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An extrapyramidal tract syndrome is reported in an 11-year-old boy following treatment of Hodgkin's disease. Results of investigations and the possible pathophysiology of this disorder are discussed.
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Spinal cord motor evoked potentials (MEPs-S) were elicited in cats by transcranial magnetic stimulation. The MEPs-S recorded from the epidural electrode at the first lumbar (L1) level consisted of four negative peaks (N1, 2.56; N2, 3.19; N3, 4.06; N4, 4.99 ms) followed by small, multiphasic waves. The mean conduction velocities of N1-N3 of the MEPs-S were calculated to be 90 to 115 m/s, which is consistent with extrapyramidal tract activation. A direct brain stem electrical stimulation and sequential transection of the spinal cord studies showed that N1-N3 of the MEPs-S originated primarily from the brain stem and conducted in the ventral funiculus of the spinal cord, which corresponded to the extrapyramidal tracts (e.g., the reticulospinal and vestibulospinal tracts). We also showed that N1 and N2 of the MEPs-S originated mainly from the lower medulla or upper cervical and that N3 originated from the lower pons or upper medulla (vestibular nucleus). On the other hand, N4 of the MEPs-S had a conduction velocity of 70 m/s, which disappeared by ablation of the sensorimotor cortex and pyramidotomy. A dorsal hemisection of the spinal cord, resulting in the disappearance of N4 of the MEPs-S, indicated that it conducted in the dorsolateral funiculus of the spinal cord. These results suggest that N4 of the MEPs-S originates from the sensorimotor cortex and conducts in the lateral corticospinal tract. MEPs-S by transcranial magnetic stimulation in cats constituted complex responses in both the pyramidal and extrapyramidal tracts.
Experiments were carried out on cats to determine the use of conductive evoked spinal cord action potentials in diagnosing motor function of the spinal cord. Direct stimulation from the dura produced three negative wave potentials, N1, N2 and N3. The intraspinal pathway of N2 and N3 was the dorsal column. The pathways of N1, determined by dorsal and ventral epidural recording, were the dorsilateral funicle and the extrapyramidal tracts. A collision experiment between potential N1 and pyramidal tract action potential did not reflect the function of the tract as the amplitude of the action potential was too small. Nevertheless, it is considered that conductive evoked spinal cord action potentials could become a valuable method of assessing spinal cord function as they reflect the function of the extrapyramidal tracts, as well as of the dorsilateral funicle and the dorsal column.
A 51-year-old woman, with progressive gait disturbance and dysarthria, had been diagnosed as Menzel-type spinocerebellar degeneration. Later, she developed dystonic posture of upper limbs and bulging eyes. She was diagnosed as Machado-Joseph disease from neurological findings, which consisted of cerebellar signs, pyramidal tract signs and extrapyramidal tract signs and peripheral neuropathy. She died suddenly of unknown origin. Her illness lasted about 13 years. Neuropathological findings showed moderate neuronal loss with gliosis in the subthalamic nucleus, globus pallidus, substantia nigra, dentate nucleus, oculomotor and hypoglossal nucleus and anterior horn. Positron emission tomography (PET) using 15O steady state inhalation technique revealed reduction of cerebral blood flow and cerebral metabolic rate of oxygen in not only cerebellum but also cerebral cortex. These findings are different from typical PET findings of spinocerebellar degeneration.
Spinal cord evoked potentials were elicited in cats by transcranial electrical stimulation with electrodes on the vertex and hard palate. Vertex motor evoked potentials (V-MEP) were also recorded. An extracellular microelectrode recording technique was then used to analyze the results by isopotential mapping. The relationship between the distribution of field potentials and the stimulation polarity was studied using the field potential distribution of the V-MEP in the lower thoracic spinal cord that had been represented on the isopotential maps. The first negative wave of the V-MEP showed maximal amplitude distribution in the anterior funiculus, which corresponds to the extrapyramidal tracts. This pattern was seen with both stimulation polarity arrangements: 1) stimulation with the cathode at the vertex and the anode at the hard palate, and 2) stimulation with the anode at the vertex and the cathode at the hard palate. When the cathode was at the vertex, the stimulation threshold was lower, and the response had higher amplitude than when the anode was at the vertex. Recording V-MEPs elicited by vertex cathode stimulation could provide an excellent method of monitoring the extrapyramidal tracts in cats.
