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PubMed2003

[Risk factors for eclampsia: a case-control study].

OBJECTIVE: Our purpose was to characterize the risk factors of eclampsia in women with preeclampsia. PATIENTS AND METHODS: A case-control study was conducted at Monastir hospital to investigate risk factors for eclampsia between 1st January 1995 and 30th June 2000. Cases were matched to preeclamptic controls on a 2:1 ratio. Univariate analysis was used to determine which of the independent variables were significantly different between the groups. Those with significant differences were then entered into multiple logistic regression analysis to determine the characteristics that were independently related to eclampsia. RESULT: A total of 41 cases of eclampsia were ascertained from deliveries. The ratio of eclampsia cases to number of deliveries over the study period was 1.87 per 1000. The first seizures occurred at home in 59% of the cases. Univariate analysis revealed statistical significance for the following variables associated with eclampsia: systolic hypertension > or =160 mmHg and diastolic > or =110 mmHg, headache, visual symptoms, vivid deep tendon reflexes, proteinuria >3+ or >3 g d(-1), uric acid concentration > or =350 micromol l(-1), serum creatinine concentration >100 micromol l(-1) and aminotransferase aspartate >30 IU l(-1). A history of abortion appears to be the protective factor against eclampsia. However, with subsequent multivariate analysis, only vivid deep tendon reflexes and elevated uric acid concentration remained significant. CONCLUSION: These data indicate a need for improved prenatal care and medical attention focused on prodroms of eclampsia as well as the detection of preeclampsia to reduce the incidence of eclampsia.

Adult↗
PubMed1993

[Stark-Kaeser type scapulo-peroneal amyotrophy of neurogenic origin. Study of a familial case].

BACKGROUND: There are several types of the rare syndrome, scapuloperoneal atrophy; the report deals with a familial case of the Stark-Kaeser type. CASE REPORT: A 16 year-old Tunisian boy was admitted for "steppage" gait. His mother had distal myoatrophy of four limbs and pes cavus. His sister, aged 8 years, also had pes cavus. She had no tendon reflexes and electromyography showed evidence of muscular denervation with normal nerve conduction velocity. The "steppage" gait in our patient began when he was 6 years old. When he was examined at 16 years, there were bilateral atrophy of the sternocleidomastoid muscle, and atrophy of the scapular girdle and the peroneal muscles with loss of tendon reflexes; there was no sensory involvement. Nerve conduction velocity was normal and electromyography showed fibrillation and pseudomyotonia potentials. Creatine kinase activities were moderately increased. Muscle biopsy showed significant neurogenic type changes. CONCLUSION: This patient has a Stark-Kaeser type spinal amyotrophy, a well-individualized entity due to progressive degeneration of the anterior horn cells.

Adolescent↗
PubMed2006

Spasticity and its association with functioning and health-related quality of life 18 months after stroke.

BACKGROUND: There is no consensus concerning the presence of spasticity or the relationship between spasticity and functioning and spasticity and health-related quality of life (HRQL) in the stable phase after stroke. OBJECTIVE: The aim of the present study was to describe, 18 months after stroke, the frequency of spasticity and its association with functioning and HRQL. METHODS: In a cohort of 66 consecutive patients with first-ever stroke, studied prospectively, the following parameters were assessed 18 months after stroke: spasticity, by the Modified Ashworth Scale (0-4 points with 1+ as the modification), muscle stiffness, by self-report, abnormal tendon reflexes, by physical examination, motor performance, by the Lindmark Motor Assessment Scale, mobility, by the Rivermead Mobility Index, activities of daily living, by the Barthel Index, and HRQL, by the Swedish Short Form 36 Health Survey Questionnaire (SF-36). RESULTS: Of 66 patients studied, 38 were hemiparetic; of these, 13 displayed spasticity, 12 had increased tendon reflexes, and 7 reported muscle stiffness 18 months after stroke. Weak (r < 0.5) to moderate (r = 0.5-0.75) correlations were seen between spasticity and functioning scores. Correlations between spasticity and HRQL were generally weak (r < 0.5). Hemiparetic patients without spasticity had significantly better functioning scores and significantly better HRQL on 1 of the 8 SF-36 health scales (physical functioning) than patients with spasticity. CONCLUSIONS: Few patients displayed spasticity 18 months after stroke. Spasticity might contribute to impairment of movement function and to limitation of activity, but seems to have a less pronounced effect on HRQL.

