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PubMed2004

Quercetin, a bioflavonoid, reverses haloperidol-induced catalepsy.

Neuroleptics are extensively used in the treatment of schizophrenia and other affective disorders. Unfortunately their use is often associated with distressing side effects involving the extrapyramidal tract, such as Parkinsonism and tardive dyskinesia. Neuroleptic-induced catalepsy has long been used as a model for extrapyramidal side effects such as Parkinsonian-like bradykinesia associated with antipsychotic use in humans. In the present study, haloperidol (2 mg/kg i.p.) was administered to mice to induce catalepsy and cataleptic response was measured for the next 4 h at 1 h intervals. Haloperidol treatment in mice induced a strong cataleptic state within 1 h of injection, reaching a maximal plateau after 2 h and lasting for 4 h. Vehicle-treated animals did not display catalepsy as they remained less than 5 sec on the bar at each time point. Pretreatment with quercetin dose-dependently (25-100 mg/kg p.o.) reduced the catalepsy score in haloperidol-treated animals. A dose response study of quercetin showed a characteristic U-shaped response curve, with maximum suppression observed at a dose of 100 mg/kg. On increasing the dose of quercetin to 300 mg/kg, no statistically significant difference in catalepsy score was observed compared with the haloperidol-treated group. In conclusion, the findings of the present study strongly suggest that quercetin can be screened as a potential drug candidate or as an adjuvant for the treatment of neuroleptic-induced extrapyramidal side effects.

Animals↗
PubMed1976

[The ultrastructure of portions of the cat spinal cord gray matter connected to fibers of different descending tracts].

Definite ultrastructural features were found in the regions of the cat spinal grey matter connected with different descending pathways, especially in the structure of dendritic membranes and relative quantities of axon terminals. Two types of destructive changes in myelinated axons and axon terminals in these regions were observed during experimental degeneration of descending fibres. Axon terminals of small size and F-type vesicles were subjected to degeneration after pyramidal tract lesion, while large terminals of both S-type and F-type were affected after sectioning of extrapyramidal tracts.

Animals↗
PubMedDate not supplied

[Strümpell Lorrain's familial spasmodic paraplegia. An anatomical and clinical review and report on a new case (author's transl)].

The authors describe the anatomical and clinical findings in a case of the pure form of Strümpell Lorrain's familial spasmodic paraplegia. This hereditary condition of the dominant autosomic type has very monomorphic pathognomonic features which are found in all patients affected. The slowly progressive isolated spastic hypertonia appears exclusively in the upright position and the extent of the disease can be ascertained by clinical observation during walking. The myelino-axial degeneration which is strictly limited to the spinal cord has an elective localization in the crossed pyramidal tracts, is less evident in the direct tracts and is mainly predominant in the lumbosacral and low dorsal regions of the cord. The extension of the process beyond the limits of the extrapyramidal tracts confirms the generally accepted unity of spinocerebellar degenerations. In this case the spinocerebellar tracts were less involved than the lumbar spinal ganglia, as well as their distant axial continuation to Goll's tracts only at the cervical level and until the bulber nucleus gracilis.

Brain↗
PubMed2000

Neurologic manifestations of Mycoplasma pneumoniae infections: diverse spectrum of diseases. A report of six cases and review of the literature.

Mycoplasma pneumoniae is a common cause of upper and lower respiratory tract infections of varying severity. It is also responsible for producing a wide spectrum of nonpulmonary manifestations including neurologic, hepatic, cardiac, and hematologic diseases. The neurologic manifestations are reported to be the most common nonpulmonary manifestations. We describe six patients demonstrating the protean neurologic manifestations of Mycoplasma pneumoniae infections. Four patients presented with the central nervous system manifestations of pyramidal and extrapyramidal tract dysfunction, seizures, cognitive abnormalities, and cerebellar dysfunction. Two patients presented with transverse myelitis. The outcome of this condition ranges from normal to severe residual deficits. Increased awareness of this disease entity may facilitate early diagnosis and thereby expedite starting appropriate therapy that may modify the outcome.

