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PubMed2001

Reduced pre-movement facilitation of motor evoked potentials in spinocerebellar degeneration.

OBJECTIVES: The aim of this study was to clarify the cerebellar effects on the motor area of the cerebral cortex and abnormal control mechanisms of voluntary movement in spinocerebellar degeneration (SCD). We used transcranial magnetic stimulation (TMS) to study the change in the motor evoked potentials (MEPs) before voluntary movement (pre-movement facilitation) in patients with SCD. SUBJECTS AND METHODS: Pre-movement facilitation of MEPs in subjects' muscles was observed during their thumb movement intention. Patients with SCD, who showed cerebellar signs, without pyramidal or extrapyramidal signs, were examined. TMS was applied randomly during the interval between the "go" signal and the onset of voluntary EMG. The MEPs were recorded from the abductor brevis pollicis muscle. RESULTS: Patients with SCD showed a delay of task performance. In control subjects, the amplitude of MEPs was significantly facilitated (increased) prior to voluntary movement. In patients with SCD, pre-movement facilitation of the amplitude of MEPs was significantly decreased in the study with subthreshold TMS. CONCLUSIONS: Disturbance of pre-movement facilitation in SCD may indicate incomplete cerebellar regulation of voluntary movements.

Aged↗
PubMed1995

Lid opening apraxia is associated with medial frontal hypometabolism.

We used [18F]fluorodeoxyglucose with positron emission tomography to explore the functional basis of lid opening apraxia (LOA). We studied four patients with LOA without associated pyramidal or extrapyramidal motor dysfunction (mean age 65.5 +/- 4.7 years). We compared measures of glucose metabolism with those calculated for 10 age-matched normal volunteers (mean age 60.6 +/- 11.4). All four patients demonstrated significant metabolic reductions (> 2 SD) in the medial frontal lobe: bilaterally in two, unilaterally in two. Significant abnormal unilateral metabolic reductions in the left basal ganglia were evident in two patients. These findings suggest that medial frontal lobe hypometabolism is associated with LOA. Abnormal striatofrontal interactions may be involved in the pathogenesis of this condition.

Aged↗
PubMed2004

Laryngeal dystonia in a case of severe motor and intellectual disabilities due to Japanese encephalitis sequelae.

Laryngeal dystonia is characterized by stridor due to vocal cord dystonia and is observed in extrapyramidal disorders. Recently, botulinum toxin injection has been used as a primary therapy. Generally, severe motor and intellectual disabilities (SMID) are frequently complicated by various types of respiratory disorders. We report a SMID case with Japanese encephalitis sequelae showing repeated vocal cord abductor disturbance due to laryngeal dystonia, in addition to generalized dystonia, in whom MRI revealed basal ganglia lesions. Tracheostomy was effective for the case, and we believe that botulinum toxin injection may be inappropriate in SMID, both ethically and technically. Also, laryngeal dystonia should be considered as a cause of respiratory disorders in SMID.

Basal Ganglia↗
PubMed2005

Neuronal intranuclear inclusions are ultrastructurally and immunologically distinct from cytoplasmic inclusions of neuronal intermediate filament inclusion disease.

Abnormal neuronal cytoplasmic inclusions (NCIs) containing aggregates of alpha-internexin and the neurofilament (NF) subunits, NF-H, NF-M, and NF-L, are the signature lesions of neuronal intermediate filament (IF) inclusion disease (NIFID). The disease has a clinically heterogeneous phenotype, including frontotemporal dementia, pyramidal and extrapyramidal signs presenting at a young age. NCIs are variably ubiquitinated and about half of cases also have neuronal intranuclear inclusions (NIIs), which are also ubiquitinated. NIIs have been described in polyglutamine-repeat expansion diseases, where they are strongly ubiquitin immunoreactive. The fine structure of NIIs of NIFID has not previously been described. Therefore, to determine the ultrastructure of NIIs, immunoelectron microscopy was undertaken on NIFID cases and normal aged control brains. Our results indicate that the NIIs of NIFID are strongly ubiquitin immunoreactive. However, unlike NCIs which contain ubiquitin, alpha-internexin and NF epitopes, NIIs contain neither epitopes of alpha-internexin nor NF subunits. Neither NIIs nor NCIs were recognised by antibodies to expanded polyglutamine repeats. The NII of NIFID lacks a limiting membrane and contains straight filaments of 20 nm mean width (range 11-35 nm), while NCIs contain filaments with a mean width of 10 nm (range 5-18 nm; t-test, P<0.001). Biochemistry revealed no differences in neuronal IF protein mobilities between NIFID and normal brain tissue. Therefore, NIIs of NIFID contain filaments morphologically and immunologically distinct from those of NCIs, and both types of inclusion lack expanded polyglutamine tracts of the triplet-repeat expansion diseases. These observations indicate that abnormal protein aggregation follows separate pathways in different neuronal compartments of NIFID.

