Anodal Direct Current Stimulation of the Cerebellum Reduces Cerebellar Brain Inhibition but Does Not Influence Afferent Input from the Hand or Face in Healthy Adults

Background

The cerebellum controls descending motor commands by outputs to primary motor cortex (M1) and the brainstem in response to sensory feedback. The cerebellum may also modulate afferent input en route to M1 and the brainstem.

Objective

The objective of this study is to determine if anodal transcranial direct current stimulation (tDCS) to the cerebellum influences cerebellar brain inhibition (CBI), short afferent inhibition (SAI) and trigeminal reflexes (TRs) in healthy adults.

Methods

Data from two studies evaluating effects of cerebellar anodal and sham tDCS are presented. The first study used a twin coil transcranial magnetic stimulation (TMS) protocol to investigate CBI and combined TMS and cutaneous stimulation of the digit to assess SAI. The second study evaluated effects on trigemino-cervical and trigemino-masseter reflexes using peripheral nerve stimulation of the face.

Results

Fourteen right-handed healthy adults participated in experiment 1. CBI was observed at baseline and was reduced by anodal cerebellar DCS only (P?<?0.01). There was SAI at interstimulus intervals of 25 and 30 ms at baseline (both P?<?0.0001), but cerebellar tDCS had no effect. Thirteen right-handed healthy adults participated in experiment 2. Inhibitory reflexes were evoked in the ipsilateral masseter and sternocleidomastoid muscles. There was no effect of cerebellar DCS on either reflex.

Conclusions

Anodal DCS reduced CBI but did not change SAI or TRs in healthy adults. These results require confirmation in individuals with neurological impairment.

     

Endoscopic third ventriculostomy for hydrocephalus in brainstem glioma: a case series

Object

A brainstem glioma is an incurable brain tumor that can be complicated by hydrocephalus. A ventriculoperitoneal (VP) shunt is generally performed for the control of hydrocephalus. This study aimed to reveal the safety and efficacy of an endoscopic third ventriculostomy (ETV) for hydrocephalus in brainstem gliomas.

Methods

Six patients who had pontine glioma with hydrocephalus underwent an ETV between May 2010 and November 2015. In all the cases, there were one or more symptoms of hydrocephalus (headache, nausea, vomiting, or lethargy). Retrospective review of these patients was performed using the medical records and neuroimagings.

Result

The ETV was performed safely and there were no intraoperative complications in all patients. The mean follow-up period was 12.3 months. An immediate symptomatic relief of hydrocephalus and an adequate control of symptoms were achieved without a VP shunt in all patients.

Conclusions

The ETV is considered to be an effective and safe procedure for the treatment of hydrocephalus in brainstem gliomas. Determining the ventriculostomy site according to the preoperative MRI in each case is considered to be important for the safe procedure.

     

MRI Detection of Cerebral Infarction in Subarachnoid Hemorrhage

Objective

To investigate magnetic resonance imaging (MRI) detection of cerebral infarction (CI) in patients presenting with subarachnoid hemorrhage (SAH).

Background

CI is a well-known complication of SAH that is typically detected on computed tomography (CT). MRI has improved sensitivity for acute CI over CT, particularly with multiple, small, or asymptomatic lesions.

Methods

With IRB approval, 400 consecutive SAH patients admitted to our institution from August 2006 to March 2011 were retrospectively reviewed. Traumatic SAH and secondary SAH were excluded. Data were collected on demographics, cause of SAH, Hunt Hess and World Federation of Neurosurgical Societies grades, and neuroimaging results. MRIs were categorized by CI pattern as single cortical (SC), single deep (SD), multiple cortical (MC), multiple deep (MD), and multiple cortical and deep (MCD).

Results

Among 123 (30.8 %) SAH patients who underwent MRIs during their hospitalization, 64 (52 %) demonstrated acute CI. The mean time from hospital admission to MRI was 5.7 days (range 0–29 days). Among the 64 patients with MRI infarcts, MRI CI pattern was as follows: MC in 20 (31 %), MCD in 18 (28 %), SC in 16 (25 %), SD in 3 (5 %), MD in 2 (3 %), and 5 (8 %) did not have images available for review. Most infarcts detected on MRI (39/64 or 61 %) were not visible on CT.