Adolescent↗
PubMed1994

3-Methylglutaconic aciduria: ten new cases with a possible new phenotype.

3-Methylglutaconic aciduria is an organic aciduria with diverse phenotypic presentations. In more than half of the cases it is a 'neurologic or silent organic aciduria', and, except for one subtype, the biochemical defect is unknown. This report describes 10 new patients. Four of them presented with early global neurologic involvement and arrested development. They rapidly became demented, developed myoclonus or tonic-clonic seizures, spastic quadriplegia, deafness and blindness, and died. Three had acidosis and hypoglycemia neonatally; later, myoclonus and deafness, and eventually severe mental retardation and spastic quadriplegia developed. One patient died. In three children who presented with sudden onset of extrapyramidal tract symptoms, with or without optic atrophy, the clinical presentation was significantly different from that described either for 'unspecified' type or for Costeff syndrome. All three patients showed clinical improvement soon after treatment with coenzyme Q.

Acidosis↗
PubMed2004

Immediate plasticity in the motor pathways after spinal cord hemisection: implications for transcranial magnetic motor-evoked potentials.

The present study evaluates motor functional recovery after C2 spinal cord hemisection with or without contralateral brachial root transection, which causes a condition that is similar to the crossed phrenic phenomenon on rats. Descending motor pathways, including the reticulospinal extrapyramidal tract and corticospinal pyramidal tracts, were evaluated by transcranial magnetic motor-evoked potentials (mMEPs) and direct cortical electrical motor-evoked potentials (eMEP), respectively. All MEPs recorded from the left forelimb were abolished immediately after the left C2 hemisection. Left mMEPs recovered dramatically immediately after contralateral right brachial root transection. Corticospinal eMEPs never recovered, regardless of transection. The facilitation of mMEPs in animals that had undergone combined contralateral root transection was well correlated with open-field behavioral motor performance. Both electrophysiological and neurological facilitations were significantly attenuated by the selective serotonin synthesis inhibitor para-chlorophenylalanine (p-CPA). These results suggest that serotonergic reticulospinal fibers located contralateral to hemisection contribute to the behavioral and electrophysiological improvement that immediately follows spinal cord injury (SCI).

Animals↗
PubMed2001

Neurophysiological evidence of spared upper motor conduction fibers in clinically complete spinal cord injury: discomplete SCI in rats.

Motor evoked potentials (MEP) were recorded and characterized by epidural electrodes (scMEP) and extracellular microelectrodes (exMEP) on T(13) level from 10 normal rats and 40 rats with chronic spinal cord injury (SCI). The spinal cord of 40 anesthetized rats were injured with various severity (sham, 35, 70, and 100 g/cm impact injury) at T(8)-T(9) cord using Allen's drop model. The incline plane and Tarlov techniques were investigated to assess clinical neurological function. MEPs in the normal rats elicited by applying transcortical suprathreshold stimulation consisted of 3-4 early negative peaks (N(1), N(2), N(3), N(4)) followed by several late waves. The N(1) and N(2) peaks had their maximal amplitudes in the anterior and ventrolateral funiculus, respectively, irrespective of the polarity of stimulation, which indicated that these impulses were conducted mostly through the extrapyramidal pathways. The 100 g/cm impact injury or transection of the cord caused abolishment of the MEP signals distal to the lesion, whereas the 35 g/cm injury resulted in a latency shift and amplitude decrement of the MEP peaks. Out of 20 rats with 70 g/cm injuries, 18 showed clinically paraplegia. Among them, seven had neurophysiological evidence of residual conduction pathways through the injured cord segment, such as the presence of N(1) and N(2) peaks in scMEP or exMEP. After 4-aminopyridine (4-AP) administration (1 mg/kg), the amplitude of spared exMEP increased significantly and spread more widely. These results suggest that MEPs evoked by transcortical stimulation travel mostly in the extrapyramid tract. The present study provides further direct and objective electrophysiological evidences of spared functional axons after discomplete SCI, since many other studies on this field have achieved similar results previously. Furthermore, pharmaceutical treatment with 4-AP or other K(+) channel blocking agents proved to be a potential therapeutic strategy for patient with chronic SCI.