Adult↗
PubMed1999

[Leigh syndrome and leukodystrophy due to partial succinate dehydrogenase deficiency: regression with riboflavin].

UNLABELLED: Succinate dehydrogenase (SDH) deficiency is rare. Clinical manifestations can appear in infancy with a marked impairment of psychomotor development with pyramidal signs and extrapyramidal rigidity. CASE REPORT: A 10-month-old boy developed severe neurological features, evoking a Leigh syndrome; magnetic resonance imaging showed features of leukodystrophy. A deficiency in the complex II respiratory chain (succinate dehydrogenase [SDH]) was shown. The course was remarkable by the regression of neurological impairment under treatment by riboflavin. The delay of psychomotor development, mainly involving language, was moderate at the age of 5 years. CONCLUSION: The relatively good prognosis of this patient, despite severe initial neurological impairment, may be due to the partial enzyme deficiency and/or riboflavin administration.

Basal Ganglia Diseases↗
PubMed2001

The spectrum of postinfectious encephalomyelitis.

The medical records of 52 consecutive patients diagnosed with postinfectious encephalitis/encephalomyelitis during the period from 1980 to 1998, including 29 males and 23 females, were reviewed. These patients were divided into three groups according to their clinical and neurodiagnostic characteristics: (1) group I: postinfectious encephalitis, 38 patients; (2) group II: acute disseminated encephalomyelitis (ADEM), 13 patients; (3) group III: multiphasic disseminated encephalomyelitis (MDEM), one patient. Fever, headache/vomiting, seizure and disturbance of consciousness were common clinical features in all patients, while pictures of pyramidal, extrapyramidal, brainstem, and spinal cord lesions were more often found in the group II and group III patients than in the group I patients. Magnetic resonance imaging (MRI) revealed abnormal lesions in six (60%) of ten group I patients, but all group II (n=7) and group III (n=1) patients who received MRI study showed abnormal signals in various regions of the brain including the cerebral hemisphere, basal ganglia, brainstem and cerebellum. Patients with ADEM and MDEM had a longer clinical course and more neurological sequelae than group I patients. This study demonstrates the breadth of the clinical spectrum of postinfectious encephalomyelitis. Thorough clinical observations and appropriate neurodiagnostic studies such as MRI are crucial for the diagnosis.

Brain↗
PubMed1989

Human reticular formation: cholinergic neurons of the pedunculopontine and laterodorsal tegmental nuclei and some cytochemical comparisons to forebrain cholinergic neurons.