We studied a case of Fahr's disease type idiopathic intracerebral calcification (Fahr's disease) associated with juvenile rheumatoid arthritis. The patient was a 15-year-old male with a chief complaint of gait disturbance. His family members had no similar signs and symptoms. His parents had no consanguinity. He was born with the normal perinatal course at 1967. He had repeated episodes of convulsive attacks during fever elevation from 2 years and 8 months to 9 years of age. Morning stiffness of bilateral hands, and pernio in the auricles, fingers, planta, and toes had occurred in every winter, since 6 years old. Swelling and pain of the bilateral knee and foot joints appeared, making ambulation difficult in 1983 (15 years old), and the patient was admitted to our hospital in July, the same year. On admission, congenital anomalies such as epicanthus and high-arched palate were noted, and swelling, deformation and contracture of limb joints, and Raynaud phenomenon were shown. His ocular fundus showed no arteriosclerotic change. He didn't have Albright's sign. Mild mental retardation and bilateral pyramidal tract signs were noted, but extrapyramidal tract and cerebellar signs, and sensory disturbance were absent. Laboratory findings exhibited markedly elevated ESR, positive CRP, RA, and antinuclear antibody. The levels of serum Ca, P, alkaline phosphatase and parathyroid hormone were normal. Peripheral blood study showed microcytic and hypochromic anemia. Anti-DNA antibody was negative. Ellsworth-Howard test was positive. Elevated antibody titer to toxoplasma, rubella virus, herpes simplex virus and cytomegalovirus were not proven. He had no chromosomal change.(ABSTRACT TRUNCATED AT 250 WORDS)
In the feline model of the motor evoked potential (MEP) test, a multiphasic spinal cord signal can be elicited in response to bipolar or transcranial brain stimulation. Previous studies have shown that signals produced by threshold stimulation travel mostly in the corticospinal tract. However, from this study we show that suprathreshold stimulation produces very large amplitude MEPs which travel in the ventral funiculus and therefore are most likely associated with extrapyramidal tract activation. The data supporting this conclusion are: (1) apparent conduction velocities of the first two large amplitude peaks are at least 80 m/s with transcranial stimulation; (2) latency of the transcranial MEP at L2 in the cord is less than or equal to 3.50 ms; (3) large amplitude, positive monophasic potentials are recorded in the ventral but not dorsal-lateral funiculus for either bipolar or transcranial MEPs; (4) both bipolar and transcranial MEPs are significantly reduced or abolished by selective lesion of the ventral funiculus. The two tracts which we believe are responsible for mediating the suprathreshold MEP in the cat are the reticulospinal and vestibulospinal tracts. This is significant because suprathreshold MEPs can be used to monitor feline ventral cord function. Furthermore, combining the use of threshold and suprathreshold MEPs may provide a differential diagnostic test for pyramidal vs. extrapyramidal motor function.
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.
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.
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.
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.
Vesicosphincteric disorders are frequent in patients with Parkinson's disease, due to a lesion of the locus niger of the extrapyramidal tract which no longer secretes sufficient dopamine to activate the nigrostriatal tract. The commonest symptoms are urgent micturition, dysuria or a combination of the two. 50% of patients with Parkinson's disease present with disturbances of micturition and these symptoms may be presenting complaint of the disease in 10% of cases. Cystomanometry reveals detrusor hyperactivity in more than 60% of cases and hypoactivity in less than 30% of case, while dyssynergia is less common. Delayed relaxation of the striated sphincter may sometimes be observed. Urological problems must therefore be distinguished from urological problems in these patients. A combined urological and urodynamic assessment is essential as the risk of incontinence following prostate surgery is 20%. Stress urinary incontinence in women may mask or be associated with urgency. These vesicosphincteric disorders must therefore be treated cautiously with a combination of medical treatment (dopatherapy increases detrusor control), retraining and occasionally surgery after a detailed urodynamic assessment.
Five patients are described who presented with major organic brain disease affecting one or more of pyramidal and extrapyramidal tracts, cerebellum, and higher cortical functions. All had a history of 10 years or more of regular occupational exposure to solvents in confined spaces, three in painting inside ships and the others in weapons maintenance and printing. All had been regularly exposed to high air vapour peaks as well as to skin contamination. Four showed some evidence of improvement after the exposure ceased. None was initially suspected of having a toxic encephalopathy by the consultant to whom he was referred. The spectrum of neurological disease presented by these men mirrors closely that described in solvent abusers. All were forced by illness to retire from their work, a circumstance which might have in the past have led to such conditions being missed in cross-sectional studies, which in general have not shown evidence of major disease. We suggest that when such disease occurs nowadays, its cause is usually not suspected. Further epidemiological study of the problem is necessary.
Two neonates of mice which manifested abnormal motions in their gait in the third generation litter, following the start of manganese (Mn) administration, were selected. One was severely affected by Mn and the other was only moderately affected. Various regions in the brains of the neonates were subjected to histochemical examination under a light microscopy. The losses of stainability in granular cells in the external layer of the cerebral cortex, and Purkinje cells in the cerebellar cortex, and the increase in stainability of the nerve fibers in the cerebellar medulla were in parallel to the degree of abnormal movement in the gait; the greater loss or gain in stainability, varying according to the regions, was associated with the more severe damages to motion. Meanwhile, the changes in the stainabilities of nerve cell nuclei in the lamellar structure of cerebral motor areas and the Nissl bodies in the cerebral medulla were already maximal in the moderately affected neonate. These results indicate that the Mn effect covers a broad area of the extrapyramidal tract even though there are some differences in the sensitivity to Mn in different regions.
In order to elucidate the clinical features and the prognosis for life in the patients with multiple system atrophy (MSA) presenting with vocal cord paralysis (VCP), we studied the correlation between VCP and other neurological findings including cerebellar, pyramidal, extrapyramidal and autonomic nervous signs. Subjects were 48 MSA patients: 23 with VCP and age- and illness duration-matched 25 without VCP. MSA in this paper comprised clinically Shy-Drager syndrome, olivopontocerebellar atrophy, and striatonigral degeneration. MSA patients with VCP had in general more severe neurological findings, compared with those without VCP. Urinary incontinence developed in the relatively early stage of illness and preceded VCP in all patients. VCP developed not only in far-advanced stage but at any time in the course of illness. As to swallowing function when a diagnosis of VCP was established, about half of the patients with VCP needed nasogastric tube feeding and the remaining half tolerated oral feeding. VCP correlated strongly with urinary incontinence but not always with the severity of orthostatic hypotension or extrapyramidal tract sign such as parkinsonism. Five of the eight patients without tracheostomy came to sudden death. The mean duration from making a diagnosis of VCP to death was 1.1 years. In contrast, nine of the 11 patients with tracheostomy were alive and the survival periods after tracheostomy reached a maximum, five years. These facts suggest that the prognosis for life in the patients with VCP depends in part upon whether tracheostomy was carried out or not.(ABSTRACT TRUNCATED AT 250 WORDS)