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PubMed2000

Outcome of children in the indomethacin intraventricular hemorrhage prevention trial.

BACKGROUND: For preterm infants, intraventricular hemorrhage (IVH) may be associated with adverse neurodevelopmental outcome. We have demonstrated that early low-dose indomethacin treatment is associated with a decrease in both the incidence and severity of IVH in very low birth weight preterm infants. In addition, we hypothesized that the early administration of low-dose indomethacin would not be associated with an increase in the incidence of neurodevelopmental handicap at 4.5 years of age in our study children. METHODS: To test this hypothesis, we provided neurodevelopmental follow-up for the 384 very low birth weight survivors of the Multicenter Randomized Indomethacin IVH Prevention Trial. Three hundred thirty-seven children (88%) were evaluated at 54 months' corrected age, and underwent neurodevelopmental examinations, including the Wechsler Preschool and Primary Scale of Intelligence-Revised (WPPSI-R), the Peabody Picture Vocabulary Test-Revised (PPVT-R), and standard neurologic examinations. RESULTS: Of the 337 study children, 170 had been randomized to early low-dose indomethacin therapy and 167 children had received placebo. Twelve (7%) of the 165 indomethacin children and 11 (7%) of the 158 placebo children who underwent neurologic examinations were found to have cerebral palsy. For the 233 English-monolingual children for whom cognitive outcome data follow, the mean gestational age was significantly younger for the children who received indomethacin than for those who received placebo. In addition, although there were no differences in the WPPSI-R or the PPVT-R scores between the 2 groups, analysis of the WPPSI-R full-scale IQ by function range demonstrated significantly less mental retardation among those children randomized to early low-dose indomethacin (for the indomethacin study children, 9% had an IQ <70, 12% had an IQ of 70-80, and 79% had an IQ >80, compared with the placebo group, for whom 17% had an IQ <70, 18% had an IQ of 70-80, and 65% had an IQ >80). Indomethacin children also experienced significantly less difficulty with vocabulary skills as assessed by the PPVT-R when compared with placebo children. CONCLUSIONS: These data suggest that, for preterm neonates, the early administration of low-dose indomethacin therapy is not associated with adverse neurodevelopmental function at 54 months' corrected age.

Anti-Inflammatory Agents, Non-Steroidal↗

PubMed2005

New practice parameters: what does the evidence say?

The American Academy of Neurology recently has published two practice parameters pertaining to children and adolescents with headache. The first focuses attention on the appropriate evaluation of the child with recurrent headache and reviews the data regarding laboratory investigation, electroencephalography (EEG), and neuroimaging. Routine laboratory investigation and EEG are not recommended. Neuroimaging is not recommended for children with recurring headaches and a normal neurologic examination unless there are historic features to suggest the recent onset of severe headache, change in the type of headache, or associated features that suggest neurologic dysfunction. If there are abnormalities on the neurologic examination (eg, focal findings, signs of increased intracranial pressure, significant alteration of consciousness) or the coexistence of seizures, then neuroimaging is recommended. The second parameter evaluates relevant data regarding the acute and preventive therapies for pediatric migraine.

Adolescent↗

PubMed2004

Subcutaneous and cerebral cysticercosis.

Cysticercosis is a human infestation, which is considered the most common cause of seizures worldwide. The subcutaneous lesions can help in the diagnosis of neurocysticercosis. We describe a case of a 45-year-old patient with multiple cutaneous nodules first seen 2 years ago that were increasing in number, and normal neurologic and fundoscopic examination. Neurologic symptoms started 3 months before hospital admission as a mild headache and muscular weakness. The imaging examinations showed a massive central nervous system involvement. Physicians must be aware of the importance of subcutaneous nodule examination for the diagnosis of neurocysticercosis.

Animals↗

PubMed2006

Primary postural instability: a cause of recurrent sudden falls in the elderly.