Choline acetyltransferase immunohistochemistry showed that the human rostral brainstem contained cholinergic neurons in the oculomotor, trochlear, and parabigeminal nuclei as well as within the reticular formation. The cholinergic neurons of the reticular formation were the most numerous and formed two intersecting constellations. One of these, designated Ch5, reached its peak density within the compact pedunculopontine nucleus but also extended into the regions through which the superior cerebellar peduncle and central tegmental tract course. The second constellation, designated Ch6, was centered around the laterodorsal tegmental nucleus and spread into the central gray and medial longitudinal fasciculus. There was considerable transmitter-related heterogeneity within the regions containing Ch5 and Ch6. In particular, Ch6 neurons were intermingled with catecholaminergic neurons belonging to the locus coeruleus complex. The lack of confinement within specifiable cytoarchitectonic boundaries and the transmitter heterogeneity justified the transmitter-specific Ch5 and Ch6 nomenclature for these two groups of cholinergic neurons. The cholinergic neurons in the nucleus basalis (Ch4) and those of the Ch5-Ch6 complex were both characterized by perikaryal heteromorphism and isodendritic arborizations. In addition to choline acetyltransferase, the cell bodies in both complexes also had high levels of acetylcholinesterase activity and nonphosphorylated neurofilament protein. However, there were also marked differences in cytochemical signature. For example, the Ch5-Ch6 neurons had high levels of NADPHd activity, whereas Ch4 neurons did not. On the other hand, the Ch4 neurons had high levels of NGF receptor protein, whereas those of Ch5-Ch6 did not. On the basis of animal experiments, it can be assumed that the Ch5 and Ch6 neurons provide the major cholinergic innervation of the human thalamus and that they participate in the neural circuitry of the reticular activating, limbic, and perhaps also extrapyramidal systems.

Adult↗
PubMedDate not supplied

Comparing the side effect profile of the atypical antipsychotics.

Typical antipsychotics exert their effect by blocking post-synaptic dopaminergic receptors; blockade of the mesolimbic and mesocortical pathways are therapeutic and help reduce positive psychotic symptoms but blockade of the nigro-striatal pathway produces extrapyramidal side effects (EPSE). Post clozapine, the Food and Drug Administration (FDA) has approved the use of four newer atypical antipsychotics; risperidone, olanzapine, quetiapine and ziprasidone for the treatment of schizophrenia. Because of their dual serotonin and dopamine receptor blocking abilities, atypical antipsychotics have greater efficacy (especially for negative symptoms) and fewer EPSE when compared to the typical antipsychotics. Given the lack of studies directly comparing these agents, we used the Physician Desk Reference (PDR) to calculate the treatment emergent placebo adjusted side effects for these atypical antipsychotics. The results are then presented in an easy to read table. To the best of our knowledge, this is the first comparison study involving these four newer antipsychotic agents.

Antipsychotic Agents↗
PubMed1995

[Multivariate analysis of spinal automatism in neurological diseases].

We investigated the relationship between spinal automatism (SA) and other neurological signs and symptoms (pyramidal, extrapyramidal, cerebellar, autonomic) by multivariate analysis in cervical spondylotic myelopathy (CSM) 112 cases, amyotrophic lateral sclerosis (ALS) 121 cases, and multiple system atrophy (MSA) 115 cases (olivo-ponto-cerebellar atrophy (OPCA) 55 cases, Shy-Drager syndrome (SDS) 42 cases, striato-nigral degeneration (SND) 18 cases). SA elicitation maneuver we used was pinprick stimulation at the dorsal pedal skin, and all cases showed twitched or phasic triple flexion pattern. As SA was elicited in 19 cases in CSM (17.0%), 23 cases (19.0%) in ALS, 36 cases (31.3%) in MSA, SA was more elicited in MSA than in ALS and CSM (p < 0.05, p < 0.01 by chi-squared test). In MSA, SA tended to be elicited more likely in SDS (21 cases, 50.0%) and in SND (8 cases, 44.4%) than in OPCA (7 cases, 12.7%) (p < 0.01 by chi-squared test). In MSA, the longer the duration of the disease became, the more SA was elicited, but not in ALS and CSM. Multivariate analysis (quantification method type II) showed that clinical signs as a statistical contribution factor for SA elicitation ranked Babinski's sign, micturition disturbance in ALS and CSM and Babinski's sign, orthostatic hypotension in MSA in order: In all 3 diseases, Babinski's sign and autonomic disturbance ranked higher. This conclusion suggested that SA was not always related to the pyramidal tract damage and was associated with the damage of small myelinated fibers in and/or around the pyramidal tract.

Adult↗
PubMed2006

Delineation of the motor disorder of Lesch-Nyhan disease.

