Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026837 (muscle rigidity)
 1,077 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In two cases of chronic schizophrenia complicated by diabetes mellitus, the concomitant use of the neuroleptica and oral antidiabetics was attended by the appearance of symptoms simulative of syndrome malin, i.e. hyperpyrexia, tachycardia, blood pressure instability, disturbances of consciousness, muscle rigidity, tremor, dysphagia, salivation and urinary incontinence. In one of these cases, the patient, a 47-year-old man, died 10 days later. In the other case, a 62-year-old woman, almost all the symptoms subsided after 14 days, and oral dyskinesia persisted for only one additional month. In both cases, hypoglycemia due to oral antidiabetics was not seen. In Case 2, a combined regimen of oral antidiabetics and neuroleptica was later resumed. Again, a similar set of symptoms as seen initially were noted, along with an elevation of the serum CPK level. Parenterally administered biperiden proved to be highly effective in the control of the symptoms. The pathogenetic mechanism of these symptoms might possibly be explained as potentiation of the action of the neuroleptica by oral antidiabetics.
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PMID:"Syndrome malin"-like symptoms probably due to interaction between neuroleptica and oral antidiabetic agents. 65 48

Stiff-man syndrome is a rare disorder of the central nervous system consisting of progressive, fluctuating muscle rigidity with painful spasms. It is occasionally associated with endocrine disorders, including insulin-dependent diabetes, and with epilepsy. We investigated the possible existence of autoimmunity against the nervous system in a patient with stiff-man syndrome associated with epilepsy and Type I diabetes mellitus. Levels of IgG, which had an oligoclonal pattern, were elevated in the cerebrospinal fluid. The serum and the cerebrospinal fluid produced an identical, intense staining of all gray-matter regions when used to stain brain sections according to an indirect light-microscopical immunocytochemical procedure. The staining patterns were identical to those produced by antibodies to glutamic acid decarboxylase (the enzyme responsible for the synthesis of gamma-aminobutyric acid). A band comigrating with glutamic acid decarboxylase in sodium dodecyl sulfate-polyacrylamide gels appeared to be the only nervous-tissue antigen recognized by cerebrospinal fluid antibodies, and the predominant antigen recognized by serum antibodies. These findings support the idea that an impairment of neuronal pathways that operate through gamma-aminobutyric acid is involved in the pathogenesis of stiff-man syndrome, and they raise the possibility of an autoimmune pathogenesis.
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PMID:Autoantibodies to glutamic acid decarboxylase in a patient with stiff-man syndrome, epilepsy, and type I diabetes mellitus. 328 Oct 11

Stiff-man syndrome is a rare neurologic disorder characterized by progressive, fluctuating muscle rigidity with painful muscle contractions affecting predominantly the back and proximal extremities. In the ED, the diagnosis can be easily overlooked and misdiagnosed as acute or chronic low back pain and muscle spasm. This syndrome is often associated with diabetes, autoimmune diseases, and cancer. This report describes an illustrative case of a 39-year-old woman who presented to the ED with a two-year history of right leg spasms and low back pain that had become so severe in the preceding two days that she was unable to ambulate. Clues to the patient's proper diagnosis coincide with the diagnostic criteria for stiff-man syndrome: the presence of a slowly progressive stiffness of the axial muscles and proximal limb muscles, making ambulation difficult; hyperlordosis of the lumbar spine; episodic spasms precipitated by jarring or sudden movement; a normal intellectual, sensory, and motor examination when not in spasm; and a marked amelioration of symptoms with the IV administration of diazepam. High-dose oral diazepam is the maintenance drug of choice.
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PMID:Stiff-man syndrome: case report. 758 54