Conclusions

The use of MRI increases the detection of CI in SAH. Unlike CT studies, MRI-detected CI in SAH tends to involve multiple vascular territories. Studies that rely on CT may underestimate the burden of CI after SAH.

     

Urological dysfunction in synucleinopathies: epidemiology,pathophysiology and management

Objective

Parkinson’s disease (PD) and multiple system atrophy (MSA) are major neurogenerative diseases characterized pathologically by abnormal alpha-synuclein aggregation. PD and MSA are clinically characterized by motor disorder and bladder dysfunction (mainly urinary urgency and frequency, also called overactive bladder). However, few literatures are available concerning bladder dysfunction in PD or MSA.

Method

A systematic review.

Results

The bladder dysfunction in MSA is more severe than that in PD for large post-void residual or urinary retention. These bladder dysfunctions presumably reflect the different nervous system pathologies. Overactive bladder in PD reflects lesions in the brain, e.g., in the prefrontal-nigrostriatal D1 dopaminergic bladder-inhibitory pathway. Overactive bladder in MSA reflects lesions similar to PD and the cerebellum (bladder-inhibitory), and the urinary retention in MSA presumably reflects lesions in the pontine micturition center and the sacral intermediolateral nucleus of the spinal cord (bladder-facilitatory). Bladder dysfunction not only impairs an individual’s quality of life, it can also cause emergency hospitalizations due to acute retention and early institutionalization. Anticholinergics are the first-line treatment for bladder dysfunction in PD and MSA patients, but care should be taken for the management of bladder dysfunction—particularly in MSA patients due to the high prevalence of difficult emptying, which needs clean, intermittent catheterization.

Conclusions

This review summarizes the epidemiology, pathophysiology, and management of bladder dysfunction in individuals with PD or MSA.

     

Tonsillar contusion associated with benign tonsillar ectopia following minor head trauma

Purpose

The relationship between tonsil position and symptomatic cerebellar contusion is unclear. To date, there are no reports of symptomatic traumatic brain injury associated with benign tonsillar ectopia. Reported cases are limited to prominent cerebellar tonsillar displacement by ≥5 mm (i.e., Chiari malformations).

Methods

The authors describe a case of symptomatic concussion in a toddler with unusual computerized tomography (CT) presentation and incidental finding of benign tonsillar ectopia, hemorrhagic contusion of the tonsils, blood-brain barrier (BBB) disruption and delayed atrophy shown using magnetic resonance imaging (MRI) studies. The radiological presentation and the clinical challenges are discussed through a review of the literature.

Conclusion

This case suggests that damage to cerebellar structures is not limited only to overt tonsillar herniation. Benign tonsillar ectopia may predispose to cerebellar contusion even after minor concussion and thus has a clinical significance. The current paradigm viewing only noticeable tonsillar herniation as a risk factor for hindbrain injury should be revisited.

     

Dobutamine,a β1 Adrenoceptor Agonist,Increases Cerebral Oxygenation During Acute Anemia and Apneic Hypoxia

Background

Refractory status epilepticus (RSE) is associated with high morbidity and mortality. Experts recommend aggressive management with continuous intravenous infusions or inhaled anesthetics such as isoflurane. However, there is concern that MRI changes in RSE reflect isoflurane neurotoxicity. We performed a case–control study to determine whether isoflurane is neurotoxic, based on MRI signal changes.

Methods

We performed a retrospective case–control study of the incidence of MRI changes in RSE treated with and without isoflurane. Charts were reviewed for demographic and treatment information. T1, T2, and FLAIR sequences of MRIs were reviewed independently by two neuroradiologists blinded to treatment group for presence or absence of signal change or atrophy in the meninges, cortex, white matter, basal ganglia, thalamus, hippocampus, brainstem, and cerebellum.