Lesch-Nyhan disease (LND) is caused by deficiency of the purine salvage enzyme hypoxanthine-guanine phosphoribosyltransferase (HPRT). Affected individuals exhibit over-production of uric acid, along with a characteristic neurobehavioural syndrome that includes mental retardation, recurrent self-injurious behaviour and motor disability. Prior studies involving relatively small numbers of patients have provided different conclusions on the nature of the motor disorder. The current study includes the results of a multi-centre international prospective study of the motor disorder in the largest cohort of patients studied to date. A total of 44 patients ranging from 2 to 38 years presented a characteristic motor syndrome that involved severe action dystonia superimposed on baseline hypotonia. Although some patients also displayed other extrapyramidal or pyramidal signs, these were always less prominent than dystonia. These results are compared with a comprehensive review of 122 prior reports that included a total of 254 patients. Explanations for the differing observations available in the literature are provided, along with a summary of how the motor disorder of LND relates to current understanding of its pathophysiology involving the basal ganglia.

Adolescent↗
PubMed1992

Sigma receptors are associated with cortical limbic areas in the primate brain.

Putative sigma receptors are a current target for antipsychotic drug development. Novel antipsychotic agents which possess selective and high affinity for sigma binding sites may serve as an alternative to the principal neuroleptic drugs currently in clinical use which mediate extrapyramidal side effects and dyskinesias through their blockade of dopamine receptors. We have used in vitro autoradiography to localize putative sigma receptors labelled with (+)-[3H]-3-(3-hydroxyphenyl)-N-(1-propyl)piperidine [(+)-[3H]-3-PPP] in the brain of the rhesus macaque. The binding characteristics of (+)-[3H]-3-PPP in the primate brain were comparable to those previously described in the rodent. Saturation analysis demonstrated a single class of sites in cerebellar and hippocampal membranes with a Kd value of 28 nM. Sigma receptors labeled with (+)-[3H]-3-PPP in the primate brain displayed the appropriate rank order of potency and stereoselectivity in competition binding assays. Haloperidol displaced (+)-[3H]-3-PPP binding in the low nanomolar range, and the (+) isomer of pentazocine was 50-fold more potent than (-) pentazocine. Computerized densitometric analysis of the autoradiograms demonstrated a striking enrichment of sigma binding sites over the paralimbic belt cortices, including the orbitofrontal, cingulate, insular, parahippocampal, and temporopolar gyri. Peak densities of sigma receptors were seen over the medial and central nuclei of the amygdala and were widely distributed within the hippocampal formation. Sigma binding sites densities were elevated over the suprachiasmatic and supraoptic nuclei of the hypothalamus. Moderate sigma receptor densities were observed over the ventromedial sectors of the caudate and the putamen. Sigma receptors were also elevated over autonomic relay nuclei of the brainstem, including the nucleus of the solitary tract and the dorsal motor nucleus of the vagus. The distribution of sigma receptors in the primate brain suggests that the paralimbic belt cortices, amygdala, hippocampus, hypothalamus, and autonomic relay nuclei of the brainstem may be interrelated by a topographic chemical linkage. The autoradiographic visualization of sigma receptor distributions in the primate brain provides further support for a role of sigma receptor mechanisms in the functions of the limbic system.

Animals↗
PubMed1988

Cerebrospinal fluid monoamine markers are decreased in dementia of the Alzheimer type with extrapyramidal features.

We measured monoamine metabolites and biopterin in the CSF of 37 patients with dementia of the Alzheimer type (DAT), with or without extrapyramidal signs, and in 14 age-matched healthy controls. Compared with concentrations in DAT and controls, the concentrations of homovanillic acid (HVA) and biopterin were significantly decreased in DAT with extrapyramidal signs (EDAT). CSF 3-methoxy-4-hydroxy-phenethyleneglycol and 5-hydroxyindoleacetic acid did not differ significantly among these groups. Age at onset of dementia was positively correlated with CSF HVA (r = 0.49, p less than 0.05). The two dementia groups did not differ significantly in the extent of ventricular dilation as measured by quantitative CT, but EDAT patients had lower Mini-Mental State Examination scores than did DAT patients. When patients were matched for age and dementia severity, CSF HVA and biopterin concentrations remained significantly lower in EDAT than in DAT patients. These results indicate that EDAT patients form a distinct subgroup of DAT with evidence of central monoamine dysfunction.