Familial polyneuropathy mimicking Charcot-Marie-Tooth disease associated with parkinsonism and dementia has been reported in literature. We present with similar peroneal muscular atrophy, rigidity of upper extremities, severe peripheral neuropathy, mental retardation and diabetes mellitus. The patient, a 42-year-old man, developed progressive muscle weakness, mental retardation and difficulty in walking in childhood. Because of his pes cavus, he had three surgical operations. At the age of 20 years, he developed distal muscular atrophy of lower limbs. On neurological examination, all limb muscles were atrophic, especially in lower one third of the thigh. Rigidity was noted in the upper extremities. Deep tendon reflexes were hyperactive in the upper and diminished in the lower extremities. Muscle CT revealed low density areas in all the muscles examined, specially in the gastrocnemius and anterior tibial muscles. Needle EMG showed neurogenic change in the forearm, but not in the lower limbs, because of no voluntary contractions obtained due to severe muscle atrophy. Marked slowing of motor conduction velocity with muscle action potentials of very low amplitude was found in the ulnar nerve. Muscle action potentials were not elicited in the median and peroneal nerves. Sensory action potentials were not elicited from the median, ulnar and sural nerves. These findings were consistent with axonal polyneuropathy. In the sural nerve biopsy, the densities of myelinated fibers were markedly decreased. However, unmyelinated fiber densities were relatively preserved. Onion bulb formation was not found. This patient may be classified into hereditary motor-sensory neuropathy (HMSN) type II based on the clinical findings delayed nerve conduction velocities and axonal degeneration in the sural nerve. He has also diabetes mellitus. CT of the brain revealed nothing particular. He is one of members with familial Parkinson's disease (PD) developed in Sagamihara. Peroneal muscular atrophies are not necessarily associated with PD, though it has been occasionally complicated in various neuro-degenerative diseases including parkinsonism. We are now following the patient to detect the symptom of Parkinson's disease for early treatment.
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PMID:[An unusual case of peroneal muscular atrophy with rigidity, polyneuropathy, mental retardation, and diabetes mellitus developed in familial Parkinson's disease]. 866 30

Do we need impotence testing? Yes, it is the clinician's obligation to establish the etiology of impotence: end organ vascular failure vs neurologic dysfunction vs psychosexual dysfunction, classify the severity of that dysfunction, and select a therapy that is not only acceptable to the patient but also addresses his pathology. The most commonly utilized diagnostic tests for erectile dysfunction are outlined in this monograph. Nocturnal erections are evaluated by tests commonly known as nocturnal penile tumescence (NPT) studies. NPT has been measured by each of the following methods: stamp test, Snap Gauges, strain gauges, NPTR (Rigiscan, Osbon Medical Systems), and sleep lab NPTR. Normal Nocturnal Penile Tumescence and Rigidity (NPTR) depends on both the integrity of the corticospinal efferents to the penis and vascular responsiveness of the penile tissues to those nerve signals. When nocturnal erections are of appropriate duration and strength the central and peripheral neuroeffectors and intra-corporal regulators of penile hemodynamics are intact. Unfortunately, abnormal NPTR is of little value in determining the etiology or classifying the severity of vascular impotence; the most prevalent kind of end organ failure. The sacral reflex arc of erection consists of somatosensory afferents via the dorsal and pudendal nerves and autonomic efferents via the pelvic and cavernous nerves. These afferents have been measured indirectly by somatosensory evoked potentials (SSEP) and bulbocavernosus reflex latency (BCR). Penile EMG's have recently been recorded, corporal cavernosal smooth muscle electrical activity: CC-EMG. This technology is far from standardized; computer-assisted interpretations of penile electrical potentials may eventually differentiate afferent nerve pathologies so long inferred in: diabetes, spinal cord injury and following radical pelvic surgery. Numerous diagnostic tests have been employed to evaluate penile hemodynamics: penile plethysmography, penile blood pressures, penile brachial index, selective internal pudendal pharmacoangiography, Doppler sonography, dynamic infusion cavernosometry/cavernosography, nuclear washout radiography, and color duplex Doppler ultrasound. Insufficient corporal veno-occlusion is implicated in up to 50% of patients. The diagnosis and demonstration of venous leakage requires complete smooth muscle relaxation. Veno-occlusive dysfunction is associated with poorly sustained erections; this pathology has traditionally been evaluated with Dynamic Infusion Cavernosometry and Cavernosography. DICC is an invasive test, and is now primarily reserved for patients considering the option of vascular reconstructive procedure. Pharmacotesting consists of intracavernous injection and visual rating of the subsequent erection; the test is the most commonly used office procedure for diagnosing erectile dysfunction. It is simple, minimally invasive, and performed without monitoring equipment. Hemodynamic investigations suggest that a positive injection test is associated with normal veno-occlusion, but not necessarily with normal arterial function. When the penile response to pharmacotesting is suboptimal or equivocal, diagnostic testing with duplex Doppler assessment should be performed. The penile blood flow study (PBFS) provides an objective, minimally invasive evaluation of a suboptimal/equivocal erectile response.
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PMID:Evidence based assessment of erectile dysfunction. 964 64

