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PubMed2005

Young onset limb spasticity with PSP-like brain and spinal cord NFT-tau pathology.

A 30-year-old white man presented with a sporadic form of gradually progressive spastic gait and, later, supranuclear vertical and horizontal gaze palsy, mild cognitive impairment, loss of postural reflexes, and falls. DNA analysis revealed H1/H1 haplotype without tau gene (exons 9 to 13) mutation. Eight years later, postmortem revealed a tauopathy similar to progressive supranuclear palsy. Unusual aspects were early age at onset, neurofibrillary tangle, and tau involvement of the cord.

Adult↗
PubMed1995

Western Nebraska family (family D) with autosomal dominant parkinsonism.

The etiology of Parkinson's disease (PD) remains uncertain. Environmental influences may have an important role, but genetic factors have been firmly implicated in several recently reported kindreds. We studied a family (family D) whose ancestors probably immigrated to the United States from England. The pedigree contains 188 individuals spanning six generations with 18 affected members. Autosomal dominant inheritance is present. Typical levodopa-responsive PD with bradykinesia, rigidity, resting tremor, and impaired postural reflexes develops. Eye movement abnormalities, pyramidal and cerebellar signs, sensory disturbances, and orthostatic blood pressure changes do not occur. Disease progression is slow. PET with [18F]-6-fluoro-L-dopa (FD) performed on an affected individual revealed decreased uptake of FD in a pattern consistent with PD. Autopsy performed on another affected individual demonstrated neuronal and pigmentary loss, gliosis, and Lewy bodies in the substantia nigra pars compacta. This large kindred appears to represent a neurodegenerative disorder closely resembling, if not identical to, idiopathic PD.

Aged↗
PubMed1997

Metric properties of nurses' ratings of parkinsonian signs with a modified Unified Parkinson's Disease Rating Scale.

We evaluated the ability of nurse clinicians to assess parkinsonian signs in older persons with a modified version of the motor section of the Unified Parkinson's Disease Rating Scale (UPDRS). After completing a structured training protocol, three nurse clinicians and a neurologist with expertise in movement disorders administered a modified UPDRS to 75 older persons. The nurses repeated the assessment about 3 weeks later. Inter-rater agreement and short-term temporal stability were estimated for each item, the total modified UPDRS score, and for summary measures of bradykinesia, postural reflex impairment, rigidity, and tremor, and a global parkinsonian sign score. We performed our assessment in Catholic religious communities in the Chicago area, using consecutive subjects at four communities participating in the Religious Orders Study, a longitudinal, clinical-pathologic study of older persons. Our results showed that nurses were not a significant source of variability, with intraclass correlations exceeding 0.97 for all items, and they showed good to excellent agreement with the neurologist for most modified UPDRS items. Correlations between nurses and neurologist exceeded 0.90 for the total modified UPDRS, ranged from 0.76 to 0.95 for the four parkinsonian domain scores, and exceeded 0.90 for the global parkinsonian sign score. Nurses showed fair to good agreement with themselves over the 3-week interval for most modified UPDRS items. Correlations over the 3-week interval exceeded 0.90 for the total modified UPDRS score, ranged from 0.70 to 0.95 for the four domain scores, and exceeded 0.90 for the global parkinsonian sign score. Ratings of parkinsonian signs by nurse clinicians corresponded closely to those of a neurologist with expertise in movement disorders and showed good inter-rater agreement and temporal stability. With appropriate training, nurse clinicians can reliably administer the modified UPDRS.

Aged↗
PubMed2002

Identification of parkinsonism and Parkinson's disease.

