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)

An autopsy case with clinically and molecular genetically diagnosed Huntington's disease (HD) accompanied with minimal non-specific neuropathological features was reported. When the patient was 45 years old, he had faulty memory, mood swing, personality change and agitation. Neurological and psychiatric examinations revealed choreoathetoid movements in limbs and trunk, generalized hyperreflexia and mental deterioration. However, cerebellar ataxia and muscle rigidity were not disclosed. Neuroimaging study did not show a definite atrophy of heads of caudate nuclei. Neuroacanthocytosis and Wilson's disease were ruled out by the peripheral blood examination and serum Cu and ceruloplasmin examination. At the age of 55 he died of pneumonia. Post-mortem examination revealed minimal non-specific neuropathological features for HD (Vonsattel's grade 0), that is, no visible fibrillary gliosis in the striatum, and few neuronal loss and only proliferation of astrocytes (astrocytosis) in the striatum. Molecular-genetic study the patient's brain tissues and his youngest son's blood was performed. These studies revealed 40 CAG repeats in the patient, 56 CAG repeats in his youngest son. These results suggest they may be HD. Vonsattel et al. [ 1998] insist that grade 0 comprises 1% of all HD brains, and grade 1 comprises 4% of all HD brains. But we could not find any reports in which the clinical and neuropathological features were described in detail on the cases with clinically and molecular genetically diagnosed HD without specific pathological findings. Therefore, we present in detail the clinical and neuropathological features of such case.
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PMID:An autopsy case with clinically and molecular genetically diagnosed Huntington's disease with only minimal non-specific neuropathological findings. 1074 90

We describe an autopsy case of parkinsonism with bradykinesia, muscle rigidity, and dementia as major symptoms. The patient had developed bradykinesia at the age of 62, and then muscle rigidity, a parkinsonian posture, bradylalia, and dementia gradually appeared. Neurological examination revealed rigidity in the neck and limbs, with motion and speech being generally slow. He lacked involuntary movements including alien hand, tremor, chorea, and dystonia. Vertical gaze palsy, both upward and downward was noted, but other cranial nerves were intact. He was diagnosed as suffering from PSP clinically based on vertical gaze palsy, bradykinesia, instability on standing and gait, and dementia. Levodopa was only transiently effective. Within three years he became bed-ridden and in a state of akinetic mutism. At age 65 he died from pneumonia. Neuropathology revealed severe neuronal degeneration and gliosis in the substantia nigra. Because atrophy of the tegmentum of brainstem, dentate nuclei, inferior olivary nuclei was very mild and Alzheimer neurofibrillary tangles in the brainstem were relatively few, PSP was ruled out. Cortical neuronal degeneration was not apparent, but in the deep layer of cingulate gyrus, frontal lobe, and insula, there were several ballooned neurons. Gallyas-Braak silver staining showed no tuft-shaped astrocytes, specific for PSP, but it disclosed astrocytic plaques in the basal ganglia and the cerebral cortex. At present, astrocytic plaques are recognized as a hallmark of corticobasal degeneration (CBD), along with ballooned neurons in the cerebral cortex. The present case thus illustrates that CBD has a wide spectrum and may include cases in which degeneration of cerebral cortex is very mild.
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PMID:[An autopsy case of corticobasal degeneration without prominent cortical pathology--an imitator of progressive supranuclear palsy]. 1096 56

Tetanus is an acute neurological disease characterized by muscle rigidity and spasms, autonomic dysfunction and in severe forms requires respiratory and hemodynamic support. Though it is entirely preventable by immunization, it still occurs in developing countries causing significant morbidity and mortality. Intensive care management of tetanus is fraught with problems of ventilator-associated pneumonia, nosocomial sepsis and a variety of other complications. Various treatment protocols have been tried in managing diverse manifestations of severe tetanus but the consensus is yet to emerge. In this review we have discussed the pathophysiology, clinical features and management controversies and suggest on basis of our experience use of high dose diazepam (20-120 mg/kg/day) and vecuronium with mechanical ventilation if required for control of spasms, and early detection of autonomic dysfunction and use of propranolol, in our circumstances.
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PMID:Post-neonatal tetanus: issues in intensive care management. 1133 25