Elderly patients with recurrent falls are frequently diagnosed with an extrapyramidal syndrome. This study aims to characterise a distinct group of patients with recurrent falls and postural instability as a hallmark of the clinical examination. The study took place in the Movement Disorders Unit, Rabin Medical Center, Petah Tiqva, Israel among 26 patients with recurrent falls who had no clinical evidence of a neurodegenerative disease. Medical records, neurological examination and brain imaging studies were assessed. Falls in these patients were sudden, unprovoked, with no vertigo or loss of consciousness. All had postural instability with minimal or no abnormality on the neurological examination. Brain imaging showed diffuse ischaemic changes in 65%. [(123)I]-FPCIT SPECT with the dopamine transporter ligand, performed in five patients, was normal in all. Recurrent falls might be caused by a neurological syndrome that primarily affects balance control. The importance of identifying this disorder is its distinction from other parkinsonian syndromes causing falls.

Accidental Falls↗

PubMed1995

An evaluation of motor-evoked potentials for detection of neurologic injury with correction of an experimental scoliosis.

STUDY DESIGN: Controlled correction of scoliosis in a rat model was used to assess the accuracy of intraoperative motor-evoked potential monitoring. OBJECTIVES: The purpose of this study was to develop a model in which motor-evoked potential changes could be compared with neurologic function after surgery, such that a threshold for responding to motor-evoked potential changes may be established. SUMMARY OF BACKGROUND DATA: Intraoperative motor-evoked potential monitoring has become technically feasible. Clinical application now depends on the development of useful interpretation parameters and correlation with neurologic sequelae. METHODS: Experimental scoliosis was produced in 30 rat pups. After growth, the rats underwent correction of their scoliosis by distraction. Changes in tcMMEP onset latency and amplitude were measured. Distraction was applied either until a 10% delay in tcMMEP onset latency (Group 1), until tcMMEP responses were ablated (Group 2), or for 10 minutes after the loss of transcranial magnetic stimulation response (Group 3). RESULTS: In Group 1 (n = 10), all animals had tcMMEP with normal onset latency and normal neurologic examinations 24 hours after surgery. In Group 2 (n = 10), tcMMEP were normal in four rats, markedly delayed in three rats, and absent in three rats 24 hours after surgery. neurologic examination was normal in the four rats with normal tcMMEP. Moderate deficit was noted in two of the three rats with prolonged onset latency 24 hours after surgery; the third was intact. Moderate neurologic injury was noted in two of three rats with absent tcMMEP 24 hours after surgery; the third rat was paralyzed. In Group 3 (n = 10), vertebral dislocation was noted on lateral radiographs in eight of 10 animals. Twenty-four hours after surgery, tcMMEP remained absent, and paralysis was noted in the eight rats with dislocation. The two rats without dislocation had delayed tcMMEP but some return of neurologic function. CONCLUSIONS: Comparison of the three groups shows a significant correlation between tcMMEP and endpoint neurologic outcome. None of the rats in Group 1 had a neurologic deficit after surgery as opposed to five of 10 rats in Group 2 and 10 of 10 rats in Group 3 with significant neurologic injury. These findings suggest that a 10% delay in onset latency would be an appropriate threshold for responsing to changes in tcMMEP.

Analysis of Variance↗

PubMed1998

Epidemiology of stroke in the elderly in Thailand.

Epidemiology of stroke in the elderly in Thailand was conducted from August 1994 to October 1996. The total of 3,036 Thai elderly were included in this study. They represented the elderly population from four regions; Central Region (615 elderly, Nakhon Pathom Province), Northern Region (840 elderly, Lampang Province), North-Eastern Region (706 elderly, Sakon Nakhon Province), and Southern Region (857 elderly, Ranong Province). All elderly in these selected areas received general physical examinations and complete neurological examinations from neurologists. Demographic data concerning each individual was recorded by specially trained nurses. Data included age, sex, occupation, education, drug usage, alcohol, smoking and pre-existing diseases. Blood was taken from each subject for complete blood count, fasting blood sugar, cholesterol, triglyceride, high density lipoprotein cholesterol and VDRL. Data on physical examinations were recorded with particular attention to blood pressure, carotid bruit, cardiac murmurs, cardiac arrhythmia, speech, posture, gait, frontal lobe releasing signs, Babinski sign and focal neurological deficit. Thirty-four stroke patients were identified from 3,036 elderly (prevalence rate of 1.12 per cent). There were 12 stroke patients from Central Region (prevalence rate of 1.99 per cent), 5 from Northern Region (0.6 per cent), 4 from North-Eastern Region (0.6 per cent) and 13 from Southern Region (1.5 per cent). Hypertension was the main risk factor for stroke in this study whereas diabetes mellitus, smoking, alcohol consumption, hyperlipidemia and underlying heart diseases were insignificant risk factors. The prevalence of hypertension in Thai elderly was ranging from 16.7 to 47.2 per cent (criteria over 140/90 mmHg) or 6.1 to 24.8 per cent (criteria over 160/90 mmHg). Prevalence of smoking and alcohol consumption in Thai elderly ranged from 19.5 per cent (Sakon Nakhon) to 62.1 (Lampang) and 16.75 per cent (Nakhon Pathom) to 33.70 per cent (Lampang) respectively. Data from physical examinations revealed that dysarthria, hemiplegic gait and Babinski sign were the significant signs for diagnosis of stroke in the community setting. The prevalence of carotid bruit, cardiac murmur and cardiac arrhythmia were ranging from 1.3 to 1.8 per cent, 3.1-7.1 per cent and 0.8-1.4 per cent respectively. From this study, it can be concluded that stroke prevention is the best policy for stroke management. Stroke prevention measures should thus be aimed at the high risk elderly group. This is best achieved by identifying risk factors among them and then controlling these risk factors properly.