Results

Eight cases of RSE receiving treatment with isoflurane were identified and double-matched with 15 controls who received only intravenous anesthetics. Baseline characteristics were similar. Hippocampal signal change was observed more frequently in cases receiving isoflurane (p = 0.026).

Conclusions

Hippocampal signal changes were associated with isoflurane use in patients with RSE. They were also associated with number of seizure days prior to MRI and the use of multiple anesthetic agents. Similar changes have been seen as a result of RSE itself, and one cannot rule out the possibility these changes represent seizure-related effects. If isoflurane-related, these hippocampal signal changes may be the result of a direct neurotoxic effect of prolonged isoflurane use or failure of isoflurane to protect the hippocampus from seizure-induced injury despite achieving electrographic burst-suppression.

     

Onset of bladder and motor symptoms in multiple system atrophy: differences according to phenotype

Purpose

To compare the order of presentation of bladder and motor symptoms between multiple system atrophy phenotypes.

Methods

Medical records were retrospectively reviewed in 144 patients.

Results

Bladder symptoms occurred either before or within 12 months after onset of motor symptoms in significantly more patients with the cerebellar phenotype than the parkinsonian phenotype (80 vs. 53%, p = 0.003); similar results were observed for urinary incontinence (79 vs. 45%, p = 0.001).

Conclusions

Urinary dysfunction is more likely to appear either before or shortly after motor symptoms in the cerebellar phenotype than in the parkinsonian phenotype.

     

Sagittal MRI often overestimates the degree of cerebellar tonsillar ectopia: a potential for misdiagnosis of the Chiari I malformation

Background

We hypothesized that by using coronal MRI, Chiari I malformation could be more precisely diagnosed, would provide simple anatomic landmarks, would provide information regarding asymmetry of hindbrain herniation, and would be a better method for analyzing the tonsillar herniation postoperatively when the opisthion has been removed.

Methods

Fifty consecutive pediatric patients diagnosed with Chiari I malformation had comparison between the measurements of their caudally descended cerebellar tonsils on midsagittal and coronal MRI images.

Results

On MRI coronal imaging, tonsillar asymmetry was found in 48 patients. Maximal left tonsillar descent was 20.9 mm, and maximal right tonsillar descent was 17.4 mm. On MRI sagittal imaging, tonsillar descent ranged from 5 to 27.4 mm. Fifty-eight % of patients had syringomyelia. Five patients (10 %) on coronal MRI were found to have both cerebellar tonsils that were less than 3 mm below the foramen magnum. However, all of these patients had greater than 3 mm of tonsillar ectopia on sagittal imaging. Nineteen patients (38 %) on coronal MRI were found to have one of the cerebellar tonsils that were less than 3 mm below the foramen magnum. Similarly, each of these had greater than 3 mm of tonsillar ecotpia as measured on midsagittal MRI. Also, based on these findings, Chiari I malformation is almost always an asymmetrical tonsillar ectopia.

Conclusions

Sagittal MRI overestimates the degree of tonsillar ectopia in patients with Chiari I malformation. Misdiagnosis may occur if sagittal imaging alone is used. The cerebellar tonsils are paramedian structures, and this should be kept in mind when interpreting midline sagittal MRI.

     

Devastating Delayed Leukoencephalopathy Associated with Bath Salt Inhalation

Background

“Bath salts” or synthetic cathinone toxicity remains a potentially deadly clinical condition. We report a delayed leukoencephalopathy with persistent minimally conscious state.

Methods

Case report.

Results

A 36-year-old man presents with delayed encephalopathy, dysautonomia, fulminant hepatic failure, and renal failure from severe rhabdomyolysis after consuming bath salts. MRI showed diffusion restriction in the splenium of the corpus callosum and subcortical white matter.

Conclusions

The combination of acute leukoencephalopathy, rhabdomyolysis and fulminant hepatic failure may point to bath salt inhalation and should be known to neurointensivists.

