Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0040822 (tremor)
 18,428 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 64-year-old Japanese woman who died one year after the onset of progressive gait disturbance and dementia. She noted a difficulty in holding a glass and hand tremor in June of 1996 when she was 63 years old. In July of 1996, she tended to lean toward left when she walked. She also noted truncal titubation. In November of 1996, she started to have visual hallucination and delusion in which she said "I see something is flying on the wall.", "Somebody has come into my room", and things like that. She was admitted to our service on November 22, 1996. On admission, she was alert and general physical examination was unremarkable. Neurologic examination revealed disturbance in recent memory. Hasegawa's dementia rating scale was 22/30. She showed vivid visual hallucination with colors in which she saw faces of dwarfs and angels, a space ship, and others. Higher cerebral functions were normal. She showed left oculomotor palsy which was a sequel of an aneurysm and subarachnoid hemorrhage nine years before. Otherwise cranial nerves were unremarkable. She showed ataxic gait, limb ataxia, truncal titubation, and postural hand tremor. She had no weakness and no muscle atrophy. Deep tendon reflexes were within normal limits. Plantar response was flexor. Sensation was intact. Laboratory examination was also unremarkable. Complete survey for occult malignancy was negative. CSF was under a normal pressure and cell count was 1/microliter, total protein 27 mg/dl, and sugar 68 mg/dl. Cranial CT scan was unremarkable. MRI was not obtained because of the presence of an aneurysm clip in the left internal carotid-posterior communication artery junction. She showed progressive deterioration in her mental function. By January 1997, she became unable to stand or walk with marked dementia. Repeated CSF exams and cranial CT scans were unremarkable. She suffered from several episodes of aspiration pneumonia. A trial of three days methylprednisolone pulse therapy was given starting on March 7, 1997, which was of no effect on her neurologic status. On March 28, 1997, she was intubated because of acute respiratory distress syndrome. In April 2, her body temperature rose to 38 degrees C. On April 9, 1997, her blood pressure dropped and resuscitation was unsuccessful. She was pronounced dead on the same day. The patient was discussed in a neurologic CPC and the chief discussant arrived at the conclusion that the patient had primary leptomeningeal lymphoma. Other possibilities entertained among the audience included brain stem encephalitis of unknown type, carcinomatous cerebellar degeneration plus limbic encephalitis, Creutzfeldt-Jakob disease, thalamic degeneration, and progressive multifocal leukoencephalopathy. Post-mortem examination revealed thickening and clouding of the leptomeninges; Gram-positive diplococci were found in the leptomeninges. This meningitis appeared to have been an complication in the terminal stage of her illness. Microscopic examination revealed astrocytosis in the midbrain tegmentum. Cerebral cortices showed only mild astrtocytosis. No cerebellar atrophy was seen and Purkinje cells were retained which excluded paraneoplastic cerebellar degeneration. Neuropathologic diagnosis was bacterial meningitis, however, the presence of brain stem encephalitis prior to the onset of bacterial meningitis could not be excluded. It is interesting to note that the diagnosis of the primary neurologic disease of this patient was not easy even after autopsy. As autopsy permission was obtained only for the brain, it was not clear whether or not this patient had an occult malignancy somewhere in her body, however, there was no evidence to indicate paraneoplastic degeneration of the central nervous system. As the patient did not have meningeal signs until one month before her death, it is difficult to ascribe her entire neurologic problems to her meningitis. Finally, her visual hallucination was vivid and colorful; we thought this might have been
 ...
PMID:[A 64-year-old woman with progressive gait disturbance and dementia for one year]. 978 11

We report a patient with familial myoclonus showing an extremely benign clinical course. The patient was a 70-year-old woman, who first noticed shaking of hands at age of 25. The symptom did not worsen for more than 40 years. She visited our hospital at the age of 70 because of disturbance in chores because of worsening of her hand shaking in the past one year. A family history showed that 4 members had similar symptoms and that the two were afflicted with fits of loss of consciousness. On neurologic examination, rhythmic myoclonic jerks were noted in all the extremities, more in the upper limbs, both at rest and during action. Tandem gait was mildly disturbed. The remainings of neurologic examination were normal. SEP and jerk-locked back averaging provided evidence of cortical myoclonus. EEG showed multifocal polyspike discharges. Gene analysis for DRPLA, pyruvate and lactate levels in serum and the cerebrospinal fluid, serum amino acid levels, and CSF HVA and 5-HIAA levels were all normal. No brain atrophy was noted in cranial MRI. Myoclonus was markedly reduced after administration of clonazepam. The clinical features and electrophysiological data of our patient are consistent with the clinical diagnosis of familial essential myoclonus and epilepsy/benign adult familial myoclonic epilepsy.
 ...
PMID:[A case of familial myoclonus showing extremely benign clinical course]. 980 89