Aged↗
PubMed1992

Responses of limbic and extrapyramidal neurotensin systems to stimulants of abuse. Involvement of dopaminergic mechanisms.

In summary, we have observed that drugs of abuse, which can cause schizophrenia-like paranoia, alter striatal and accumbens NT systems in a similar, dramatic fashion. The NT responses to these drugs, in particular METH, are mediated by activation of DA D1 receptors. We have observed that NMDA-type glutamate receptors are essential for the D1-NT interaction. NMDA receptors are selective, since they do not contribute to the antagonistic effects of DA D2 receptors on NT activity. This observation suggests that NT responses to D1 and D2 regulation are mediated through separate and distinct mechanisms. Finally, we found that the presence of METH dramatically reduces striatal NT release, which most likely leads to NT accumulation in nerve terminals and the observed increase in NT tissue level. The blockade of NT release by a psychotogenic drug, such as METH, is consistent with the hypothesis that NT has antipsychotic activity and a decrease in its release may contribute to some forms of schizophrenia similar to that caused by intense use of the stimulants of abuse.

Animals↗
PubMed1996

Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes.

We describe eight patients with slowly progressive speech production deficit combining speech apraxia, dysarthria, dysprosody and orofacial apraxia, and initially no other deficit in other language and non-language neuropsychological domains. Long-term follow-up (6-10 years) in 4 cases showed an evolution to muteness, bilateral suprabulbar paresis with automatic-voluntary dissociation and frontal lobe cognitive slowing without generalised intellectual deterioration. Most disabled patients presented with an anterior opercular syndrome (Foix-Chavany-Marie syndrome), and pyramidal or extrapyramidal signs. CT and MRI findings disclosed asymmetric (left > right) progressive cortical atrophy of the frontal lobes predominating in the posterior inferior frontal region, notably the operculum. SPECT and PET revealed a decreased cerebral blood flow and metabolism, prominent in the left posterior-inferior frontal gyrus and premotor cortex, extending bilaterally in the most advanced cases. Pathological study of two cases showed non-specific neuronal loss, gliosis, and spongiosis of superficial cortical layers, mainly confined to the frontal lobes, with no significant abnormalities in the basal ganglia, thalamus, cerebellum, brain stem (except severe neuronal loss in the substantia nigra in one case), and spinal cord. We propose to call this peculiar syndrome Slowly Progressive Anarthria (SPA), based on its specific clinical presentation, and its metabolic and pathological correlates. SPA represents another clinical expression of focal cortical degeneration syndromes, that may overlap with other similar syndromes, specially primary progressive aphasia and the various frontal lobe dementias.

Aged↗
PubMed1976

The relationship between striatal and mesolimbic dopamine dysfunction and the nature of circling responses following 6-hydroxydopamine and electrolytic lesions of the ascending dopamine systems of rat brain.

The importance of extrapyramidal and mesolimbic function for circling behaviour was investigated by placing 6-hydroxydopamine (6-OHDA) and electrolesions in the cell bodies, axons and terminals of each system. Circling behaviour was weak when 6-OHDA was placed at the centre of the substantia nigra (SN), but the characteristic contralateral/ipsilateral turning to apomorphine/amphetamine were recorded. Circling was more marked when 6-OHDA was placed anterior to the SN but was generally absent following injections posterior to the SN. However, 6-OHDA placed in the medial forebrain bundle in the lateral hypothalamus resulted in intense contralateral/ipsilateral turning to apomorphine/amphetamine. Generally, the intensity of circling responses was related to the degree of striatal dopamine (DA) depletion but the more effective lesions also caused reductions in mesolimbic DA content. However, circling was not observed following any 6-OHDA injection into the mesolimbic DA system and it is concluded that mesolimbic DA function is not essential for the initiation of circling. In contrast to the 6-OHDA lesions, rats circled ipsilateral to both apomorphine and amphetamine when the SN was damaged by electrocoagulation to cause marked depletion of striatal dopamine. Lesser depletions of striatal dopamine after electrocoagulation in different regions of the medial forebrain bundle were associated with a lower intensity of ipsilateral circling to both drugs. In general, the differences between 6-OHDA and electrolesions could not be explained by additional damage to ascending noradrenaline or 5-hydroxytryptamine pathways. Lower doses of apomorphine were effective in the 6-OHDA circling rats, and the ipsilateral striatum of such rats was more sensitive to directly applied DA. Higher doses of apomorphine were required to produce circling after chronic electrolesions which rendered the ipsilateral striatum insensitive to DA. The contralateral circling to apomorphine after 6-OHDA lesions was abolished by chronic but not by acute electrolesion of the SN. It is suggested that electrolesions of the SN cause different effects to 6-OHDA because they destroy neuronal pathways in addition to the dopaminergic nigrostriatal tract. These appear to be required for the expression of circling behaviour caused by stimulation of the denervated striatum. Whereas 6-OHDA lesions result in super-sensitivity of the denervated strital DA receptors, electrolesions may cause a hypo-sensitivity of the same receptor sites.