Activities of Daily Living↗
PubMed1978

Obesity and thyroid function. 3. Relationship between some indicators of thyroid function and the energy metabolism of striated muscle in obese women.

1. In a group of 23 obese women the relations between some indicators of thyroid function (thyroxine-binding globuline--T4BG, triiodothyronine-binding globuline--T3BG, Achilles tendon reflex--ART) on the one hand and activities of enzymes of the energy metabolism (hexokinase--HK, triose phosphate dehydrogenase--TPDH, lactate dehydrogenase--LDH, glycerol-3-phosphate dehydrogenase--GPDH, citrate synthease--CS, malate dehydrogenase--MDH, hydroxyacyl--COA dehydrogenase) in the quadriceps femoris muscle on the other hand were investigated. 2. Correlations were found between T4BG and TPDH, LDH and GPDH activities, between T3BG and TPDH and GPDH activities and between the value of the Achilles tendon reflex and TPDH activity. Functionally these enzymes activities are associated with glycolysis and hydrogen transport from cytoplasmatic NADH2. No correlations were found between enzymes of the aerobic metabolism incl. enzymes of fatty acid oxidation and indicators of thyroid function. 3. The results indicate a relationship between thyroid function and enzymes involved in glycolysis and hydrogen transport from cytoplasmatic NADH2. They do not suggest, however, the unequivocal conclusion that in obese women with reduced thyroid function there is a generally reduced energy supplying metabolism in skeletal muscle.

Achilles Tendon↗
PubMed1993

[Movement disorders in weightlessness].

The system MONIMIR has been developed to study the coordination of eye, head and arm movements as well as spinal reflexes in microgravity and was used during three spaceflights on board of the station MIR. The following investigations in the course of the experiment MONIMIR were performed: (1) slow head movements in three planes, (2.3) fast pointing movements of eyes, head and arm to acoustic and visual targets, (4) tracking movements of eyes, head and arm to visual targets, (5) head and arm movements based on short term memory and (6) patellar-tendon-reflex. In microgravity different functions and effectors showed different nature and degree of disturbance and different courses in adaptation; in most of the tests exactness and velocity of head and arm movement was decreased; head movements were more disturbed than arm movements; fast pointing movements were more severely affected than slow tracking movements which partly improved; visual controlled movements showed better adaptation as only proprioceptive controlled movements; the patellar-tendon-reflex was highly increased. Disturbances were most pronounced in the early stage of the spaceflights; at later stages most of the performances improved. Methods and results can be used not only for improvement of election and health control of cosmonauts/astronauts for future longterm space missions but also for diagnostics and research of adaptational processes in course of diseases or extreme conditions on earth.

Extraterrestrial Environment↗
PubMed1994

Mechanisms of tizanidine action on spasticity.

This investigation estimated the mechanisms of tizanidine action on spasticity using a battery of neurophysiological methods. Thirty patients with old post-stroke spastic hemiparesis took part in the investigation. They were treated with tizanidine-mean daily dose 15.8 +/- 5.6 mg for a mean of 23.3 +/- 4.8 days. A questionnaire for assessment of subjective improvement after treatment used a 5-point scale. For standardization of the neurological examination 5-point scales were used to assess muscle tone, muscle force and tendon reflexes. A battery of neurophysiological methods was used to analyze different mechanisms of spasticity: for alpha motoneuron excitability--the F wave parameters; for presynaptic inhibition--the ratio of H reflex amplitudes before and after vibration of the achilles tendon (Hvibr/Hmax); for common interneuron activity--the flexor reflex parameters. Our results revealed that tizanidine reduces spastically increased muscle tone, but has no influence on muscle force, tendon reflexes, Babinski sign and ankle clonus. Tizanidine is supposed to act by increasing the presynaptic inhibition and decreasing of alpha motoneuron excitability. When spasticity has decreased presynaptic inhibition and increased motoneuron excitability, it is better to treat with tizanidine.

Adult↗
PubMed1995

The independent contributions of diabetic neuropathy and vasculopathy in foot ulceration. How great are the risks?

