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Biomedical subjects

J Jankovic

Publications and source records attributed to J Jankovic.

At least 235 records · Page 13Linked to original sources
PubMed1992

Low body weight in Parkinson's disease.

Low body weight is a characteristic symptom of Parkinson's disease (PD), but the mechanism is unknown. To determine whether bulbar involvement is responsible for the weight loss, we compared the mean body weight (MBW), height, and body mass index (BMI) of 281 patients with PD and 86 patients with progressive supranuclear palsy (PSP). Although the patients with PSP had significantly worse dysphagia and dysarthria than the patients with PD, their MBW and BMI were not appreciably different. We conclude that bulbar dysfunction is not the reason for weight loss in PD.

Aged↗
PubMed1992

The alien hand and related signs.

Alien limb sign includes failure to recognise ownership of one's limb when visual cues are removed, a feeling that one body part is foreign, personification of the affected body part, and autonomous activity which is perceived as outside voluntary control. Although the hand is most frequently affected, any limb or combination of limbs may fulfil the alien limb criteria. Alien hand sign should be reserved for cases in which the hand feels foreign together with observable involuntary motor activity. To characterise this phenomenon, seven patients with alien hand sign and other motor or behavioural manifestations are described. Aetiologies included multiple infarcts and cortobasal ganglionic degeneration (CBD). In this study, all patients had apraxia in response to verbal commands and problems with bimanual coordination. Most displayed non-goal directed involuntary motor activities, and two had self destructive motor behaviours. Grasp reflex occurred with alien hand due to either aetiology. Cortical reflex myoclonus was frequently seen in CBD patients. The phenomenological spectrum is reviewed, a diagnostic protocol proposed, and possible anatomical bases of alien hand discussed.

Aged↗
PubMed1992

Acoustic reflex abnormalities in cranial-cervical dystonia.

Previous anatomic and physiologic studies suggest brainstem dysfunction in cranial-cervical dystonia. To further explore this, we studied suprathreshold acoustic reflex waveforms in 15 such patients. A unique feature of this technique is its ability to reject movement artifacts before averaging the acoustic reflex waveforms. Thirteen patients (87%) showed some abnormality in reflex waveform morphology. There were both amplitude and latency abnormalities. These findings support the hypothesis that cranial-cervical dystonia reflects dysfunction of the brainstem.

Acoustic Stimulation↗
PubMed1992

Chorea and ballism.

Chorea and ballism are hyperkinetic movement disorders which can be caused by a variety of conditions including metabolic abnormalities, neurodegenerative and immunologic disorders, as well as structural lesions. In addition, paroxysmal dyskinesias may be associated with chorea. Chorea is increasingly recognized as a symptom of a variety of different conditions. The pathophysiologic mechanisms underlying choreic disorders, however, need further elucidation.

Athetosis↗
PubMed1992

Differential diagnosis of Parkinson's disease and the parkinsonism plus syndromes.

Although Parkinson's disease (PD) is thought to represent a specific clinical-pathologic entity, up to 20% of patients diagnosed as having PD will have another disorder at autopsy. Furthermore, pathologic features typically associated with PD can also be observed in patients with other neurodegenerative disorders. This article attempts to point out the difficulties in differentiating PD from progressive supranuclear palsy and other parkinsonism plus syndromes and various causes of parkinsonism associated with cognitive changes. The clinical and pathologic differentiation of these disorders are discussed. These disorders are usually associated with postsynaptic receptor changes and therefore levodopa and dopamine agonists provide limited benefit.

Alzheimer Disease↗
PubMed1992

Neuro-ophthalmic findings in progressive supranuclear palsy.

We studied 104 patients with progressive supranuclear palsy (PSP), 38 of whom were examined by both a neurologist and a neuro-ophthalmologist. Neuro-ophthalmic findings that may help differentiate PSP from Parkinson's disease include vertical supranuclear ophthalmoparesis and fixation instability. Eyelid abnormalities, particularly lid retraction, blepharospasm, and "apraxia" of eyelid opening and closure, were important distinguishing signs. Although downgaze palsy is felt to be the clinical hallmark of PSP, upgaze and downgaze were equally affected at the time of diagnosis in our patients.

Aged↗
PubMed1991

The coexistence of tics and dystonia.