Animals↗
PubMed2002

Information provided by the skeletal muscle and associated neurons is necessary for proper brain development.

Previously, motor cortex of term Myf5(-/-):MyoD(-/-) fetuses (e.g. have ablated skeletal myogenesis and consequent early loss of lower motor and proprioceptive neurons) was found to lack giant pyramidal cells. We further investigated how the absence of the extrinsic stimuli from the lacking structures influences brain development. Apparently normal motor cortex of mutant fetuses was found to have dramatically reduced presence of nestin-expressing processes of neural precursors, calretinin-expressing pyramidal neurons and calbindin-expressing neurons. Consistently, some areas of the extrapyramidal tract had significantly decreased number of differentiated neurons in mutant brains. Surprisingly, we were unable to detect any change in proliferation or cell death in the mutant neuroepithelium. Together, it appears that the information provided by the lacking structures influences the ratios of the differentiated neuronal types and their progenitor cells.

Animals↗
PubMed2003

Progressive neurodegenerative disease in presumed spinal cord injury: case report of a patient with prion disease.

BACKGROUND: Prion diseases or transmissible spongiform encephalopathies (TSEs) are neurodegenerative syndromes caused by proteinaceous infectious particles (or "prions"), are fatally progressive, and affect humans and animals. Human prion disease may be familial, sporadic, or due to iatrogenic causes. The signs and symptoms include dementia, ataxia, myoclonus, dysautonomia, pyramidal and extrapyramidal tract signs, and akinesia. The incubation period of iatrogenic TSE ranges from 15 months to 30 years, and clinical presentations may be atypical. DESIGN: Case report. FINDINGS: This article presents the case study of a 39-year-old man who fell at work and subsequently complained of subjective lower extremity weakness, followed by onset of ataxia, bowel and bladder incontinence, and progressive decline in ambulation over 6 months. In the absence of a unifying diagnosis, the patient was presumed to have had a spinal cord injury (SCI). Because neuro-axis imaging studies failed to explain his symptoms, the patient's complaints were thought to have a large psychologic component. The patient then developed neurologic abnormalities proximal to the presumed SCI. Somatosensory evoked potentials were suggestive of a thoracic or lumbar cord myelopathy and cerebrospinal fluid analysis was suggestive of prion disease. Family members eventually revealed that the patient had had injections of growth hormones derived from cadaveric human pituitary glands as a child. Postmortem brain examination later revealed definitive Creutzfeldt-Jakob disease.

Adult↗
PubMed1989

Motor evoked potential monitoring during upper cervical spine surgery.

Motor evoked potential (MEP) produced by transcranial electrical stimulation was recorded from an epidural electrode in 20 consecutive patients during upper cervical spine surgery. In 5 patients, transient attenuation to approximately 50% followed by complete recovery was observed, and no neurologic deficit was noted. One patient had complete loss of MEP and was left a respiratory quadriplegic. In 2 cases, MEP amplitudes increased after tumor extirpation and remarkable remissions were observed. The MEP correlated with clinical outcomes and was a useful monitoring technique for upper cervical spine surgery, free of complication. In cat experiments designed to analyze conducting pathways, the maximal amplitude of the initial spike of MEP existed in the ventromedial spinal cord, which contains the extrapyramidal tracts. Motor evoked potential was proven to reflect motor function based on the spinal cord compression study.

Animals↗
PubMed2004

The effects of occupational exposure to chlorpyrifos on the neurologic examination of central nervous system function: a prospective cohort study.

Questions persist about adverse effects such as impaired cognition and attention, incoordination, spasticity, or parkinsonism from chronic, low-level exposures to organophosphate (OP) compounds. In a prospective cohort study, we evaluated chlorpyrifos-manufacturing workers and a referent group on 2 occasions, 1 year apart, to determine whether occupational exposure to chlorpyrifos produced clinically evident central nervous system (CNS) dysfunction. Chlorpyrifos subjects had significantly higher TCP excretion and lower average BuChE activity than referents in a range in which physiological effects on B-esterases exist. Few subjects had neurologic symptoms or signs, and there were no significant group differences in terms of signs at baseline or second examinations. Chronic chlorpyrifos exposure produced no clinical evidence of cortical, pyramidal tract, extrapyramidal, or other CNS dysfunction among chlorpyrifos subjects compared with referents, either at baseline or after 1 year of additional chlorpyrifos exposure.

