1/16. Troyer syndrome: a combination of central brain abnormality and motor neuron disease?Hereditary spastic paraplegia is a group of clinically and genetically heterogeneous disorders consisting of pure and complicated forms. A variant with the additional features of severe atrophy of the small hand muscles, dysarthria, mental retardation, and short stature has been termed Troyer syndrome (MIM#275900) after the name of Old Order amish families suffering from these symptoms. We report here an Austrian family with two individuals who exhibit all the features of Troyer syndrome, and provide additional data on this disorder. Electrophysiological studies showed chronic denervation and reduced motor nerve conduction velocities but normal sensory potentials. Muscle biopsy revealed a neurogenic pattern while the sural nerve was normal on histological examination. brain abnormalities on magnetic resonance imaging consisted of a thin corpus callosum with a poorly developed cingulate gyrus and mild periventricular signal hyperintensities. These findings characterize the Troyer syndrome as a disorder of the first and second motor neuron with additional damage in the brain. The morphological features observed in this family may contribute to the grouping and subsequent understanding of complicated forms of hereditary spastic paraplegia, together with similar observations in other, more recently reported families.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
2/16. Motor neuron presentation of an ulnar neuropathy and Riche-Cannieu anastomosis.A Riche (7)-Cannieu (2) anastomosis (ulnar-to-median anastomosis in the hand) in the setting of an ulnar or median nerve lesion can produce confusing clinical and electrodiagnostic findings. We report a patient with a deep branch ulnar neuropathy complicated by a Riche-Cannieu anastomosis. His clinical presentation led to an initial diagnosis of motor neuron disease. Extensive electrophysiologic studies clarified the extent of the Riche-Cannieu anastomosis and the ulnar neuropathy.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
3/16. motor neuron disease after electric injury.The occurrence of motor neuron disease after electrical injury in six patients is reported and compared with patients from the literature. The patients were five men with spinal onset and one woman with bulbar motor neuron disease after electric shock. Two patients were struck by lightning and four by industrial electric shock. For all six of them, the disease started at the site of the electrical trauma. The mean delay for onset of motor neuron disease was 44 months. In four of the spinal patients the disease progressed slowly with mild handicap after several years. For the fifth patient, improvement was noted progressively. The patient with bulbar disease died 26 months after onset. A link between electric shock and motor neuron disease is likely, given the homogenous profile of the patients both in the five spinal cases presented here and in the literature. Bulbar onset has not been reported to date. However, in this patient the long delay between the electrical injury and motor neuron disease, together with the rapid evolution may suggest a chance association.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
4/16. Lower motor neuron dysfunction in patients with multiple sclerosis.A patient in whom multiple sclerosis (MS) was ultimately diagnosed presented with a lower motor neuron syndrome involving 1 hand, with EMG evidence of denervation. Twelve other patients were subsequently identified with definite MS and asymmetric hand atrophy. These patients were studied clinically and electrophysiologically. Evidence of chronic and ongoing denervation was noted in the hands of 12 of the 13 patients; in only 3 patients could the EMG abnormalities be accounted for by peripheral nerve lesions. Thus, lesions resulting in lower motor neuron damage may occur in the central nervous system in MS patients. We suggest that demyelination in the region of the ventral root exit zone may account for these findings.- - - - - - - - - - ranking = 3keywords = hand (Clic here for more details about this article) |
5/16. Transient reversal of hiv-associated motor neuron disease following the introduction of highly active antiretroviral therapy.Neurological diseases occur frequently in patients with human immunodeficiency virus (hiv) infection, and include a variety of neuromuscular disorders. On the other hand, only a few cases of motor neuron disease (MND) have been reported to date in hiv-positive patients, even though this neurological complication occurs with a 27-fold greater frequency in these subjects compared with the general population. A retroviral etiology for MND has long been hypothesized, and epidemiological and experimental data suggest a pathogenetic link between hiv infection and MND, because retroviral infections may cause motor neuron damage in both laboratory animals and humans, as a result of various pathways. Furthermore, the introduction of potent, protease inhibitor-based antiretroviral combinations has had a great impact on the natural history of hiv disease and produced a dramatic improvement in some patients with hiv-associated MND, but optimal treatment for this progressive neurological complication has not been well defined. A case of MND in a male hiv-infected patient with significant but transient reversal of neurological symptoms after the use of protease inhibitor-containing antiretroviral regimen is described.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