We report a case of dystonia with a partial deletion of the short arm (p) of chromosome 18 and androgen insensitivity. Neurologic findings in the 18p syndrome are reported to include mental retardation, seizures, incoordination, tremor, and chorea. A 15-year-old girl with a denovo 18p deletion [karyotype 46, XY, del (18)(p11.1)] developed progressive asymmetric dystonia. She had oromotor apraxia and partial expressive aphasia since childhood, and she was able to partially communicate through elementary sign language. At the age of 15 years, she developed subacute and progressive choreic movements of the right arm, severe dystonic posturing of the left arm, and spastic dystonia in both legs. Her response to parenteral or oral benzodiazepines, oral trihexyphenidyl, benztropine mesylate, baclofen, and L-dopa were brief and inadequate. The response to intrathecal baclofen has been sustained over 18 months. In all likelihood, the 18p deletion syndrome affecting this patient is significant in the pathogenesis of her acquired dystonia. Chronic intrathecal baclofen therapy via pump has been effective in this case and should be considered as a treatment modality in carefully selected patients with dystonia.
 ...
PMID:Progressive dystonia in a child with chromosome 18p deletion, treated with intrathecal baclofen. 1007 26

Utilization of thoracic pedicle screws is controversial, especially in the treatment of scoliosis. We present a case of a 15-year-old girl seen 6 months after her initial surgery for scoliosis done elsewhere. She complained of persistent epigastric pain, tremor of the right foot at rest, and abnormal feelings in her legs. Clinical examination revealed mild weakness in the right lower extremity, a loss of thermoalgic discrimination, and a forward imbalance. A CT scan revealed at T8 and T10 that the right pedicle screws were misplaced by 4 mm in the spinal canal. At the time of the revision surgery the somatosensory evoked potentials (SSEP) returned to normal after screw removal. The clinical symptoms resolved 1 month after the revision. The authors conclude that after pedicle instrumentation at the thoracic level a spinal cord compression should be looked for in case of subtle neurologic findings such as persistent abdominal pain, mild lower extremity weakness, tremor at rest, thermoalgic discrimination loss, or unexplained imbalance.
 ...
PMID:Unusual presentation of spinal cord compression related to misplaced pedicle screws in thoracic scoliosis. 1561 22

We report on two patients with unilateral hyperkinetic movement disorders associated with contralateral hemiparesis and ipsilateral basal ganglia lesions. The first patient, a 47-year-old woman, had a low-grade astrocytoma located in the right basal ganglia extending into the subthalamic area and the cerebral peduncle. She presented with left hemiparesis, right hemichorea, and intermittent right-sided tremor at rest. The second patient, a 85-year-old woman, had hypertensive hemorrhage to the right posterior basal ganglia, the posterior limb of the internal capsule, the lateral thalamus, and the subthalamic region with accompanying intraventricular bleeding. She developed right-sided transient hemichorea-hemiballism. A videotape illustration of one of the patients is provided. The literature on the rare occurrence of ipsilateral hemichorea-hemiballism is discussed and possible pathomechanisms are reviewed. We postulate that hemiparesis contralateral to basal ganglia lesions might have a conditioning effect on the appearance of ipsilateral dyskinetic movement disorders.
 ...
PMID:Hemichorea and hemiballism associated with contralateral hemiparesis and ipsilateral basal ganglia lesions. 1034 77

A 16-year-old female presented to an emergency department with complaints of nausea and vomiting, dehydration, increasing weakness, and resting tremor. The past history included mild exercise-induced asthma. She denied sexual activity, but a urine test for beta-human chorionic gonadotropin was positive and she was transferred to a gynecology service for management of pregnancy. She also had primary amenorrhea and delayed growth for age. Further complaints included headaches accompanied with worsening of visual activity. Pelvic ultrasound revealed no intrauterine or ectopic pregnancy. Head CT scan showed a suprasellar tumor, better defined on an MRI as a hypothalamic tumor. Pathology following partial tumor resection revealed a mixed germ cell tumor with negative metastatic work-up.
 ...
PMID:Another Positive Pregnancy Test. 1035 84

Stereotactic thalamotomy has been used with some benefit in the treatment of essential tremor. We report a 73-year-old woman whose essential tremor of the right hand spontaneously disappeared after thalamic infarction. She had suffered hand tremor of the right hand for seven years. One morning, she noticed mild muscular weakness in her right upper and lower extremities, numbness around her mouth and paresthesia in her right arm. Simultaneously, she noticed disappearance of the tremor of her right hand. Several days later, right hemiplegia and paresthesia completely resolved. Neurological examination revealed no postural tremor or resting tremor. T 2-weighted brain MR imaging showed a high-intensity signal in the left thalamus that involved the ventralis intermedius nucleus. Clinical recovery from the effect of the infarct on essential tremor was complete. Therefore, it seems that thalamic infarction in this patient had an effect on essential tremor similar to that achieved with thalamotomy.
 ...
PMID:[Disappearance of essential tremor after thalamic infarction]. 1039 Oct 77