     

Early Withdrawal Decision-Making in Patients with Coma After Cardiac Arrest: A Qualitative Study of Intensive Care Clinicians

Background

Crossed cerebellar diaschisis is a rare finding of hemispheric cerebellar depression following contralateral cerebral injury, hypothesized to result from excessive neuronal excitatory synaptic activity along cortico-pontine-cerebellar pathways. The phenomenon is typically observed following ischemic stroke, but has also been characterized during seizure activity—in particular, status epilepticus (SE). Neurological outcome has varied widely in published reports, with some patients achieving full neurologic recovery, while others experience persistent disability.

Methods

Case report and literature review.

Results

We present a 54-year-old man found unresponsive with a right hemispheric syndrome several days after discharge following amygdalohippocampectomy for refractory right temporal lobe epilepsy. Prolonged electroencephalogram demonstrated one subclinical right frontal seizure, along with right frontal periodic lateralized epileptiform discharges, presumed to be associated with SE preceding his admission. Initial MRI demonstrated restricted diffusion on diffusion weighted imaging in the right cerebral hemisphere, ipsilateral thalamus, and contralateral cerebellum. A head CT one week later showed diffuse sulcal effacement with loss of gray–white differentiation in the right frontal and insular regions with low attenuation changes of right thalamus. An MRI showed worsened diffusion restriction, despite a corresponding increase in perfusion. The patient remained paretic at discharge and follow-up. Follow-up MRI at 2 months demonstrated pronounced right cerebral and left cerebellar atrophy, loss of gray matter in much of the right cerebrum, and scattered areas of T2 hyperintensity, consistent with permanent right fronto-temporal neuronal loss.

Conclusions

Collectively, these observations indicate that imaging findings of persistent cerebral restricted diffusion and cytotoxic edema in the subacute post-ictal period may predict irreversible neuronal injury and poor long-term outcome—even when accompanied by evidence of cortical hyperperfusion and recovery of second- and third-order neurons along the involved circuit.

     

Spatial Disparities in the Distribution of Parks and Green Spaces in the USA

Background

Little national evidence is available on spatial disparities in distributions of parks and green spaces in the USA.

Purpose

This study examines ecological associations of spatial access to parks and green spaces with percentages of black, Hispanic, and low-income residents across the urban–rural continuum in the conterminous USA.

Methods

Census tract-level park and green space data were linked with data from the 2010 U.S. Census and 2006–2010 American Community Surveys. Linear mixed regression models were performed to examine these associations.

Results

Poverty levels were negatively associated with distances to parks and percentages of green spaces in urban/suburban areas while positively associated in rural areas. Percentages of blacks and Hispanics were in general negatively linked to distances to parks and green space coverage along the urban–rural spectrum.

Conclusions

Place-based race–ethnicity and poverty are important correlates of spatial access to parks and green spaces, but the associations vary across the urbanization levels.

     

The Relationship Between the Decreased Rate of Initial Blood Glucose and Neurologic Outcomes in Survivors of Out-of-Hospital Cardiac Arrest Receiving Therapeutic Hypothermia

Background

Hyperglycemia in out-of-hospital cardiac arrest (OHCA) survivors is associated with poor outcomes. However, in the control of initial hyperglycemia, an adequate strategy to improve patients’ neurologic outcomes remains undetermined. Prior to the establishment of such strategy, we need to determine whether a decreased rate of initial blood glucose (BG) affects patient outcomes.

Methods

One hundred and forty-five adult non-traumatic OHCA survivors treated with therapeutic hypothermia between April 2007 and December 2011 were enrolled in this single-center retrospective cohort study. Based on the cerebral performance category (CPC) at 6 months after OHCA, study populations were categorized as “Good CPC group” (favorable outcome, CPC1 and CPC2) and “Poor CPC group” (unfavorable outcome, CPC3–CPC5). Variables related to BG were obtained, and the rate of BG change was calculated.