Adult↗
PubMed1992

Neuroradiological findings in glutaric aciduria type I: report of four Japanese patients.

We examined neuroradiological computerized tomography (CT) findings and the clinical course of four Japanese children with glutaric aciduria type I (GA1) whose enzyme activity of glutaryl-CoA dehydrogenase was undetectable. Brain CT in all cases examined showed low density white matter, fluid collection in bilateral frontotemporal regions (particularly surrounding the Sylvian fissures), enlargement of the lateral ventricles and slight atrophy of the basal ganglia. Although these findings seemed to be characteristic for GA1, they were unlikely to be more extended, at least over 2 years after infancy. The low density white matter was observed more evidently in the neonatal or early infantile periods than in later periods. The degree of enlargement of fissures in bilateral frontotemporal regions about the Sylvian fissures appeared to correlate with the severity of symptoms such as dystonia or choreoathetosis. Magnetic resonance images (MRI) in one case showed bilateral linear-shaped low intensity in areas of the external capsules and putamen on a T1-weighted image. These CT and MRI findings, as well as clinical symptoms such as choreoathetosis or dystonia, may suggest that metabolic abnormalities in GA1, such as glutaconate, are toxic to the extrapyramidal tract system in the central nervous system, and that the clinical symptoms of the patients are attributable to atrophy of basal ganglia. Brain CT may be useful in diagnosis and evaluation of the clinical course of GA1 patients.

Brain↗
PubMed1992

Postauricular response in motor paresis with intracranial lesions.

The clinical usefulness of the postauricular response (PAR) in the evaluation of motor paresis was studied in 105 patients with intracranial lesions and 25 normal volunteers. Click stimuli at 90 dB hearing level were delivered in each ear, and PARs recorded from bilateral posterior auricular muscles with reference to the vertex (Cz). No volunteer demonstrated PAR in the relaxed posture, but in tensed postures increasing muscle tone PARs were elicited with a latency of 11.5 +/- 0.7 msec and an amplitude of 6.2 +/- 2.8 muV. Sixty-two patients (59%) had high-amplitude PARs in the relaxed posture. PARs were enhanced more frequently in paretic patients than in non-paretic patients (p less than 0.01). In subcortical lesions, the PAR latency had a significant correlation with the degree of motor paresis (p less than 0.005). In patients, the enhanced PAR amplitude suggested the presence of motor paresis associated with deep-seated mass lesions. The enhanced response is thought to be associated with dysfunction of the pyramidal and extrapyramidal tracts.

Brain Diseases↗
PubMed2001

[Findings of bedside swallowing assessment and brain computerized tomography in patients with chronic cerebral infarction, and their outcome].

To estimate the usefulness of the bedside swallowing assessment proposed by Smithard et al and neuroimaging findings characteristic for dysphagia, we studied the outcome of 102 patients with chronic cerebral infarction after assessment of swallowing by this test with brain computerized tomography (CT). All patients had a variety of motor disturbance and were admitted on a long-term medicare basis. They were divided into two groups according to the findings: the positive group (n = 33), who showed any of the listed types of difficulty in swallowing water, and the negative group (n = 69). Followed up to 2.2 years, their outcomes were studied. CT findings were studied on type of infarction, number and laterality of infarction, grade of periventricular lucency (PVL), presence of ventricular dilatation (VD), and severity of cortical atrophy (CA). The mean age was 76.4 years at registration and 61 were men. The frequency of severe dementia and disturbed ADL were significantly higher in the positive group. Eighteen patients died during the observation period and 15 of those were in the positive group, indicating higher annual death rate (29.9% vs 2.2% in the negative group). All of the 15 patients in the positive group died of pneumonia. CT findings showed high incidence of multiple infarction, bilateral hemispheric lesion, severe PVL, VD, and severe CA in the positive group. These findings indicated that this evaluation method was useful in screening swallow function for patients with cerebral infarction in the chronic phase. Furthermore, CT findings suggested that severe white matter lesion, VD, and severe CA as well as multiple infarction seen in bilateral hemisphere was related to dysphagia, probably due to multiple factors involving pyramidal- and extrapyramidal-tracts with higher brain function.