Parkinson's disease is a common movement disorder associated with considerable disability. The clinical syndrome of parkinsonism is based on the presence of core clinical features of rest tremor, bradykinesia, rigidity and impaired postural reflexes or gait. Parkinsonism is most often caused by Parkinson's disease, but can also be caused by other disorders, including cerebrovascular disease, multiple-system atrophy, progressive supranuclear palsy and other disorders. Parkinsonism can be identified by questionnaires and confirmed in person or by videotaped clinical examinations. The identification of presymptomatic cases remains problematic but is motivated by the hope for treatment before symptoms appear. Quantitative approaches to the diagnosis of parkinsonism based on the measurement of the cardinal features are available. Clinical approaches should include identification of features atypical for Parkinson's disease, which exclude the diagnosis, and documentation of a response to dopaminergic medications, which support a diagnosis of Parkinson's disease. Loss of smell and visual dysfunction are found in early patients and may be useful in screening protocols. In addition, behavioral changes, including depressive symptoms, may be detected in presymptomatic cases. Cognitive changes, such as impaired set shifting, have been observed in early Parkinson's disease, but can be seen with other causes of parkinsonism. Neuroimaging techniques, including positron emission tomography or single-photon emission computed tomography, are available to quantify dopaminergic neurons, while magnetic resonance imaging may be helpful in differentiating other forms of parkinsonism from Parkinson's disease. There are numerous approaches available to the identification of parkinsonism and Parkinson's disease. The gold standard remains a clinical diagnosis, confirmed by autopsy.

Humans↗
PubMed2006

Parkinson's disease management strategies.

Parkinson's disease (PD) treatment strategies should consider each patient individually. Drug therapy is the mainstay of treatment. An average 62-year-old male first diagnosed with PD will likely live for 20 years and treatment should be geared for long-term control of symptoms and quality of life. Of the currently available drugs, none are neurotoxic to the human substantia nigra and none are neuroprotective. As PD is a progressive disorder, all drugs have adverse effects and reduced efficacy with time. PD patients need regular follow-ups to make necessary medication adjustments. There is no perfect treatment. The authors have discussed their treatment methods and the reasoning behind it. Depending on the patient's age, the predominant symptoms and quality of life, treatment is individualized. In an average patient the least potent drugs, such as anticholinergics or amantadine, are administered first adding a dopamine agonist later on. Levodopa (LD) remains the most useful drug for PD and is reserved for later stages of disease. The objective is to keep the patient at Hoehn and Yahr Stage 2.0 or lower level of disability (bilateral findings with preserved postural reflexes) during off-stage and to avoid adverse effects. There is no long-term difference between standard preparations and control release formulations of LD/carbidopa or LD/benserazide. In older subjects, the first choice is LD. In patients who cannot be managed medically, surgical treatment is an option in selected patients (nondemented, <70 years old, previous good LD response). The surgical treatment of choice is currently subthalamic nucleus deep-brain stimulation. Physiotherapy, occupational therapy and speech therapy are valuable in advanced PD cases.

Age Factors↗
PubMed1994

Familial dysautonomia (Riley-Day syndrome).

Familial dysautonomia, also known as Riley-Day syndrome, is a disorder of autonomic nervous system with an autosomal recessive mode of inheritance. Reduction and/or loss of unmyelinated and small myelinated fibers is found, as reduction of dopamine beta-hydroxylase in blood. The diagnosis is based on clinical features: diminished lacrimation, insensitivity to pain, poor temperature control, abolished deep tendon reflexes, postural hypotension, vomiting attacks, poor motor coordination, and mental retardation. The treatment is symptomatic and many children die during the first years of life, usually as a result of repeated aspiration pneumonia. We report the case of a 1 year-old child with familial dysautonomia.

Dopamine beta-Hydroxylase↗
PubMed1992

Central motor conductivity in aged people.

The conductivity of motor neurons in 26 aged females (mean age 79 years) was analyzed by the conventional conduction method and by pulsed magnetic stimulation and compared with that in 14 younger controls. In aged people, slow motor conduction velocities were found in peripheral nerves. Central motor conduction time (CMCT) in relaxed muscle was shorter in the aged people, although CMCT was normal in mildly contracted muscle. These findings coincide with the results studied in Parkinson's disease, although these subjects were not diagnosed as having Parkinson's disease. Aged people generally have an anteflexed posture, slow movements, and poor postural reflexes, and have been reported to have a decrease in the dopamine level which is relatively earlier than that of other transmitters in the basal ganglia. This may account for the present finding that aged people have neurophysiological abnormalities in CNS which are similar to those in Parkinson's disease.

Adult↗
PubMed1993

The effects of external picoTesla range magnetic fields on the EEG in Parkinson's disease.