OBJECTIVE: To describe the relative contributions of neurological and vascular abnormalities to the overall risk of diabetic foot ulceration. RESEARCH DESIGN AND METHODS: A case-control study of diabetic veterans from the Seattle Veterans Affairs Medical Center was conducted using data collected from 46 patients with diabetic foot ulcers and 322 control subjects. Neuropathy was determined by vibratory, monofilament, and tendon reflex testing. Macrovascular disease was measured by ankle-arm blood pressure index, and cutaneous perfusion was measured by transcutaneous oxygen tension (TcPO2) on the dorsal foot. A multivariate logistic regression model was used to adjust for confounding variables and to calculate the odds ratios (ORs) for each independent risk factor. RESULTS: Three variables were significant independent predictors of foot ulceration: absence of Achilles tendon reflexes (adjusted OR 6.48, 95% confidence interval [CI] 2.37-18.06), insensate to the 5.07 monofilament (adjusted OR 18.42, 95% CI 3.83-88.47), and TcPO2 < 30 mmHg (adjusted OR 57.87, 95% CI 5.08-658.96). Absent vibratory sensation and low ankle-arm blood pressure index were not significant independent risk factors. CONCLUSIONS: Both neuropathy and vasculopathy are strong independent risk factors for the development of diabetic foot ulcers. In our model, the strongest risk factor is impaired cutaneous oxygenation. However, in the clinical setting, sensory examination with a 5.07 monofilament probably remains the single most practical measure of risk assessment.

Age Factors↗
PubMed1993

[An autopsied case of type 2 Machado-Joseph's disease or spino-pontine degeneration].

The authors present the clinico-pathological findings in a member of a family residing in Akita Prefecture located in the north-eastern region of Japan. Four members in three generations of the family developed ataxia. The autopsied patient was a 42-year-old woman, who, at the age of 25, had developed progressive cerebellar ataxia with pyramidal spasticity and increased deep tendon reflexes predominant in the lower extremities. However, she retained fine movement of the hands and fingers and showed no dysarthria until the age of 35. She could no longer walk unassisted at 38 years old. She showed cerebellar ataxia in both hands and legs, dysarthria, bulging eyes, progressive extraoculomotor palsy with nystagmus, bradykinesia, sensory disturbance, and dystonia in the face, upper extremities, and fingers. Deep tendon reflexes were decreased, especially in the lower extremities. Subacute generalized muscular atrophy developed at the age of 39. She became bedridden and died of pneumonia. The clinical diagnosis was Type-2 of the entity known in Japan as Machado-Joseph disease. At neuropathological examination, the brain weight was 1,250 g. The spinocerebellar system including Clarke's column and the spinocerebellar tracts were degenerated, but the cerebellar cortex and inferior olivary nucleus were spared. Slight-to-moderate degeneration was observed in the pontocerebellar system. In the dentate nucleus, most of the neurons showed what is known in Japan as "grumose degeneration", but there was no neuronal loss or gliosis. The hilus of the dentate nucleus and the superior cerebellar peduncle were intact.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1987

Maintenance of specificity by sprouting and regenerating peripheral nerves. I. Normal variability.

Inter-animal variability in the spinal representation of a single hindlimb muscle, tibialis anterior (TA), in the cat, was examined by retrograde transport of intramuscularly injected HRP, dissection of the lumbosacral plexus and reflex testing after acute section of spinal nerves L5, L6, S1 and S2 sparing L7. No variability between the two sides of the same animal was seen. The transverse position of the TA motor nucleus and the number of labeled cells was constant between the two sides in each animal. Inter-animal variability was considerable, however, in that the number of motor neurons and rostrocaudal extent of the motor neuron column supplying TA varied considerably from animal to animal. According to the relationship between the position of the lumbosacral plexus and the distribution of spinal nerves, 3 classes of representation of the plexus were found: prefixed, postfixed and intermediate. In animals in which the lumbosacral plexus was prefixed, more than one half of labeled cells were rostral to the L7 segment; in those with postfixed plexus more than half the cells were caudal to L7. Section of L5, L6, S1 and S2 spinal nerves weakened the tibialis anterior tendon reflex in 'prefixed plexus' animals but abolished that reflex in 'postfixed' plexus animals, in spite of the presence of labeled motor neurons projecting through the spared L7 nerve. This suggests that some of the afferents and efferents comprising the TA tendon reflex may travel in different spinal roots or that a particular distribution of motor axons within a muscle is required for the maintenance of this particular reflex activity.

Animals↗
PubMed1991

A case of Marfan's syndrome phenotype associated with neurogenic muscle atrophy.