Adult↗
PubMed2001

Recording of spared motor evoked potentials and its augmentation by 4-aminopyridine in chronic spinal cord-injured rats.

OBJECTIVE: To research the direct electrophysiological evidence of discomplete spinal cord injury (SCI) and the effect of 4-aminopyridine on it. METHODS: Motor evoked potentials (MEPs), both spinal cord recorded MEPs (scMEPs) and extracellularly recorded MEPs (exMEPs) were recorded and characterized on a T13 epidural electrode (scMEPs) and an extracellular microelectrode (exMEPs) for 10 normal rats and 40 rats with lesions of various severity (sham, 35 g.cm force (gcf), 70 gcf, 100 gcf impact injury) at the T8-T9 cord using the Allen's drop model. The incline plane and Tarlov techniques were used to assess clinical neurological function. RESULTS: MEPs in the normal rats were elicited by applying transcortical suprathreshold stimulation consisting of 3-4 early negative peaks (N1, N2, N3 and N4) followed by several late waves. The N1 and N2 peaks were largest in the anterior and ventrolateral funiculus, respectively, which was indicative of extrapyramidal pathways. The 100 gcf impact injuries and the cord transection abolished the MEP distal to the lesion, whereas the 35 gcf injuries resulted in a latency shift and amplitude decrement of the MEP peaks. Eighteen of the 20 rats with 70 gcf-injuries showed clinical paraplegia. Among them, 7 rats had neurophysiological evidence of residual conduction pathways through the lesioned cord segment, such as the presence of N1 and N2 peaks in the scMEPs or exMEPs. After 4-aminopyridine (4-AP) administrations (1 mg/kg), the amplitude of the spared exMEP increased significantly and spread more widely. CONCLUSIONS: MEPs evoked by transcortical stimulation travel mostly in the extrapyramidal tract. MEP monitoring could provide an excellent method of detecting the functional integrity of the motor tracts after SCI, and could even detect spared motor fibers after discomplete SCI. Furthermore, the use of 4-AP or other K+ channel blocking agents may be a potential treatment for patients with chronic moderate to severe SCI.

4-Aminopyridine↗
PubMedDate not supplied

[Paroxysmal stereotypy-tic-dystonia syndrome].

CASE REPORTS: We report the cases of four males from four different families, who presented paroxysmal episodes from the 1st 2nd year. These episodes were characterised by asymmetrical bilateral dystonia of the upper limbs, predominantly in both hands, and were associated with orofacial dyskinesias, stereotipies (jumping, arm flapping, etc.), facial tics and, occasionally, phonic tics. Consciousness is not affected in any of the cases. These movements are triggered in situations where the patient is relaxed or excited. They occur daily and last from a few seconds to 30 minutes. Between the bouts, they remain asymptomatic. Family cases suggest it is inherited by autosomal dominant transmission, perhaps linked to the X chromosome; in addition, two cases are sporadic. In the only adult, the movements progress to a series of rhythmic bilateral dystonic myoclonias and facial tics dyskinesias. All the studies carried out, EEG, hemogram, biochemical analysis, neuroimaging, copper and ceruloplasmin levels, were normal. CONCLUSIONS: 1. We report a non epileptic paroxysmal disorder originating in the extrapyramidal tracts with its own characteristics, with onset during early childhood, which is associated with stereotipies, tics and dystonia; 2. It occurs predominantly in males; 3. It is inherited by autosomal dominant transmission, or perhaps sex linked autosomal dominant inheritance, and there are also sporadic cases; 4. The range of clinical features is very wide and includes cases in which there are few symptoms to others where the extent and gravity of the disorder is very significant.

Adult↗