We report a 68 year old man with a 7 year history of Parkinson's disease (PD) who obtained little benefit from treatment by dopaminergic and anticholinergic agents. During the six months prior to presentation, he experienced more rapid deterioration in symptoms including memory functions, increasing depression, and dystonia of the foot. External application of picoTesla range magnetic fields (MF) resulted in rapid attenuation of tremor and foot dystonia with improvements in gait, postural reflexes, mood, anxiety, cognitive, and autonomic functions. Plasma prolactin and luteinizing hormone (LH) levels rose three days after initiation of treatment. In addition, distinct electroencephalographic (EEG) changes were recorded nine days after two treatments with MF and included enhancement of alpha and beta activities as well as resolution of the theta activity. These findings demonstrate, for the first time, objective EEG changes in response to picoTesla range MF in PD. Since the pineal gland is a magnetosensor and as some of the clinical effects produced by MF such as relaxation, sleepiness, mood elevation, increased dreaming, and enhancement of alpha and beta activities in the EEG have also been noted in healthy subjects administered melatonin, we propose that the clinical effects as well as the EEG changes noted after treatment with MF were mediated by the pineal gland which previously has been implicated in the pathophysiology of PD.

Aged↗
PubMed2000

[Worsened orthostatic hypotension due to levodopa administration in a case of Parkinson's disease].

A 70-year-old woman began to experience gait disturbance in 1995, followed by the appearance of action tremor of the left hand in 1996. This tremor was subsequently noted in the right hand. Her doctor initially diagnosed Parkinson's disease and administered amantadine and levodopa. The gait disturbance and tremor improved. However, she stopped taking the medication and her symptoms worsened in April, 1998. She was admitted to our hospital, and showed rigidity and action tremor predominantly on the left side, frozen gait, postural reflex disturbance and orthostatic hypotension. Following levodopa administration, her UPDRS score improved from 61.3 +/- 1.2 (mean +/- SD) to 41.7 +/- 5.4. However, she experienced a greater decrease in systolic blood pressure following administration upon standing, from 12.5 +/- 5.8 mmHg to 17.8 +/- 9.2 mmHg. Thus, although levodopa administration led to an improvement in rigidity, tremor and akinesia, her orthostatic hypotension worsened. Based on the present results clinicians should be aware of the potential of worsened orthostatic hypotension when prescribing levodopa to treat Parkinson's disease.

Aged↗
PubMed1991

The effect of age on outcome following traumatic brain injury in rats.

Age of the patient is one of the most important predictors of outcome following human traumatic brain injury. This study employs the fluid-percussion model to investigate the effects of aging on outcome following traumatic brain injury in rats. The results revealed that there was an age-associated increase in mortality rate following both low (1.7 to 1.8 atm) and moderate (2.00 to 2.25 atm) levels of traumatic brain injury. Age-related changes in systemic physiological, neurological, and histopathological indexes of brain injury were also examined following a low level of traumatic brain injury. Traumatic brain injury produced equivalent acute hypertension and increased plasma glucose levels in both young adult and aging rats. Injury produced an acute increase in heart rate in the young adult rat group, while the heart rate decreased in the aged rats. At low levels of brain injury, no significant gross histopathological alterations were produced in either age group. Neurological outcome was assessed by measuring the duration of suppression of a number of nonpostural and postural reflexes and more complex somatomotor functions (righting, escape, head support). Except for head support, there was a significant age-related increase in the duration of the suppression of these reflexes following brain injury. These data demonstrate that aging is associated with an increased mortality rate and greater acute neurological deficits following traumatic brain injury. These data also demonstrate the usefulness of the fluid-percussion model for studying the mechanisms responsible for the age-related increase in vulnerability to brain injury.

Aging↗
PubMed1986

Early post-surgical prosthetic fitting with a pre-fabricated plastic limb.