Aged↗

PubMed2002

Intrathecal thiotepa: reappraisal of an established therapy.

PURPOSE: Intrathecal thiotepa is recommended as a treatment of leptomeningeal metastases (LM) in children, although published data to support this approach are limited. The authors sought to determine the efficacy of intrathecal thiotepa for pediatric LM. PATIENTS AND METHODS: The authors reviewed all children treated with intrathecal thiotepa for LM at two tertiary children's hospitals, assessing outcome by cerebrospinal fluid cytology, neuroimaging, neurologic examination, and overall survival rate. RESULTS: Fifteen children with LM evidenced by malignant cells in the cerebrospinal fluid (mean age 7.3 years; five medulloblastoma, one anaplastic astrocytoma, one glioblastoma, one retinoblastoma, one neuroblastoma, two rhabdomyosarcoma, one non-Hodgkin lymphoma, two acute lymphoblastic leukemia, and one acute myelogenous leukemia) were treated with intrathecal thiotepa at 5 to 11.5 mg/m2 per dose for two to seven doses. Five children received concomitant craniospinal irradiation; 12 received simultaneous systemic or other intrathecal chemotherapy, or both. Four children experienced clearance of malignant cells from the spinal fluid, but this response was sustained in only two. All four children with cytologic response received concurrent radiotherapy, chemotherapy, or both. No patients showed partial or complete response on neuroimaging. Only one child had improvement on the neurologic examination; six were unchanged and eight had worsening neurologic signs. Median survival was 15.1 weeks, with a 1-year overall survival rate of 26.7% (standard error 11.4%). CONCLUSIONS: The unfavorable outcomes observed suggest that intrathecal thiotepa adds little to combination therapy for pediatric LM.

Adolescent↗

PubMed1986

Early predictors of neurodevelopmental outcome of very low-birthweight infants at three years.

Seventy-nine premature infants weighting less than 1501 g at birth but appropriate for gestational age underwent a neurodevelopmental examination at one, three, six, nine and 12 months post-term, and a standard neurological examination and the Stanford-Binet Intelligence Scale at three to four years of age. Children were classified as normal, suspect or abnormal on the three-year neurological examination, on the IQ test, and on composite neurodevelopmental outcome at age three years. Results showed that items from both the nine- and 12-month neurodevelopmental examinations correctly classified about 80 per cent of the children as to composite outcome at three years. In addition, the 12-month examination enabled correct prediction for 89 per cent of the children as to neurological outcome and for 82 per cent as to IQ. Neurodevelopmental examination of high-risk infants in the last quarter of the first year of life (post-term) should assist pediatricians in predicting which children will be normal and which will require early intervention.

Cerebral Palsy↗

PubMed1996

[A 63-year-old woman with muscle weakness, myotonia, and parkinsonism].

