chemotherapy. Twenty were classified as poor-risk due to either incomplete resection or evidence of disease outside of the posterior fossa at diagnosis. RESULTS: The 5-year posterior fossa control and overall survival rates were 61% and 58%, respectively. The majority of relapses occurred in the posterior fossa (14 of 17). Multivariate analysis revealed that age (favoring older patients), gender (favoring female patients), and extent of disease at diagnosis (favoring localized disease) were important prognostic factors for posterior fossa control. There was a trend toward improved posterior fossa control with higher radiation dose to the posterior fossa in patients with a complete resection. Gender and extent of disease at presentation were significant prognostic factors for survival. The 5-year survival rates were 92% for female patients versus 40% for male patients, and 67% for patients with localized disease versus 25% for those with disseminated disease. The prognosis following recurrence was poor; all died of the disease. DISCUSSION: Survival for adult medulloblastoma was comparable to its pediatric counterpart. In patients with localized disease at presentation, gender (favoring female patients) and age (favoring older patients) were important prognostic factors for posterior fossa control and survival. In patients with disseminated disease at presentation, the prognosis is poor, and innovative therapy is needed to improve survival.     

Cephalocranial disproportion. A complication of the treatment of hydrocephalus in children

Eight children with hydrocephalus showed signs of posterior fossa tonsillar impaction despite arrest of the hydrocephalus and patency of their CSF shunts. Contrast studies performed because of their clinical deterioration demonstrated cerebellar tonsillar herniation, and at posterior fossa decompression, this was confirmed in all cases. Improvement followed decompression. The pathogenesis of a state of cephalocranial disproportion following successful treatment of hydrocephalus resulting in symptomatic tonsillar herniation, is discussed.     

Thyroid function in lead smelter workers: absence of subacute or cumulative effects with moderate lead burdens

The electroencephalographic abnormalities seen in Landau-Kleffner syndrome (LKS) (language deterioration) are non-specific, and consist of a variety of epileptiform discharge patterns including continuous slow spike-wave discharges during sleep, focal sharp waves with spikes, and centrotemporal (rolandic) spikes. Similarly, the EEG abnormalities seen in autistic epileptiform regression (language and social/behavioral deterioration) are non-specific and overlap with those seen in LKS. By contrast, distinct epilepsy syndromes in otherwise normal children occur in the EEG-defined benign focal epilepsies of childhood. Occipital spikes or spike-wave present either in the older child with visual symptoms and headache or in the younger child with autonomic symptoms followed by brief or prolonged partial motor seizures. Seven young children (five from a consecutive series of 42) presenting clinically with autism or autistic regression and possible or definite seizures, whose EEGs revealed occipital spikes or spike-wave characteristic of the benign epilepsies, are reported. Although occipital spikes are commonly seen in young children as an age-dependent EEG-defined benign focal epilepsy, their high frequency in this population with cognitive difficulties suggests a possible causal relation. The effects of the epileptiform discharge on cognitive functioning presumably reflect extension into temporal and parietal lobes, rather than occipital disturbances per se.