Results

In the Good CPC group, the time required to attain target BG levels was shorter [7.4 (2.97–18.13) vs. 13.17 (7.55–27.0) h, p < 0.001], and the average rate of glucose decrease until the attainment of target BG levels was faster [17.06 (6.67–34.49) vs. 8.33 (4.26–18.55) mg/dl/h, p = 0.005] than in the Poor CPC group. Using multivariate analysis, the faster rate (odds ratio 1.074; 95% confidence interval 1.029–1.12; p = 0.001) and the shorter time (odds ratio 13.888; 95% confidence interval 2.271–84.906; p = 0.004) required to attain target BG levels were independently related to favorable neurologic outcomes.

Conclusions

Faster rates of initial BG decrease and the shorter time required to attain target BG levels were associated with favorable neurologic outcome in survivors of OHCA receiving therapeutic hypothermia.

     

Spectrum of abnormalities of sympathetic tyrosine hydroxylase and alpha-synuclein in chronic autonomic failure

Objective

Lewy body forms of primary chronic autonomic failure (CAF) such as incidental Lewy body disease (ILBD), Parkinson’s disease (PD), and pure autonomic failure evolving into dementia with Lewy bodies (PAF+DLB) feature cardiac sympathetic denervation, whereas multiple system atrophy (MSA) in most cases does not. What links Lewy bodies with cardiac sympathetic denervation in CAF? In familial PD, abnormalities of the alpha-synuclein (AS) gene cause CAF and cardiac sympathetic denervation; and in sporadic PD, brainstem Lewy bodies contain AS co-localized with tyrosine hydroxylase (TH), a marker of catecholaminergic neurons. Cytotoxicity from AS deposition within sympathetic neurons might explain noradrenergic denervation in Lewy body forms of CAF. We used immunofluorescence microscopy (IM) to explore this possibility in sympathetic ganglia obtained at autopsy from CAF patients.

Methods

Immunoreactive AS and TH were imaged in sympathetic ganglion tissue from 6 control subjects (2 with ILBD), 5 PD patients (1 with concurrent PSP), and 3 patients with CAF (2 PAF + DLB, 1 MSA).

Results

MSA involved normal ganglionic TH and no AS deposition. In ILBD TH was variably decreased, and TH and AS were co-localized in Lewy bodies. In PD TH was substantially decreased, and TH and AS were co-localized in Lewy bodies. In PAF + DLB TH was virtually absent, but AS was present in Lewy bodies. The PD + PSP patient had AS co-localized with tau but not TH.

Conclusions

Sympathetic denervation and intraneuronal AS deposition are correlated across CAF syndromes, consistent with a pathogenic contribution of synucleinopathy to cardiac noradrenergic deficiency in Lewy body diseases.

     

Physical Education and Student Activity: Evaluating Implementation of a New Policy in Los Angeles Public Schools

Background

California law has standards for physical education (PE) instruction in K-12 public schools; audits found that the Los Angeles Unified School District did not enforce the standards. In 2009, the district adopted a PE policy to comply with these standards.

Purpose

This study aimed to evaluate the outcomes of the PE policy in district schools.

Methods

PE class observations were conducted using the System for Observing Fitness Instruction Time in the 2010–2011 and 2011–2012 school years in an income-stratified random sample of 34 elementary, middle, and high schools to assess changes in PE class size, class duration, and time students spent in moderate to vigorous physical activity.

Results

PE class duration increased in high-income elementary schools. Mean class size decreased in low-income middle schools.

Conclusions

There was limited implementation of the PE policy 2 years after passage. Opportunities exist to continue monitoring and improving PE quantity and quality.

     

Do the cerebellar tonsils move during flexion and extension of the neck in patients with Chiari I malformation? A radiological study with clinical implications

Background

In the past, diagnosis of the Chiari I malformation has primarily been made on midsagittal MRI. We hypothesized that based on the frequent presentation of opisthotonos in patients with hindbrain hernia (primarily Chiari II malformation but sometimes Chiari I malformation) that the hyperextension might be a compensatory technique used by such patients to bring the cerebellar tonsils up out of the cervical spine.

Patients and methods

This prospective study reviewed imaging of patients with Chiari I malformation who underwent flexion/extension MRI for evaluation of their hindbrain herniation. Age-matched controls were used for comparison.