We studied nine patients with motor and phonic tics and other features of Tourette's syndrome, who developed persistent dystonia in addition to their tics. All, except one, were males (mean age, 35.8 years; range, 8 to 59 years), and had onset of tics prior to age 18 years (mean age, 9 years; range, 1.5 to 17 years). None of the patients were treated with neuroleptic drugs prior to the onset of dystonia. Torticollis and blepharospasm were the most common forms of dystonia. Seven patients had a history of tics in first degree relatives. While these patients were seen in a specialized movement disorder clinic and may, therefore, represent a population with atypical and more severe symptoms, the high prevalence rate of dystonia (5.0% of all patients with Tourette's syndrome seen in the clinic) suggests that some patients with tics may have an increased risk for dystonia.

Adolescent↗
PubMed1991

Clinical correlates of response to botulinum toxin injections.

We studied 242 patients with cervical dystonia who had adequate follow-up after botulinum toxin injections to determine which clinical variables had a predictive value in the treatment outcome. Twenty-one patients (16%) categorized as nonresponders were compared with 113 patients (47%) considered to be definite responders. On average, the nonresponders had symptoms for 14 years longer than responders. Seventy-eight of 100 patients with complications were female compared with 54% of 190 patients without complications. In addition, patients with complications weighed less than those without complications. Both findings suggest that the occurrence of complications is related to smaller mean neck muscle mass. Botulinum toxin antibodies were detected in 35.7% of the nonresponders tested and in none of the responders. This comprehensive analysis of outcome variables leads us to conclude that patients with a long duration of dystonia before their first botulinum toxin injection respond less well than those with a short duration of symptoms, that some patients lose their responsiveness because of the development of blocking antibodies, and that women are more likely to develop complications, such as dysphagia and neck weakness, than are men.

Adolescent↗
PubMed1991

Stiff-person syndrome: an autoimmune disease.

Stiff-person syndrome (SPS) is characterized by progressive, usually symmetric rigidity of the axial muscles with superimposed painful spasms precipitated by tactile stimuli, passive stretch, volitional movement of affected or unaffected muscles, startling noises, and emotional stimuli. Electromyography demonstrates continuous normal motor unit potentials in the affected muscles. Both the rigidity and the spasms are relieved by sleep, general anesthesia, myoneural blockade, peripheral nerve blockade, and partially by diazepam. Evidence for an autoimmune etiology of SPS includes its association with other autoimmune diseases and autoantibodies and the presence of antibodies against glutamic acid decarboxylase (GAD) in the cerebrospinal fluid (CSF) of many affected patients. We describe two patients with this syndrome who had GAD antibodies in both CSF and serum. Partial relief of the symptoms in these patients by corticosteroid therapy provides additional evidence of an autoimmune etiology of SPS and of the role of immunotherapy in its treatment.

Autoantibodies↗
PubMed1991

Orthostatic tremor: an association with essential tremor.

Orthostatic tremor is characterized by tremor of the trunk and legs while standing. Rapid frequency has been emphasized as an important criterion for the diagnosis of this tremor. We observed five patients who had the typical findings of orthostatic tremor but had a wide range of frequencies. All five also had postural hand tremor and a family history of essential tremor, suggesting a relationship between orthostatic tremor and essential tremor. This report also emphasizes the association of orthostatic tremor with painful cramps and a relatively consistent improvement with clonazepam.

Aged↗
PubMed1991

Dystonic tics in patients with Tourette's syndrome.

Dystonic tics, in contrast to clonic tics, are relatively slow and temporarily sustained twisting, pulling, or squeezing movements producing briefly maintained abnormal postures. Because dystonic tics often present diagnostic difficulties, we studied these motor tics in 156 patients with Tourette's syndrome (TS). In addition to clonic motor and vocal/phonic tics, seen in all patients, 89 (57%) of them exhibited one or more dystonic tics. The most common dystonic tics were oculogyric deviations (43 patients), blepharospasm (23), and dystonic neck movements (11). Blinking (110) and facial twitching (86) were the most common clonic tics. Except for possibly a greater familial occurrence, a higher frequency of previous head trauma, and associated attention deficit disorder, the group of patients with dystonic tics did not significantly differ in any of the major clinical variables from those TS patients without dystonic tics. We conclude that dystonic tics are typical motor manifestations of TS and that patients with these motor tics are not different from those with more typical clonic tics.

Adolescent↗
PubMed1991

Environmental study of firefighters.