We report a 60-year-old woman with juvenile Parkinson disease (PD) with vocal cord abductor paralysis (VCAP). She had suffered from juvenile PD for 30 years. She was admitted in February 1998 to our clinical unit, because of malignant syndrome induced by dehydration. Neurological examination revealed disturbance of consciousness, hand tremor, dyskinesia of the trunk and all extremities, and rigidity. Laboratory examinations disclosed leukocytosis, renal dysfunction, hypermyoglobinemia, and elevation of the serum creatine kinase. Six days after admission, dyspnea and inspiratory stridor were noted, and the respiratory distress worsened. Endoscopy of the upper airways revealed that the vocal cord was in the midline or paramedian position. There are some cases of PD with VCAP, but such a case is very rare in Japan. We discussed the pathogenic mechanisms of these conditions, and speculated that VCAP was associated with malignant syndrome in our case.
 ...
PMID:[A case of juvenile Parkinson disease with vocal cord abductor paralysis in the course of malignant syndrome]. 1039 Oct 85

We report an 80-year-old Japanese woman who presented levodopa-responsible parkinsonism followed by progressive dementia. She was well until her 61 years of age (in 1978) when she noted onset of resting tremor in her right hand followed by tremor in her right leg. She was treated with levodopa and trihexyphenidyl with good response, however, later on, she suffered from gait disturbance. In 1985, she had an episode of cardio-pulmonary arrest from which she was resuscitated, however, she started to show hypermetamorphosis, memory defect, and aggressive behaviors. She also developed motor fluctuations and dyskinesias from levodopa. She was admitted to our service in 1986; she showed rather typical parkinsonism and mild dementia. She received left Vim thalamotomy in the same year. Her dyskinesias improved, however, her gait disturbance became progressively worse. In 1995, she was admitted to our service again; she showed marked dementia and advanced parkinsonism; she was unable to walk unsupported. She became bedridden in 1996 and gastrostomy was placed. She was transferred to Zushi Aoki Hospital. Her dementia became progressively worse, and she was in the akinetic and mute state. She expired on April 22, 1998. She was discussed in a neurological CPC. The chief discussant arrived at a conclusion that the patient had Parkinson's disease with complication by Alzheimer's disease in her later clinical course. The diagnoses of participants were divided among Parkinson's disease with dementia, Parkinson's disease and Alzheimer's disease, and diffuse Lewy body disease. Postmortem examination revealed marked neuronal loss in the substantia nigra and the locus coeruleus. Lewy bodies were found in the substantia nigra. In addition, rather many Lewy bodies of cortical type were seen in the cingulate gylus, inferior temporal gylus, and in the amygdaloid nucleus. These Lewy bodies were positive for alpha-synuclein. Also, tau-positive intra-neuronal tangles were seen in the hippocampus and in the substantia nigra. The Meynert nucleus showed marked neuronal loss. Pathologic findings were consistent with the diagnosis of diffuse Lewy body disease.
 ...
PMID:[An 80-year-old woman with parkinsonism and progressive dementia]. 1042 59

A previously healthy 15-year-old female was admitted to our hospital complaining of nausea and vomiting. She did not complain of diarrhea. A physical examination revealed a lower right quadrant abdominal tenderness without rebound or spontaneous pain and a knocking pain of the costovertebral angle. A high fever, knocking pain of costovertebral angle, and urinary findings including Gram's stain, lead us to suspect a urinary tract infection, cefotiam was administered intravenously. Spiking fever with shaking chills continued for three days, and three sets of blood cultures were positive for Salmonella Oranienburg, but her urine culture was negative. Her history was taken again, revealing an intake of a processed squid product. The product was confirmed by the local public health center to be Salmonella Oranienburg. Finally food poisoning by Salmonella Oranienburg with sepsis was diagnosed. With cefotiam she became better and was discharged from the hospital on the 10th hospital day. During admission to the hospital she did not experience any diarrhea, and her stool culture was negative. Epidemics of Salmonella Oranienburg food poisoning are relatively rare in the literature. In Japan, one has arisen as a result of contamination of a processed squid product in March 1999. However, there have been no cases without so-called gastroenteritic symptoms (abdominal pain and diarrhea) who were previously healthy and developed sepsis caused by Salmonella Oranienburg, reported in Japan. Even in previously healthy patients, with an epidemic situation of non-typhoidal salmonellosis, salmonella sepsis must be ruled out. Among such cases, those who present with spiking fever and shaking chills should be given antibiotic therapy after taking appropriate cultures.
 ...
PMID:[Sepsis due to Salmonella Oranienburg--a case report]. 1048 26


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>