1. We examined 156 patients 33 years after CO poisoning occurred at the Miike Mikawa Mine, Fukuoka, Japan. The subjects were classified according to age as follows: between 55 and 59 years (n = 14), 60 and 69 years (n = 62), 70 and 79 years (n = 60), and 80 and 87 years (n = 18). The mean age was 69.2 years old. Concerning the duration of coma that occurred soon after the accident, 64 remained comatose from 0 to 6 hours, 46 from 6 to 12 hours and 46 from 12 to 48 hours. 2. Subjective symptoms were observed in 96.8% of the patients. Among them, forgetfulness was noted in 89.7%, followed by irritability in 66.7%, headache in 59.6%, insomnia in 55.8%, limb pain in 46.8%, dull head feeling in 42.9% and dizziness in 36.5%. 3. Intellectual disturbances were observed in 68.6% of the patients, including impression disturbance in 58.3%, memory disturbance in 51.9%, calculation disturbance in 63.5%, thinking disturbance in 61.5% and disorientation in 14.1%. 4. Apathy and disorder of volition and interest which were found in 72.4% were included in personality change because all symptoms persisted for many years. Personality change was classified as follows: weakness of emotion and will (hypobulia) in 54.4%, infantilism in 35.2%, hyperactive, talkactive and lack of inhibition in 18.5%, lack of self-possession and unstable temper in 9.6%, depression in 15.3%, neurosis in 7.6% and schizophrenic state in 2.5%. Among these symptoms of personality change, weakness of emotion and will and infantilism were conspicuous among the patients who remained in a coma for more than 6 hours soon after the accident but showed no relationship with age. 5. Neurological symptoms that were found in 48.7% of the patients were classified as sensory disturbance in 25.6%, peripheral nerve symptoms in 16.0%, pyramidal symptoms in 14.1%, ataxia and cranial nerve symptoms in 7.1%, paroxysmal symptoms in 6.4% and focal symptoms in 4.5%, extrapyramidal symptoms in 21.8% (Parkinsonism in 4.5%, tremor in 10.9% and muscle rigidity in 16.0%) and vegetative symptoms in 37.2%. 6. At the time of investigation, 5 CO poisoning patients were classified as serious cases (3.2%), 20 as comparatively serious (12.8%) medium-degree cases, 28 as comparatively mild (17.9%) medium-degree cases, 37 as comparatively serious (23.7%) mild cases, 42 as comparatively mild (26.9%) mild cases, 24 (15.4%) as having symptoms which were not problematic, and 24 (15.4%) as having symptoms that markedly worsened due to complication. 7. A total of 138 (88.4%) cases had complications were classified as follows: 78 cases (50.0%) of hypertension, 62 cases (39.7%) of cerebral infarction, 24 cases (15.4%) of cardiac disturbance, 21 cases (13.5%) of diabetes mellitus, 14 cases (9.0%) of hepatic disturbance and six cases of silicosis (3.8%). 8. Cranial MRI was carried out for 129 cases (82.7%). Of the abnormal findings identified, cerebral atrophy accounted for 72.0% (93 cases), including moderate and severe cases in 47.2% (61 cases), pallidum lesion for 37.9% (49 cases), lacunar infarction (including cerebral infarction) for 52.7% (68 cases), and hippocampal atrophy for 18.6% (24 cases). Many cases of cerebral atrophy and hippocampal atrophy were observed in patients who remained in the initial coma for more than 12 hours and were 80 years of age or old. The cases of pallidum lesion were observed in patients who remained in the initial coma for more than 6 hours, and no relationship with age was found. The other findings, cerebral atrophy and lacunar infarction showed a slight relationship with age. 9. Among the moderate and serious cases of intellectual disturbance, cerebral atrophy constituted to 62.5%, lacunar infarction 68.7% and pallidum lesion 50.0%. Among the moderate and serious cases of personality change, cerebral atrophy constituted 78.5%, lacunar infarction 35.0% and pallidum lesion 50.0%. Moreover, among extrapyramidal symptoms, pallidum lesion constituted 58.6%, cerebral atrophy 55.1% and lacun
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PMID:[Long-term follow-up study on sequelae of carbon monoxide poisoning; serial investigation 33 years after poisoning]. 1050 96