A 23 year-old man was admitted because of weakness and atrophy in both tights. He was 184 cm in height with arachnodactyly and had a mild dilatation of the ascending aorta which was detected by cardiac echogram and chest CT scan. A laboratory examination revealed normal serum values for mucopolysaccarides and amino acids, and 46XY chromosomes. These findings indicate a Marfan's syndrome phenotype. A neurological finding showed a proximal dominant muscle atrophy and increased deep tendon reflexes. The EMG revealed a polyphasic action potential, and a subsequent muscle biopsy demonstrated a small group atrophy and atrophy of Type II fibers. Neuroradiological studies of brain and spinal cord indicated no substantial abnormalities. Only 4 cases of Marfanoid habit accompanied by primary nervous system disturbances have been reported and these had variable complications or severe neurological deficits. The present case, which involved mild neurogenic muscle atrophy and brisk deep tendon reflexes without complications except myopia, was unique.

Adult↗
PubMed1990

[A case of Binswanger disease with numerous diffuse plaques in the neocortex].

A case of Binswanger disease with numerous diffuse plaques in the neocortex was reported. This male patient had a previous history of hypertension and myocardial infarction. From the age of 60, he developed dysarthria, bradykinesia, marche à petit pas and falling down. Neurological examination at his first admission disclosed muscular rigidity and increased jaw and deep tendon reflexes, but dementia was not found. Brain CT showed moderate brain atrophy and EEG consisted of slow wave dysrhythmia. He was diagnosed of Parkinsonism and treatment started without effects. During his second admission for the treatment of myocardial infarction, at the age of 64, delirium developed. Progressive dementia began and finally he was confined to bed. From the age of 69, spontaneous speech became almost lost. Contracture of the extremities, increased deep tendon reflexes and force grasping were noted. Brain CT showed symmetrical low attenuation in the frontal and parietal white matter with moderate dilatation of the lateral ventricles. At the age of 70, he died of general prostration about ten years after the initial symptoms. Neuropathological findings: Macroscopic findings: The brain weighed 1300 g. Atherosclerotic changes of the large arteries at the base of the brain were moderate. Coronal sections of the brain showed moderate enlargement of the lateral ventricles with multiple small lacunes in the basal ganglia. Microscopic findings: Bilateral diffuse demyelination of the white matter with sparing of the U-fibers was noted. Holzer stain revealed fibrillary gliosis in the left parietal and occipital white matter. Marked adventitial fibrosis of the deep white matter arteries and terminal stages of hyalinosis of the perforating arteries were found. Basal ganglia showed status lacunaris. Bilateral pyramidal tracts were atrophic secondly.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1995

[Phenotypic heterogeneity in Japanese Charcot-Marie-Tooth disease type 1A patients with PMP-22 gene duplication].

We studied phenotypic heterogeneity in 18 Japanese patients with Charcot-Marie-Tooth disease type 1A (CMT1A) with PMP-22 gene duplication, together with heterogeneity of duplication size. In order to detect the duplication of PMP-22 gene region, the PMP-22 cDNA and a polymorphic marker VAW409R3 were used as probes for Southern blot analysis. As the clinical phenotypes, we assessed the degree of foot deformity, muscular weakness and atrophy, tendon reflexes, sensory impairment and electrophysiologic and sural nerve biopsy findings. Although the degree of muscular weakness and atrophy was slightly more severe in the advanced age, there was a patient with calf hypertrophy in the older age or a patient with marked muscular atrophy of the leg in the younger age. The incidence of foot deformities and sensory impairment was high and these phenotypes did not relate to aging. Diminished or absent tendon reflexes and slowing of motor conduction velocities were commonly seen, but the motor conduction velocity varied greatly among the patients. The clinical phenotypes were extensively variable among the CMT 1A patients with the same gene mutation of PMP-22 gene duplication, suggesting that there is a factor other than PMP-22 gene duplication, which influences phenotypic manifestation in CMT 1A.

Adult↗
PubMed1988

Friedreich's ataxia: a clinical review of 20 childhood cases.

The authors report the clinical review of 20 childhood cases with Friedreich's ataxia. The mean age at onset of symptoms was 6.1 years. The main presenting symptom was abnormal gait (100%). Ataxia of gait and limbs and depressed or absent tendon reflexes were found in all cases. Clinical findings are in accordance with the findings of Harding and Werdelin. Neurophysiological studies (especially sensory) are important in the confirmation of the diagnosis. Of the 10 cases in which sensory nerve conduction velocity measurements were performed, 7 had absent sensory action potentials, 2 had decreased potentials and one was normal. In our study, it is shown that in patients having ataxic gait, ataxia of limbs and tendon reflexes depression or loss, Friedreich's ataxia may be diagnosed with the help of electrophysiological studies.