We report a 63-year-old woman who presented myotonia and parkinsonism. The patient was well until 15 years of the age when she noted that the ring finger of her left hand at times flexed when she did not intend to do so. She noted weakness in her left upper extremity at the age of 40, and difficulty in relaxing her hand grip at 45. She had an onset of tremor in her right foot at age 50, which was followed by difficulty in gait and hand writing. She was admitted to Juntendo University Urayasu Hospital when she was 63-year-old. Her mother, two sisters, and a son were affected with similar muscle weakness and myotonia. Although some of them developed stooped posture in the late stage of the disease, none of them had overt parkinsonism. General physical examination was unremarkable. Neurologic examination revealed an alert and oriented woman with some recent memory loss. She had bilateral ptosis, facial weakness, and a masked face. Myerson's sign was present. Her speech was small and monotonous. The sternocleidomastoid muscles were markedly atrophic and weak. The remaining of the cranial nerves were intact. She walked in small steps with freezing with support. She showed bradykinesia, retropulsion, and resting tremor in her right leg. Slight distal dominant weakness was noted in both upper and lower extremities more on the left. No cerebellar signs were noted. Muscle stretch reflexes were within normal limits in the upper extremities and diminished in the lower limbs. Sensation was intact. Routine laboratory findings were unremarkable. Cranial CT scan and MRI revealed slight cortical atrophy and leukoaraiosis. She responded to levodopa and she became able to walk by herself. She was transferred to another hospital one month after her admission. She had several bouts of airway obstruction with one episode of respiratory arrest. She expired six month after the transfer. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that this patient suffered from myotonic dystrophy and Parkinson's disease which set in later years. Postmortem examination on the iliopsoas muscle revealed uneven muscle fiber diameters, central nuclei, and type 1 fiber predominance; the pathologic finding was consistent with myotonic dystrophy. The substantia nigra showed marked cell loss and Lewy bodies in the remaining neurons. The finding was consistent with Parkinson's disease. In myelin stain, diffuse myelin pallor was noted in the cerebral white matter which was the pathologic substrate of leukoaraiosis in this patient. Combination of these two disorders have never been reported in the literature to our knowledge. It appears to be that the coincidence is just a by-chance phenomenon, but it seems interesting to note that accelerated aging process appears to be present in both myotonic dystrophy and Parkinson's disease.

Diagnosis, Differential↗

PubMed1992

Computed tomography in the initial evaluation of patients with blunt trauma.

Two hundred sixty-six of 374 consecutive blunt trauma patients underwent emergency computed tomographic (CT) scanning during evaluation at a level I trauma center. The purpose of this study was to develop guidelines for use of CT scanning in the initial evaluation of blunt trauma patients. Of the 131 CT scans of the head obtained, 20 (15%) had positive results. Seven patients whose initial neurologic examinations were normal had abnormal results on head CT scans; none required emergent treatment of their head injury. This suggests that, in the presence of a normal neurologic examination, head CT scans can safely be delayed until other more serious injuries are addressed. Twenty-six CT scans of the chest were performed and ten (38%) were interpreted as abnormal. Chest CT scans provided information about the extent of the injury but did not alter the initial management of any patient and therefore are rarely indicated in the acute evaluation of trauma patients. A total of 110 abdominopelvic CT scans were performed and 20 (19%) were interpreted as positive. Seventy-five percent of those patients with positive CT scans were treated successfully in a nonsurgical fashion.

Chicago↗

PubMed1991

[Bilateral traumatic hemorrhage in the basal ganglia: report of two cases].

Hematomas of the basal ganglia in head injury have long been recognized by pathologists with an interest in head injury but their mechanism has not been revealed clearly. We report two cases of bilateral traumatic hemorrhage in the basal ganglia. Case #1, a 17-year-old male was admitted to our hospital immediately after a traffic accident. Neurological examination revealed that the patient was comatose and had right hemiparesis. CT scan showed bilateral hemorrhage of the basal ganglia and subarachnoid hemorrhage in the perimesencephalic cistern. MRI showed high signal intensity areas in the bilateral basal ganglia, perimesencephalic cistern, cerebral white matter and corpus callosum. The patient was diagnosed as having diffuse axonal injury coinciding with bilateral hemorrhage of the basal ganglia. Stereotactic aspiration for the hematoma of the left basal ganglia was carried out. Case #2, a 75-year-old male was admitted immediately after falling from the roof of his house. Neurological examination revealed no neurological deficit except for headache and nausea. CT scan on the day of injury revealed no abnormality. But CT scan 12 hours following the injury showed bilateral hemorrhage of the basal ganglia. Blood pressure of the patient was within normal range and he was diagnosed as having traumatic bilateral intracerebral hematoma. Conservative treatment was carried out and the patient was discharged 7 days after injury with no neurological deficit. The mechanism of traumatic hemorrhage of the basal ganglia has not been clear. In case #1, diffuse axonal injury (DAI) may have played an important role in the bilateral hemorrhage. But in case #2, non-DAI factor such as vasoparalysis syndrome may have existed.(ABSTRACT TRUNCATED AT 250 WORDS)