Erythrocytes from diabetic patients exhibit impaired viscoelastic properties when estimated by various methods. We determined erythrocyte filterability through 5-microm pores, in 51 patients with non-insulin-dependent diabetes mellitus, 18 healthy controls, 15 patients with homozygous beta-thalassemia and 15 with beta-thalassemia traits. The filtration measurements were made with a Hemorheometer, which uses the "initial flow rate" principle. To determine the Index of Rigidity (IR) of the red blood cells, we measured the passage time of white blood cell-free erythrocyte suspensions, 8% per volume, through the filter. Diabetic patients had significantly increased IR in comparison to healthy controls and to patients with beta-thalassemia trait, but not at the level found in patients with homozygous beta-thalassemia. In diabetic patients, a strong correlation between IR and the percentage of glycosylated haemoglobin was found (r=0.737, P < 0.0001), and a weaker one with serum unconjugated bilirubin (r=0.363, P=0.0097) and serum total lipids (r=0.321, P=0.0286). Patients with severe retinopathy also had significantly increased IR, in comparison to those with or without mild retinopathy. Anaemic diabetic patients, especially those with the anaemia of chronic disease, also had significantly increased IR in comparison to non-anaemic diabetics. No correlation between IR, MCV, MCH, MCHC, RDW, RBC morphology, serum LDH or the presence of erythrocyte inclusions after incubation with nitrous sodium solution was found. Our findings suggest that glycosylation of skeletal proteins probably contributes significantly to the increased membrane rigidity of diabetic erythrocytes.
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PMID:Impairment of erythrocyte viscoelasticity is correlated with levels of glycosylated haemoglobin in diabetic patients. 1148 49

Stiff-Person Syndrome (SPS) is a very rare disorder characterised by progressive fluctuating muscle rigidity and episodic spasm. So far, only two reports have demonstrated a significant clinical improvement in the patients with SPS when muscle were injected with Botulinum Toxin A (BTA). We investigated the effectiveness of intramuscular injections of BTA in a patient with clinical, biochemical and electrophysiological evidence of SPS. A 41-year-old woman with coexisting epilepsy and insulin-dependent diabetes mellitus was hospitalised in our Department because of stiffness and paroxysmal spasm of trunk and proximal limbs muscles. Because of not sufficient results of the pharmacological treatment the injections of BTA into involved muscles were done. Clinical observations included measure of pain, frequency of spasm, well-being and selection's activities were performed at baseline and in 1, 2, 7, 11, 16, 20, weeks. Significant improvement started one week after injections and lasting about 4 months was observed. Using BTA injections into involved muscles for the treatment of SPS can be followed by marked functional improvement and reducing the need for systemic drugs.
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PMID:[Botulinum toxin A in the treatment of stiff man syndrome]. 1509 44

To date, there is much evidence confirming that impaired arterial elastic properties have a direct impact on prognosis in patients with chronic renal failure, arterial hypertension, or diabetes mellitus in the general population and in the elderly. At the same time, in most cohort studies the manifestations of coronary heart disease (CHD) constitute about two thirds of developed cardiovascular events (CVE); the value of arterial stiffness after CHD manifestations has not been conclusively ascertained. There are various opinions on the mechanism and pattern of this association. Rigidity of large vessels may be a marker of cardiac artery lesion, on the one hand, and promote deterioration of myocardial ischemia in the presence of coronary atherosclerosis, on the other. This review of literature discusses the pathogenetic and clinical aspects of the impact of stiffness of the great vessels on the development of CVE.
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PMID:[Arterial stiffness as a predictor of cardiovascular events in coronary heart disease]. 2036 7

We report a case of stiff-person syndrome associated with several autoimmune diseases. A 49-year-old male with type 1 diabetes presented with a 6-month history of muscle rigidity and spasms of his upper and lower extremities. Anti-glutamic acid decarboxylase 65 antibody was elevated at 609 nmol/L. Electromyography revealed continuous motor unit activity in agonist and antagonist muscles. He responded favorably to diazepam, baclofen, and intravenous immunoglobulin infusions. This case report describes stiff-person syndrome in association with pernicious anemia and diabetes mellitus. A review of the literature discusses the diagnosis and treatment of this rare entity.
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PMID:Stiff-person syndrome: a case report and review of the literature. 2128 11


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