Adolescent↗
PubMed1993

[Some problems on the clinical phenotype of Machado-Joseph disease in relation between their ages at onset].

UNLABELLED: This study proposed that three phenotypes of Machado-Joseph disease (MJD) are closely related to the patients' ages at onset. MATERIALS: Six patients from two families. Autopsy performed in three of them (case 2, 4, 5). Two patients are a father (case 4) and his son (case 5). RESULTS: 1. Clinical features. All cases showed cerebellar ataxia and nystagmus. Progressive nuclear oculomotor palsy was common except for one case who killed himself in the early clinical stage. Pyramidal symptom which is increased deep tendon reflexes, spasticity, and extesor plantar response was common for three patients (case 3, 5, 6) whose ages of onset are under 40 years. One case (case 5) developed dystonia of foot at the age of ten and he developed the symptom of type 1 of MJD. However, the other three patients (case 1, 2, 4) who developed ataxia after the middle of the fifth decade showed hypotonia and decreased or absence of deep tendon reflexes from the beginning. The latter did not revealed spasticity or dystonia. Their clinical symptoms were identical with the type 3 of MJD. In spite of ages at onset, they showed general muscular atrophy and sensory disturbance in the advanced clinical course. 2. Neuropathological findings. 1) cerebellar system: Severe degeneration in the spinocerebellar system and mild to moderate one in the pontocerebellar system and dentate nuclei. The inferior olivary nucleus and the cerebellar cortex were almost preserved. 2) extrapyramidal system: Moderate to severe degeneration in the substantia nigra, globus pallidus (prominent in the internal segment) and subthalamic nucleus. 3) Degeneration in the oculomotor nuclei, motor neurons in the anterior horn and dorsal column of the spinal cord. CONCLUSION: These clinico-pathological findings indicate the difference of clinical phenotype is not always reflected those of neuropathological findings. The review of our experience and the literature suggests that the clinical features of MJD symptoms are related to the patients' ages at onset and clinical progression of the disease. When the disease begins before the age of ten, dystonia is an initial symptom, followed by pyramidal symptoms and cerebellar ataxia (type 1). In the early adult cases, the onset in which is earlier than forty, cerebellar ataxia and pyramidal symptoms are the initial symptoms, followed by extrapyramidal symptoms such as dystonia or choreoathetoid movements or both (type 2). In both instances however, decreased DTRs, muscular atrophy and sensory disturbance are common clinical manifestations at the advanced clinical stage. In late adult MJD cases with the age at onset after forty, the initial symptom is progressive cerebellar ataxia with hypotonia, followed by muscular atrophy and sensory disturbance (type 3). In spite of a marked degeneration in the extrapyramidal system, few or no extrapyramidal symptoms are detected in the last cases.

Adult↗
PubMed1999

Sensory impairment: a feature of chronic venous insufficiency.

OBJECTIVE: Clinical and microscopic evidence suggests the existence of sensory neuropathy in patients with severe chronic venous insufficiency (CVI). A clinical evaluation was conducted to determine whether a sensory neuropathy was present and, if so, to determine its extent and distribution. METHODS: The study was performed in a university-affiliated Veterans Affairs Medical Center. Twenty-three limbs were studied in 14 male veterans with mild or moderate CVI. The exclusions included diabetes, previous ipsilateral extremity surgery, or other diseases associated with neuropathy. Sensory thresholds in the limbs with CEAP class 2 disease (n = 11) were compared with the thresholds in the limbs with CEAP class 5 disease (n = 12) at nine different sites on the foot, ankle, calf, thigh, and palm. Thenar and hypothenar thresholds were measured as internal controls. Thresholds were determined by a pressure aesthesiometer consisting of 20 graduated filaments that ranged from 1.65 to 6.65 (log(10)mg)(10) of pressure. A complete, sensory motor assessment of the limb was performed by an experienced neurosurgeon who specialized in peripheral nerve evaluation. The clinical variables assessed were deep tendon reflexes, vibration, proprioception, and light touch. Venous reflux was determined with duplex ultrasound scanning and air plethysmography. RESULTS: Sensory thresholds at the most common site of venous ulceration-just proximal to the medial malleolus--were significantly (P <.05) different between mild (class 2) and severe (class 5) CVI. Sensory abnormalities coincided with the extent of trophic changes and did not reflect specific dermatomal or cutaneous nerve distributions. In addition to light touch or pinprick, vibration sense and deep tendon reflexes were also significantly worse in those with severe CVI. CONCLUSION: Sensory neuropathy is a feature of severe CVI, and its distribution is coincident with trophic changes. Because this is often unappreciated by the patient, it probably contributes to the propensity for deterioration from minor trauma.