Afferent Pathways↗
PubMed1996

Obstructive and restrictive pulmonary dysfunction increases disability in Parkinson disease.

OBJECTIVE: The purpose of this study was to determine in Parkinson disease the impact of pulmonary dysfunction on daily living activities (DLA). PATIENTS: Extrapyramidal motor impairment, pulmonary dysfunction, and DLA disabilities were studied in 58 Parkinson patients consecutively enrolled in a rehabilitation service at a university hospital. MAIN OUTCOME MEASURES: Extrapyramidal motor impairments were assessed by the Unified Parkinson's Disease Rating Scale (UPDRS) and the DLA disabilities by the UPDRS, Hoehn-Yahr, and Schwab-England scales. The pulmonary dysfunctions were assessed by spirometry with flow-volume loops, body plethysmography with lung volumes computation, and maximal inspiratory and expiratory static mouth pressures. RESULTS: Parkinson patients showed important modifications of pulmonary function with a decrease in forced vital capacity, forced expiratory volume in the first minute, and arterial PO2, and an increase in residual volume and total airway resistance (RAW). In addition, they showed a high incidence of airway ventilatory obstructions and restrictive dysfunction. The impact of lung disease on daily living activities in Parkinson disease patients was higher in subjects with restrictive pulmonary dysfunctions (Schwab-England test and turning in bed and adjusting bedclothes, walking, falling, and freezing when walking items of UPDRS) and airway obstructions (handling utensils, dressing and hygiene items of UPDRS). CONCLUSIONS: Airway obstructions or restrictive pulmonary dysfunctions present a high prevalence in Parkinson disease, contributing as a main factor for DLA dysfunctions. The evaluation and rehabilitation of respiratory disturbances should be systematically included in the management of these patients.

Activities of Daily Living↗
PubMed1997

Mortality in current and former users of clozapine.

Clozapine (Clozaril), a tricyclic dibenzodiazepine, causes fewer extrapyramidal side effects than do other antipsychotic drugs. Because it can induce agranulocytosis, however, clozapine is indicated only for schizophrenia that is not responsive to other therapies. To describe the drug's effects on mortality, we compared rates of various causes of death in 67,072 current and former clozapine users. We linked data from a national registry of clozapine recipients to the National Death Index and Social Security Administration Death Master Files, obtained death certificates, and calculated mortality rates for underlying causes of death using standardization to adjust for age, sex, and race. During 1991-1993, there were 396 deaths in 85,399 person-years for patients ages 10-54 years. Mortality was lower during current clozapine use than during periods of non-use. Mortality from suicide was decreased in current clozapine users by comparison with past users [rate ratio (RR) = 0.17; 95% confidence interval (CI) = 0.10-0.30]. During clozapine use, there were elevations in mortality rates for less common causes of death, including pulmonary embolism (RR for current exposure compared with past clozapine use = 5.2) and respiratory disorders (RR = 2.9). Clozapine appears to reduce mortality in severe schizophrenics, mostly by decreasing suicide rates.

Adolescent↗