Accidental Falls↗

PubMed1993

[A case of primary intracranial malignant lymphoma presenting opsoclonus-polymyoclonia syndrome].

A 46-year-old woman was doing well until December 1989, when she noted vertigo and difficulty in walking. She was admitted to our department on February, 7, 1990. General physical examination was unremarkable. Neurological examination revealed opsoclonus, limbs and truncal ataxia, and myoclonus over the facial muscle, neck, and all the extremities, suggestive of opsoclonus-polymyoclonia syndrome. Other neurological finding was not apparent. T2 weighted MRI (Siemens 1.5 Tesla) showed abnormal high intensity area without mass effect at the dentate nucleus of left cerebellum. She was treated with 60 mg of oral prednisolone, followed by gradual improvement of her neurological signs and abnormal MRI findings. However, in May, she gradually developed right hemiparesis, consciousness disturbance and pseudobulbar palsy. MRI showed multiple abnormal intensity area at left frontal lobe, right basal ganglia, and right cerebellar hemisphere. Open brain biopsy from the left frontal lesion revealed malignant lymphoma (diffuse large cell type, B cell type). She was treated by radiation therapy at the dose of 50 Gy (whole brain 40 Gy, local 10 Gy) with subsequent disappearance of opsoclonus, myoclonus, and ataxia. To our knowledge, this is the first case of primary intracranial malignant lymphoma presenting opsoclonus-polymoclonia syndrome.

Brain Neoplasms↗

PubMed1999

Quantitative assessment of subclinical spasticity in human T-cell lymphotropic virus type I infection.

OBJECTIVE: To compare human T-cell lymphotrophic virus type I (HTLV-I) seropositive and seronegative women for symptoms and signs of spasticity. BACKGROUND: Infection with HTLV-I causes tropical spastic paraparesis/ HTLV-I-associated myelopathy (TSP/HAM). Certain populations, including female commercial sex workers (FSW), are at increased risk of developing this infection. Fewer than 5% of HTLV-I-seropositive persons develop TSP/HAM, which is typically associated with spasticity. METHODS: Cross-sectional study of 255 registered FSW in Callao, Perú, involving a questionnaire detailing demographics and neurologic symptoms, standard neurologic examination, quantitative assessment of spasticity (QSA) of muscle tone, and serologic testing for HTLV-I. Participants and examiners were blinded to serology results. RESULTS: On the questionnaire and neurologic examination, none of the 32 HTLV-I-seropositive or 223 seronegative women had signs or symptoms of spasticity. However, mean values on QSA were significantly higher among seropositive women (27.1 Newton-meters/radian [N-m/r]) than among seronegative women (21.6 N-m/r, p = 0.01), indicating a subclinical increase in lower extremity tone. With values of QSA divided into tertiles, and the first tertile serving as the comparison group, the odds ratio for seropositivity was 1.4 (95% confidence interval [CI] 1.0 to 2.0) in the second and 3.1 (95% CI 2.2 to 4.3) in the third tertile, after adjusting for age and place of birth. CONCLUSIONS: Although a standard neurologic evaluation could not distinguish between women with and without HTLV-I infection, QSA indicated significantly increased lower extremity tone in those with infection. Long-term follow-up will determine whether these subclinical findings in asymptomatic women progress to overt TSP/HAM.

Adult↗

PubMed1993

Predictive value of general movements in asphyxiated fullterm infants.