Arm↗
PubMed1998

Sarcoid neuromyopathy with selective involvement of the intramuscular nerves.

A 35-year-old man affected with pulmonary sarcoidosis had a 12-year history of fatigue and pain in the limbs, with normal neurological examination, except for diffusely absent deep tendon reflexes. Muscle biopsy samples showed multiple noncaseating granulomas, most prominent around the intramuscular nerves, with predominance of CD4+ cells. Intramuscular nerve bundles surrounded by granulomas were immunolabelled with laminin alpha1, alpha2, beta1 and gamma1 chain, and collagen IV. Sural nerve biopsy samples were normal. This patient showed a unique histopathological pattern of sarcoid neuromyopathy characterized by distribution of granulomas or infiltrating cells around intramuscular nerve fibers. The clinical picture, restricted to nonspecific symptoms of fatigue and myalgia, and loss of deep tendon reflexes, correlated well with the selective localization of sarcoid lesions in contiguity with the intramuscular nerves. To our knowledge, this peculiar clinico-pathological correlation has not been reported previously.

Adult↗
PubMed2001

Indicators of rapid clinical recovery in Guillain-Barré syndrome.

To elucidate the features of patients with Guillain-Barré syndrome who show markedly rapid clinical recovery, clinical, serological, and electrophysiological data of 80 consecutive patients were reviewed. Antigangliosides, and Campylobacter jejuni and Haemophilus influenzae antibodies were measured by enzyme linked immunosorbent assays. Nine (11%) patients showed rapid recovery (improvement by two or more Hughes grades within 2 weeks). They often had electrodiagnosis of acute motor axonal neuropathy (AMAN; 67%), preserved tendon reflexes (44%), anti-GM1 antibodies (89%), preceding H influenzae infection (44%), and received immunoglobulin treatment (44%). On the other hand six patients with poor prognosis often had AMAN (100%) and anti-GM1 antibody (83%), but a higher incidence of preceding C jejuni infection (83%). It is concluded that patients with Guillain-Barré syndrome with AMAN and anti-GM1 antibodies have either faster or slower recoveries. Among the axonal subgroup of patients with Guillain-Barré syndrome, preserved tendon reflexes, H influenzae infection, and the patient having received immunoglobulin treatment may be indicators of rapid recovery.

Adolescent↗
PubMed1989

[Two cases of post-poliomyelitis muscular atrophy].

We report two males with progressive post-poliomyelitis muscular atrophy (PPMA) who developed slowly progressive weakness and atrophy in the previously unaffected muscles 50 years after acute poliomyelitis. Case 1 is a 61-year-old male who had suffered from poliomyelitis at the age of 3 years. After recovery from acute symptoms, paralysis remained in the right face, left upper extremity and right lower extremity. Paralysis had remained unchanged until the age of 55 years, when he felt increased fatigue and progressive muscle weakness of all extremities. Neurological examination revealed muscle atrophy of the left upper and right lower extremities, and muscle hypertrophy of the left lower extremity. Deep tendon reflexes were decreased or absent. Babinski sign was negative. Reduction of his physical level and administration of anticholinesterase agents improved his feeling of fatigue. Case 2 is a 66-year-old male who suffered from poliomyelitis in his infancy, and paralysis remained in the left hand and right lower extremity. At the age of 50 years, he felt fatigue, muscle weakness of the previously unaffected legs and joint pain. Neurological examination revealed proximal muscle atrophy and weakness of lower extremities. Deep tendon reflexes were decreased and Babinski sign was negative. Needle electromyography of both cases showed typical neurogenic pattern or high amplitude and polyphasic long duration MMUs. Muscle biopsy of them from quadriceps femoris showed variability in fiber size, moderate increase of internal nuclei, fiber splitting, type grouping, grouped atrophy and scattered small angulated fibers, indicating chronic denervation and reinnervation. The clinical features and laboratory findings of the present two cases are consistent with those of PPMA.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