Results

In general, there was elevation of the cerebellar tonsils with extension and increased descent with flexion of the cervical spine. In 72 % of patients, flexion of the neck resulted in descent of the cerebellar tonsils. In 64 % of patients, extension of the neck resulted in ascent of the cerebellar tonsils. In the 14 patients with an associated syrinx, 71 % were found to have caudal movement of the cerebellar tonsils with neck flexion, and only 43 % were observed to have any movement of the cerebellar tonsils in neck extension compared to patients without a syrinx where ascent of the tonsils was seen in only nine during neck extension. Two patients were observed to have the reverse finding of ascent of the cerebellar tonsils with neck flexion and descent of the cerebellar tonsils with neck extension. Five patients had no movement of the cerebellar tonsils in either flexion or extension of the neck, and one of these had a small syrinx.

Conclusions

Although minimal and not in all patients, we observed elevation of the herniated cerebellar tonsils with extension of the cervical spine in patients with Chiari I malformation. This finding provides evidence as to why some patients with hindbrain herniation present with opisthotonos and supports earlier findings that CSF flow is reduced at the craniocervical junction in flexion in patients with Chiari I malformation.

     

A thoracic spinal bone giant cell tumor in a skeletally immature girl. A case report and literature review

Purpose

Giant cell tumors (GCT) are benign primary bone tumors, locally aggressive, affecting in long bones in young adults during the third decade. It is rare to experience this lesion in skeletally immature patients. GCT are related to a risk of local recurrence and malignant transformation.

Method

We report a rare case of a giant cell tumor of the thoracic spine in a skeletally immature girl presenting with a painful right scoliosis.

Results

MRI, CT scan, and bone scintigraphy were discordant and the percutaneous biopsy non-contributive.

Conclusion

A marginal “en bloc” resection was performed and revealed the GCT. Based on a literature review, the diagnosis and the surgical management of this case are discussed.

     

Evolution of cerebellar tonsillar ischemia to cerebellar tonsillar cysts in the Chiari I malformation: radiological,surgical, and histological evidence

Background

Rare reports in the literature have documented the presence of a cyst at the tip of the cerebellar tonsil in patients with Chiari I malformation. The current study aimed to better elucidate these cysts and identify their etiology via a histological and radiologically analysis.

Patients and methods

We prospectively reviewed all new Chiari I malformation (CM-I) patients who presented to our clinic during a 2-year period. This was a pediatric cohort made up of 340 children aged 3 to 18 years. Specifically, all patients were screened for ischemic or cystic lesions of the herniated cerebellar tonsils on MRI.

Results

Out of 340 patients, 2.9 % were found to have signal changes on MRI consistent with ischemia or cysts in the cerebellar tonsils. Of the 340 patients, 20 % underwent posterior fossa decompression. Of the 67 patients, cerebellar tonsillar ischemia was observed in 10.4 % and cerebellar tonsillar cysts were seen in 6 %. Four of the seven operative patients with cerebellar tonsillar ischemia and concomitant syringomyelia and three of these patients with tonsillar cysts concomitant syringomyelia and cerebellar tonsillar cysts underwent subpial dissection of some cerebellar tonsil to ensure that CSF egress from the fourth ventricle to the cervical subarachnoid space, and this transected tissue was also sent for histological analysis. Three of the four patients found to have intraoperative tonsillar cysts were noted to have tonsillar ischemic changes on preoperative imaging in this same region. For both ischemic and cystic cerebellar tonsils, histologically, the tissue demonstrated loss of Purkinje cells with concomitant Bergmann gliosis. The ischemic and cystic tissues were virtually the same, histologically.

Conclusions

Based on our findings, cerebellar tonsillar ischemia and cysts in patients with CM-I can often be seen radiologically. Histologically, these ischemic and cystic tissues are the same. Moreover, we document patients where ischemic lesions progressed to cysts, radiologically. Taken together, cerebellar tonsillar ischemia and cysts are on a continuum and represent chronic compression of this herniated part of the cerebellum.