The developmental course of spontaneous motility was investigated in a group of 26 fullterm infants, affected by mild to severe hypoxic-ischaemic encephalopathy. Serial 1-h videorecordings were carried out from birth to 15-22 weeks and a quality assessment of general movements (GMs) was made from a replay of the videos. Neurological follow-up of the infants were continued until at least 18 months of age; neonatal EEG and neuro-imaging techniques (US-scan, CT or MRI) were also carried out in all cases. The results indicate that perinatal asphyxia has important effects on the spontaneous motility of fullterm infants. Hypokinesis occurred very frequently during the first days of life, followed by a transient or prolonged (lasting longer than 15-22 weeks) abnormal quality of GMs. In the first 2 weeks the results of GM assessment did not correlate with the simultaneous findings of neurological examination, neuro-imaging and EEG, whereas they did when the results at 15-22 weeks were considered. The changes in spontaneous motility and especially GM developmental trajectories were good predictors of the neurological outcome. The predictive value of GM assessment was found to be similar to that of EEG and neuro-imaging, and better than neurological examination.

Asphyxia Neonatorum↗

PubMed1998

[A 64-year-old woman with progressive gait disturbance and dementia for one year].

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

Brain Stem↗

PubMed1995

[A 65-year-old woman with dysarthria, dysphagia, weakness, and gait disturbance].

We report a 65-year-old woman with progressive dysarthria, dysphagia, weakness, and gait disturbance. The patient was well until 59 years of age (January of 1986) when she noted bilateral ptosis. One year later, she noted a gradual onset of difficulty in speech (articulation). Her speech slowly deteriorated and she noted weakness in chewing power and difficulty in swallowing in addition. In October 1987, she developed emotional incontinence. In January of 1988, she started to drag her left foot. She was admitted to our hospital on June 13 of 1988. On admission, she was alert and general physical examination was unremarkable. Neurologic examination revealed no dementia; her higher cerebral functions appeared intact. Ptosis was present bilaterally more on the right. She showed difficulty in opening her eyes on command; no contraction of the frontal muscles was seen upon attempted eye opening. There was a moderate limitation in the vertical gaze. Forced laughing and crying were seen. Facial muscles were moderately weak without apparent atrophy. The movement of the soft palate was very weak, and swallowing disturbance was more prominent for liquid staff. The tongue appeared somewhat small, however, no fasciculation was noted. Her step was small and the posture was stooped. Retropulsion was present, however, Romberg's sign was absent. No muscle atrophy was apparent, however, diffuse mile to moderate muscle weakness was noted in all four limbs. Cerebellar sign was absent. Deep tendon reflexes were exaggerated bilaterally, and Babinski sign was present on the left side. Sensation was intact. Routine blood tests were unremarkable as was a cranial CT scan. Her ptosis did not improve after 10 mg of edrophonium injection. CSF was also normal. She was transferred to another hospital but her neurological disabilities further progressed. In 1989, she was totally unable to move her limbs; she could only move her eyes; still consciousness was clear without dementia. She developed respiratory difficulty and expired on July 25, 1992. She was discussed in a neurological CPC, and the opinions were divided into ALS and primary lateral sclerosis (PLS). The chief discussant arrived at the conclusion that the patient might have had the pyramidal form of ALS. Postmorten examination revealed marked myelin pallor in the anterior as well as lateral corticospinal tracts. Pyramidal tract degeneration was prominent starting at the level of the cerebral peduncle and was continued to be seen until the level of lumbar cord. The number of anterior horn cells showed only slight decrease in the cervical level, however, it was normal in the lumbar cord.(ABSTRACT TRUNCATED AT 400 WORDS)

Aged↗

PubMed1997

[A 62-year-old man with an acute onset of consciousness disturbances].