Early prosthetic fitting and independent ambulation are major goals in the rehabilitation of amputees with early walking being extremely valuable in the physical and psychosocial rehabilitation of the amputee. With early resumption of weight bearing and ambulation, deterioration of postural reflexes can be prevented and the residual limb (stump) can be shaped for definitive prosthetic fitting. A new temporary prosthesis was evaluated in its use on new below-knee amputees. The prosthesis is a plastic, hollow form which fits over the conventional rigid, plaster postoperative dressing. It comes in one size and is cut to fit the patient. The prosthesis is readily available, needs little training for its application, can be re-used, and is inexpensive. Twenty-two consecutive patients treated at Hines Veteran's Administration Hospital and Loyola University Medical Center underwent below-knee amputations. All were determined to be potential walkers. The average age at surgery was 61 years with a range of 17 to 74 years. Amputation was performed for trauma in two patients and gangrene in the other 20. All wounds were dressed with conventional rigid, plaster dressings with cast changes five days postoperatively. The prefabricated temporary prosthesis was applied at the second cast application at an average of 8.1 days postoperatively with permanent prosthetic fitting at 7.7 weeks. There were no complications related to the use of the plastic temporary prosthesis. The use of a new plastic temporary prosthesis has been found to be advantageous in the early rehabilitation of below-knee amputees.

Adolescent↗
PubMedDate not supplied

Evaluation of a new physical exercise taken from salat (prayer) as a short-duration and frequent physical activity in the rehabilitation of geriatric and disabled patients.

BACKGROUND: The major function of rehabilitation and physical medicine specialists is to provide the proper therapy that helps in improving the physical activities of impaired, disabled and handicapped persons through improvement in their muscle strength. In performing their function, the rehabilitation team should always take heed of the social and mental well-being of such patients. Having observed millions of Muslims perform the salat (prayer) regularly at specified times throughout the world, we postulated that salat, along with its various postures, can play a role in increasing psychological well-being including self-reliance and self-esteem, improving musculo-skeletal fitness, motor behavior and cerebral blood flow that may be beneficial in the rehabilitation of geriatric and disabled persons. SUBJECTS AND METHODS: The various postures of salat were studied and a range of joint motions were measured by goniometer, an instrument for measuring angles. Brain blood pressure was calculated from the effect of gravity on blood pressure at different positions. RESULTS: We found that during the offering of salat, most of the joints and muscles of the body were involved in physical activities with little effort, which probably play a vital role in cerebral blood flow and postural reflexes. CONCLUSION: The physical activities involved in the performance of salat helps in the rehabilitation process in disabled geriatric patients by improving blood flow and increasing musculoskeletal fitness. The salat prayer involves little effort (standing, bowing, prostration and sitting), has a short duration and is beneficial for mental and physical health. More studies are needed in future to determine the full beneficial effects of the salat prayer on the rehabilitative process of disabled persons.

Journal Article↗
PubMed1999

[Memory disorders in Parkinson's disease].

52 patients with Parkinson's diseases (PD), 11 patients with suspected Alzheimer's disease (AD) and 20 healthy individuals were observed. The age of the examined persons was 55-74 years. Both quantitative evaluation of the neurologic symptoms and estimation of high psychic functions were performed by means of A.R.Luria's method. Memory tests were also used. It was found that PD was practically always accompanied by memory impairments which were out of the normal age limits. The main mechanism of memory impairment in PD without dementia included insufficiency of independent organization of the mnestic activity at the stages of memorizing and reproduction. Besides, a primary disorder was observed in PD with dementia. There were memory impairments in PD with dementia (more crude disturbances of both involuntary memory and processing of information during memorizing) and in AD (disorders of a semantic memory). It was found that disorders of memory trace consolidation were more dependent on the age of PD beginning, whereas insufficiency of the independent organization of the mnestic activity--on its duration. The majority of memory indices correlated in PD with a degree of the disorders of a gait and postural reflexes. It is suggested that memory defects in PD are manifestations of the main pathologic process which had some differences from the other neurogeriatric diseases.

Aged↗
PubMed2000

Hereditary juvenile-onset craniocervical predominant generalized dystonia with parkinsonism.