     

Perceptions of Neighborhood Park Quality: Associations with Physical Activity and Body Mass Index

Background

Parks are important resources for physical activity (PA), yet few studies have examined how perceptions of park characteristics relate to PA and health.

Purpose

This study investigated associations between perceptions of neighborhood park quality and overall moderate-to-vigorous PA (MVPA), park-based PA, and body mass index (BMI).

Methods

Data were collected via questionnaire from 893 households in Kansas City, Missouri.

Results

The newly developed neighborhood park quality scale demonstrated good test–retest and internal reliability. Residents’ perceptions of neighborhood park quality were related to PA and health outcomes. Perceiving parks as a benefit was positively related to overall MVPA and park-based PA and negatively related to BMI. Perceptions of well-used parks were positively related to BMI, while perceived cleanliness was negatively related to park-based PA.

Conclusions

Better measuring and understanding how perceptions of local parks are associated with PA and health can improve appreciation of how parks facilitate active living.

     

Primary Posterior Fossa Lesions and Preserved Supratentorial Cerebral Blood Flow: Implications for Brain Death Determination

Background

Patients with primary posterior fossa catastrophic lesions may clinically meet brain death criteria, but may retain supratentorial brain function or blood flow. These patients could be declared brain-dead in the United Kingdom (UK), but not in the United States of America (USA). We report the outcome of adult patients with primary posterior fossa lesions without concurrent major supratentorial injury.

Methods

Henry Ford Hospital database was reviewed over a period of 88 months in order to identify all adult patients with isolated brainstem or posterior fossa lesions. We excluded patients with concurrent significant supratentorial pathology potentially confounding the clinical brain death examination. One more patient from a different hospital meeting these criteria was also included.

Results

Three patients out of 161 met inclusion criteria (1.9% of all brain deaths during this period). With the addition of a fourth patient from another hospital, 4 patients were analyzed. All four patients had catastrophic brainstem and cerebellar injuries meeting the clinical criteria of brain death with positive apnea test in the UK. All had preserved supratentorial blood flow, which after a period of 2 h to 6 days disappeared on repeat testing, allowing declaration of brain death by US criteria in all four. One patient became an organ donor.

Conclusions

Patients with primary posterior fossa catastrophic lesions, who clinically seem to be brain-dead, evolve from retaining to losing supratentorial blood flow. If absent cerebral blood flow is used as an additional criterion for the declaration of death by neurological criteria, these patients are not different than those who become brain death due to supratentorial lesions.

     

First symptoms in multiple system atrophy

Purpose

The initial symptoms of multiple system atrophy (MSA) and, in particular, early autonomic symptoms, have received less attention than motor symptoms. Whereas pathognomonic motor signs are essential to diagnostic specificity, early symptoms important to recognition of a neurodegenerative disorder may be less apparent or diagnostically ambiguous. This observational study sought to identify the very earliest symptoms in the natural history of MSA.

Methods

Detailed clinical histories focusing on early symptoms were obtained from 30 subjects recently diagnosed with MSA. Historical data were correlated with neurological examinations and laboratory autonomic testing.

Results

Subjects’ mean age was 63.9 years. Ten were classified as having MSA-P and 20 MSA-C. The evaluations occurred 2.9 ± 0.4 months after diagnosis. The first symptom of MSA was autonomic in 22 (73%) and motor in 3 (10%) subjects (p < 0.0001). The most frequent first symptom was erectile failure, which occurred in all men beginning 4.2 ± 2.6 years prior to diagnosis. After erectile failure, postural lightheadness or fatigue following exercise, urinary urgency or hesitancy, and violent dream enactment behavior consistent with REM behavioral sleep disorder were the most frequent initial symptoms. Neither the order of symptom progression, which was highly variable, nor autonomic severity scores differentiated between MSA-P and MSA-C.

Conclusions

The first symptoms of MSA are frequently autonomic and may predate recognition of motor manifestations. Orthostatic hypotension and, in men, erectile failure are among the first symptoms that, when evaluated in the context of associated clinical findings, may facilitate accurate and earlier diagnosis.