6/16. magnetic resonance imaging of thoracic epidural venous dilation in Hirayama disease.Hirayama disease is a nonprogressive, asymmetric amyotrophy of the hands and forearms, possibly caused by compression of the lower cervical cord on neck flexion. The authors used phase-contrast MR angiography to study a patient with this disorder and observed abnormal spinal epidural venous dilation on neck flexion. In addition to mechanical compression of the lower cervical cord, venous congestion in the spinal canal may have a role in promoting anterior horn damage.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
7/16. A case of camptocormia (bent spine) secondary to early motor neuron disease.Camptocormia is a gait disorder, characterized by hyperflexion of thoracolumbar spine which increases on walking, and disappears in the supine position. A 48 year-old man developed progressive gait deterioration for one year and slight weakness and tremor of both hands for five months. It eventually became apparent that the patient had motor neuron disease, as well as symptoms of extrapyramidal disorder.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
8/16. A Korean case of juvenile muscular atrophy of distal upper extremity (Hirayama disease) with dynamic cervical cord compression.We present a Korean case of Hirayama disease with its typical neuroradiological findings of forward displacement of cervical dural sac and compression of the lower cervical cord during neck flexion. A 15-yr-old boy was presented with a one-year history of progressive weakness and atrophy affecting bilateral hands and forearms. The electrodiagnostic findings were compatible with the lesion of the anterior horn cells at the C7, C8, and T1 spinal segments. With neck flexion, cervical magnetic resonance imaging (MRI) showed the anterior shifting of the lower cervical dural sac resulting in the cord compression of those segments. Presumably, this disease might have been prevalent in korea frequently under the diagnosis of "benign focal amyotrophy". In this regard, we discuss the clinical importance of cervical MRI with neck flexion and anticipate the increasing reports of the case substantiated by its characteristic radiological features.- - - - - - - - - - ranking = 1keywords = hand (Clic here for more details about this article) |
9/16. Motor neuronopathy with dropped hands and downbeat nystagmus: a distinctive disorder? A case report.BACKGROUND: eye movements are clinically normal in most patients with motor neuron disorders until late in the disease course. Rare patients are reported to show slow vertical saccades, impaired smooth pursuit, and gaze-evoked nystagmus. We report clinical and oculomotor findings in three patients with motor neuronopathy and downbeat nystagmus, a classic sign of vestibulocerebellar disease. CASE PRESENTATION: All patients had clinical and electrodiagnostic features of anterior horn cell disease. Involvement of finger and wrist extensors predominated, causing finger and wrist drop. Bulbar or respiratory dysfunction did not occur. All three had clinically evident downbeat nystagmus worse on lateral and downgaze, confirmed on eye movement recordings using the magnetic search coil technique in two patients. Additional oculomotor findings included alternating skew deviation and intermittent horizontal saccadic oscillations, in one patient each. One patient had mild cerebellar atrophy, while the other two had no cerebellar or brainstem abnormality on neuroimaging. The disorder is slowly progressive, with survival up to 30 years from the time of onset. CONCLUSION: The combination of motor neuronopathy, characterized by early and prominent wrist and finger extensor weakness, and downbeat nystagmus with or without other cerebellar eye movement abnormalities may represent a novel motor neuron syndrome.- - - - - - - - - - ranking = 4keywords = hand (Clic here for more details about this article) |
10/16. amyotrophic lateral sclerosis with marked neurological asymmetry: clinicopathological study.We attempted to correlate the marked neurological asymmetry observed in two amyotrophic lateral sclerosis patients with their histopathological lesions. Patient 1, a 52-year-old man, developed dysarthria and dysphagia, followed by muscle weakness in the left arm and then of the left leg. Patient 2, a 44-year-old man, developed muscle weakness in the left hand, left leg, tongue with left-sided predominance, right hand and right leg in that order of progression. Both patients exhibited moderate to marked left-sided predominant involvement of the lower motor neuron system, accompanied by retained or hyperactive deep tendon reflexes on the left side in the early stage of their illness. Most of the asymmetry in the lower motor neuron system involvement persisted until the death of the patients. Histopathological examinations, including semiquantitative analysis, revealed that both patients exhibited left-sided predominant degeneration of the lower motor neuron system at those spinal cord levels where the neurological asymmetry was of a moderate to marked degree. In addition left-sided predominant degeneration of the lateral corticospinal tracts was seen in both patients and right-sided predominant involvement of Betz cells in the leg area of the motor cortex of patient 1. This pattern of both the neurological and histopathological asymmetry suggested the probable existence of an intimate somatotopically related linkage between the upper motor neuron system degeneration and lower motor neuron system degeneration in both patients.- - - - - - - - - - ranking = 2keywords = hand (Clic here for more details about this article) |
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