We report a 62-year-old man who developed coma and died in a fulminant course. The patient was well until May 1, 1996 when he noted chillness, tenderness in his shoulders, and he went to bed without having his lunch and dinner. In the early morning of May 2, his families found him unresponsive and snoring; he was brought into the ER of our hospital. He had histories of hypertension, gout, and hyperlipidemia since 42 years of the age. On admission, his blood pressure was 120/70, heart rate 102 and regular, and body temperature 36.3 degrees C. His respiration was regular and he was not cyanotic. Low pitch rhonchi was heard in his right lower lung field. Otherwise general physical examination was unremarkable. Neurologic examination revealed that he was somnolent and he was only able to respond to simple questions such as opening eyes and grasping the examiner's hand, but he was unable to respond verbally. The optic discs were flat; the right pupil was slightly larger than the left, but both reacted to light. He showed ptosis on the left side, conjugate deviation of eyes to the left, and right facial paresis. The oculocephalic response and the corneal reflex were present. His right extremities were paralyzed and did not respond to pain Deep tendon reflexes were exaggerated on the right side and the plantar response was extensor on the right. No meningeal signs were present. Laboratory examination revealed the following abnormalities; WBC 18,400/ml, GOT 131 IU/l GPT 50 IU/l, CK616 IU/l, BUN 30 mg/dl, Cr 2.1 mg/ dl, glucose 339 mg/dl, and CRP 27.4 mg/dl. ECG showed sinus tachycardia and ST elevation in II, III and a VF leads and abnormal q waves in I, V5, and V6 leads. Chest X-ray revealed cardiac enlargement but the lung fields were clear. Cranial CT scan revealed low density areas in the left middle cerebral and left posterior cerebral artery territories. The patient was treated with intravenous glycerol infusion and other supportive measures. At 2: 10 AM on May 3, he developed sudden hypotension and cardiopulmonary arrest. He was pronounced dead at 3:45 AM. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that the patient had acute myocardial infarction involving the inferior and the true posterior walls and left internal carotid embolism from a mural thrombus. Post mortem examination revealed occlusion of the circumflex branch of the left coronary artery due to atherom plaque rupture and myocardial infarction involving the posterior and the lateral wall with a rupture in the postero-lateral wall. Marked atheromatous changes were seen in the left internal carotid, the middle cerebral and the basilar arteries; the left internal carotid and the middle cerebral arteries were almost occluded by thrombi and blood coagulate. The territories of the left middle cerebral and the occipital arteries were infarcted; but the left thalamic area was spared. The neuropathologist concluded that the infarction was thrombotic origin not an embolic one as the atherosclerotic changes were severe. Cardiac rupture appeared to be the cause of terminal sudden hypotension and cardiopulmonary arrest. It appears likely that a vegetation which had been attached to the aortic valve induced thromboembolic occlusion of the left internal carotid artery which had already been markedly sclerotic by atherosclerosis. It is also possible that the vegetations in the aortic valve came from mural thrombi at the site of acute myocardial infarction, as no bacteria were found in those vegetations.

Acute Disease↗

PubMed2004

[Phantom sensations and peripheral induced neglect following implantation of total hip prosthesis].

OBJECTIVES: O. Sacks describes in his book "A leg to stand on" the inability to move his leg for along period after rupture of the tendon of m. quadriceps. He refers the history of similar cases, especially of a woman with a hip fracture. He discusses this as a result of disruption of proprioception (peripheral induced neglect). The aim of this investigation is to answer the question how frequently this symptom may be found in patients with hip fracture and/or arthrosis perioperativly after implantation of a total prosthesis. METHODS: we examined 106 patients aged 70.35 +/- 10.01 years (36-male 67.54 +/- 9.74 years and 69 female 71.9 +/- 9.9 years) who underwent an implantation of a hip prosthesis - antero-lateral access Watson-Jones - (right side n = 61, left n = 44) because of arthrosis (n = 81) or a hip fracture (n = 18) on postoperative day 1./2. and 10. - 14. Besides exact neurologic examination, we particularly asked for changed perception of the operated leg. RESULTS: by neurological examination we found only rarely signs of peripheral nerve lesion as a complication of implantation of hip prosthesis (1 femoral nerve lesion, 1 ischiadicus nerve lesion). 14 patients reported a changed perception at the first examination: 5 patients felt their operated leg shorter or longer, 4 patients described their leg changed in a strange manner (like a block of wood or lead), 2 patients felt their leg changed, but could not tell how. At follow-up examination there was no patient with a changed perception of the operated leg. CONCLUSIONS: at first examination only a small number of patients report a changed perception of the operated leg. Early mobilization of the operated leg may be the cause of quick normalization of perception. The peripherally induced neglect, described by O. Sacks, was possibly caused by decrease of cortical representation because of immobilization, but not by disrupted proprioception.

Aged↗