A study of firefighter exposures was undertaken at the request of the U.S. Fire Administration. This work was part of a larger study which included field evaluation of the performance of the self-contained breathing apparatus (SCBA) worn by firefighters during structural firefighting. Measurements were made for a variety of contaminants including CO, CO2, benzene, HCN, HCl, H2SO4, HF, acrolein, CH4, formaldehyde and PNAs. Many of the analyses were performed by collection of bag samples followed by Fourier transform infrared spectroscopy using a field mobile spectrometer. Measurements were also made using solid sorbent tubes and direct-reading meters. Sampling was done both during the knockdown and during overhaul phases of structural firefighting. Also, in order to estimate exposures including those when the SCBAs were worn, measurements were made both inside and outside the SCBA facepiece. Carbon monoxide was the most common contaminant found during knockdown, and about 10% of the samples were greater than 1500 ppm. Formaldehyde, acrolein, hydrogen chloride, hydrogen cyanide, sulphuric acid and hydrogen fluoride all exceeded their respective short-term exposure limits (STEL) on some occasions. Approximately 50% of the knockdown samples for acrolein exceeded the STEL. During overhaul, when masks were usually not worn, many of the contaminants found during knockdown were detected, but typically at much lower concentrations. Inside-mask sampling data suggest that exposure to low concentrations of a variety of compounds is occurring but this is believed to be principally the result of early mask removal or of non-use during knockdown rather than of leakage. The three basic sampling approaches (bag sampling, sorbent tubes and direct-reading meters) proved in this study to be complementary and served to maximize our ability to detect and quantify a wide range of combustion products.

Air Pollutants, Occupational↗
PubMed1991

Proton magnetic resonance spectroscopic studies of hypertrophied muscle. Effect of botulinum toxin treatment.

In vivo image-guided localized proton magnetic resonance spectroscopy (MRS) studies have been performed in a patient with hypertrophied tibialis anterior (TA) muscle and following two injections of botulinum toxin into the affected muscle. Prior to treatment, lipid levels in hypertrophied muscle were found to be low compared to normal. Lipid levels were observed to approach control levels after the second injection. Three months following treatment the trend in exercise-induced changes in lipids was observed to be similar between the hypertrophied and normal TA muscle. This preliminary report demonstrates the potential clinical utility of MRS in evaluating patient response to medical treatment.

Adult↗
PubMed1991

Essential tremor: clinical correlates in 350 patients.

To study the demographic and clinical correlates of essential tremor (ET), we analyzed a comprehensive database of 350 patients evaluated at the Movement Disorders Clinic at Baylor College of Medicine from 1982 to 1989. The age at onset of tremor showed bimodal distribution for both male and female patients, with peaks in 2nd and 6th decades. ET appeared most frequently in hands, followed by head, voice, tongue, leg, and trunk. Half of the patients (47%) had associated dystonia, including cervical dystonia, writer's cramp, spasmodic dysphonia, and cranial dystonia, and 20% of the patients had associated parkinsonism. At least one 1st-degree relative of 62.5% of ET patients reported tremor. Alcohol relieved tremor in 2/3 of ET patients. Sixty-eight percent of patients who had adequate follow-up improved with propranolol, and 72% with primidone. There was no significant difference in various clinical variables between the 219 patients with familial ET and 131 with sporadic ET. Patients with early-onset ET were more likely to have hand involvement and associated dystonia than patients with late-onset ET. Dystonia was more frequently associated with mild ET than with severe ET. Patients with low-frequency tremor were older and had more head but less hand involvement than patients with high-frequency tremor. The lack of relevant differences between ET subgroups suggests that, despite variable expression, ET represents a single disease entity.

Adult↗
PubMed1991

Cervical dystonia: clinical findings and associated movement disorders.

We studied 300 patients, 61% women, with mean age 49.7 years and mean duration of dystonia 7.8 years, to determine the demographic and clinical characteristics of cervical dystonia (CD) and its relationships to other movement disorders. Torticollis was present in 82%, laterocollis in 42%, retrocollis in 29%, and anterocollis in 25%; however, the majority (66%) had a combination of these abnormal postures. Scoliosis was present in 39%, local pain reported by 68%, and 32% had evidence of secondary cervical radiculopathy. In addition to CD, 16% of patients had oral dystonia, 12% mandibular dystonia, 10% hand/arm dystonia, and 10% had blepharospasm. Tremor was noted in 71% of patients; head-neck tremor was present in 60%, and tremor in other body regions was present in 32%. A family history of a movement disorder was present in 44% of the CD patients. Tardive dystonia was the cause in 6%; 11% had posttraumatic dystonia. Anticholinergic drugs provided moderate improvement in 33% of patients, but local intramuscular botulinum toxin injections relieved CD, local pain, or both in over 90% of all treated patients.

Adolescent↗
PubMed1991

Intrathecal baclofen for intractable axial dystonia.

Following spinal instrumentation for scoliosis, a patient developed intractable axial dystonia refractory to conservative medical management. We describe the successful treatment of this dystonia with a continuous infusion of intrathecal baclofen and the subsequent long-term management.

Adolescent↗