Rhabdomyolysis is a disorder characterized by skeletal muscle injury and fatal complications at times. The causes of rhabdomyolysis are usually traumatic and non-traumatic, such as neuroleptic malignant syndrome and rhabdomyolysis associated to septicemia. The cases of 2 schizophrenic patients with rhabdomyolisis during pneumonia infection and neuroleptic therapy are reported. At admission, both patients had important respiratory distress and hyperthermia; the clinical conditions required endotracheal intubation. Blood and urine cultures were always negative, while the bronchial sputum culture was positive. The diagnosis of rhabdomyolysis was confirmed by myoglobinemia dosage and ortholuidine test. Pneumonia infection was treated with antibiotic specific therapy whereas renal failure was treated with adequate hydratation and strained diuresis. The absence of muscle rigidity, the improvement of X-r images and the reduction of corporeal temperature, during antibiotic treatment, excluded neuroleptic malignant syndrome. The impro-vement allowed extubation and discharge of the patients from intensive care unit. In both cases neuroleptic malignant syndrome was excluded, therefore rhabdomyolysis was the consequence of pneumonia infection or of a combination of factors capable to cause an important damage of skeletal muscles.
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PMID:[Rhabdomyolysis associated with respiratory infection in chronic psychiatric patients during neuroleptic treatment]. 1456 56

Neuroleptic malignant syndrome (NMS) is the most dangerous side effect of phenothiazines therapy. In the period of time from 1995 to 2002 in the Intensive Toxicological Unit there were five patients, 3 men and 2 women, aged from 25 to 62 (average 44.2) years-old, admitted from the regional inpatients psychiatric units with the diagnosis of pneumonia and/or sepsis. The patients about 48-72 hours before admittance were given some phenotiazine derivatives (promazine, perphenazine, clozapine, pipamperon) and/or buthyrophenone (haloperidol) because of psychotic state. Altered consciousness, muscle rigidity, hyperpyrexia (39.0-41.0 degrees C), sweating, tachycardia (120-150/min.), tachypnoea (respiratory rate more than 25/min.) and high level of creatine kinase activity (23,751-112,288 U/l) dominated. Only one patient had clinical picture of pneumonia. Because of the rapid development of acute respiratory failure, respirathorotherapy was initiated and continued for 8 and 10 days in two patients respectively. Transient thrombocytopenia (26,000/microliter) in one subject was observed. The neuroleptic drug was withdrawn and intensive supportive care with administration of bromocriptine (15-20 mg/24 h) was provided. None one of the doctors told the patients about the possibility of NMS during phenothiazines therapy.
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PMID:[Neuroleptic malignant syndrome]. 1456 9

A 27-year-old woman with schizophrenia showed signs of neuroleptic malignant syndrome with disturbed consciousness, high fever, muscle rigidity, and autonomic dysfunction (including tachycardia and enhancement of saliva secretion). Since the age of 15, she had been treated at a local psychiatric clinic with a diagnosis of schizophrenia. On the day she was brought to the emergency room, she was asleep in the morning, but tachycardia was observed in the evening in the absence of consciousness. The patient was brought to our hospital by ambulance. It was revealed that she had taken a massive dose of chlorpromazine hydrochloride in the morning on the same day. On arrival, the Japan coma scale, pulse, respiratory rate, body temperature, and Sp(O2) were 300, 114 beats x min(-1), 26 breaths x min(-1), 39.0 degrees, and 91% (room air), respectively. The CPK level was 1,776 IU x l(-1). Sp(O2), bilateral pneumonia, and right atelectasis improved 2 hours after admission. Endotracheal intubation was performed for artificial respiration. Salivation, marked sweating, and rigidity of the limbs were noted. Under a diagnosis of neuroleptic malignant syndrome, dantrolene was administered. For pneumonia, ceftriaxone and pazufloxacin were administered. The consciousness became clear 2 days after admission. The patient was discharged 10 days after admission.
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PMID:[Comatose patient with neuroleptic malignant syndrome brought by ambulance]. 2233 65