OBJECTIVE: To report a unique hereditary, juvenile onset, craniocervical predominant, generalized dystonia and parkinsonism affecting four members of one family. FAMILY DESCRIPTION: A father and three of his four daughters presented to us over the past 30 years with a similar picture of generalized dystonia, starting in the craniocervical region in the second or third decade of life. They later developed moderate parkinsonism, mainly manifesting bradykinesia, rigidity and abnormal postural reflexes. Biochemical and genetic tests excluded Wilson's disease, Huntington's disease and Oppenheim's dystonia. CONCLUSION: This is a new type of familial dystonia-parkinsonism where the craniocervical dystonic symptoms are most prominent in the early stages while parkinsonism becomes the predominant problem later in life. A search for the genetic mutation in this family is underway.

Adolescent↗
PubMed2000

[Effects of tandospirone citrate on frozen gait in patients with early stage of progressive supranuclear palsy, investigated by walk-induced activation single photon emission computed tomography method].

We reported a 64-year-old woman in the early stage of progressive supranuclear palsy presenting as pure akinesia syndrome who showed marked improvement with tandospirone citrate. She revealed bradykinesia, severe frozen gait and disturbance of postural reflex without rigidity or tremor. Treatment with L-dopa and L-threo DOPS was not effective, but tandospirone citrate at a daily dosage of 30 mg significantly lessened the severity of frozen gait. Activation single photon emission computed tomography study with 99mTc-ethyl cysteinate dimer during gait revealed a significant increase in brain activity in the right cingulate cortex after tandospirone citrate treatment. The effect lasted ten months until neck rigidity and ventricular supranuclear palsy were evident.

Brain↗
PubMed2002

[The influence of metabolic disturbances present in diabetes mellitus type I on vestibulo-spinal reflexes in children and young adults].

Diabetic neuropathy encompasses various disturbances concerning somatic and autonomic nervous system and has significant impact on prognosis and course of diabetes mellitus. The aim of the work is an evaluation of vestibulo-spinal reflexes in children and young adults suffering from diabetes mellitus type 1. Material--95 children and young adults aged from 6 to 28 years with diabetes mellitus type 1 diagnosed. The control group consisted of 44 otoneurologically healthy subjects aged from 6 to 28 years. After detailed medical history collection and physical ENT examination stato-posturography was performed in each case. Posturographer PE 62 Model 04 was applied in the studies. Static posturography as well as dynamic one (one leg standing test) was performed in each case. 6 patients belonging to diabetic group complained about vertigo or dizziness. There were worse stabilograms parameters in diabetic group in comparison to control one, statistically significant in younger children. There were better stabilogram parameters in diabetic patients with longer history of the disease. The parameters analysed were significantly worse in the subgroup with not compensated diabetes. The parameters were slightly better in relation to the presence of hypoglycaemic incidents. No apparent differences in stabilograms parameters were present in relation to the presence of diabetic complications. Diabetes mellitus type 1 with slight or without complications does not have significant influence on vestibulo-spinal reflexes and posture stability of the patients. Balance organ disturbances in diabetes mellitus type 1 in children and young adults despite their presence have subclinical course. Perhaps one should consider monitoring of those disturbances in the course of the disease.

Adolescent↗
PubMed2002

[Acquired stuttering as an early symptom in a patient with progressive supranuclear palsy].

We report a 57-year-old man with progressive supranuclear palsy (PSP) showing acquired stuttering (AS) as an early symptom. He had noticed micrographia at age 48, and thereafter he began to suffer from progressive speech disturbance at age 49. Neurological examination at age 57 revealed prominent acquired stuttering, supranuclear vertical gaze palsy, axial rigidity, disturbance of postural reflex, motor perseveration, and catalepsy, but no aphasia. A brain MRI study demonstrated atrophy of the midbrain tegmentum and dilatation of the third ventricle with a few lacunar infarcts in the basal ganglia. Anti-parkinsonian drugs were ineffective. We diagnosed his illness as PSP. His speech was characterized by repetition of sounds and syllables. It was more dominant at repetition of sentences than at that of words, and was improved with rhythmic stimulation. Although pathophysiology underlying AS remains unknown, the AS in this patient seems to be closely related to his motor perseveration and catalepsy. These symptoms may be caused by the disturbance of dopaminergic system from midbrain to limbic system and frontal cortex as reported in PSP. In conclusion, As can be an early and prominent symptom which may be related to characteristic impairment of the dopaminergic system.

Catalepsy↗