Showing papers in "Movement Disorders in 1989"

Lower body parkinsonism: Evidence for vascular etiology

Abstract: We studied 10 patients with marked gait difficulty and no or only minimal upper limb involvement, defined here as lower body parkinsonism (LBP). They were compared to a control group of 100 patients with otherwise typical Parkinson's disease (PD). Both groups were of comparable age, but the mean duration of symptoms was significantly shorter in the LBP group (2.6 +/- 1.5 years versus 7.5 +/- 4.9 years). Gait disturbance was the initial symptom in 90% of LBP patients, as opposed to 7% of controls. Hypertension was present in 70% of LBP patients, and only 22% responded to levodopa. In contrast, only 21% of controls had a history of hypertension, and 96% improved with levodopa. We conclude that these 10 LBP patients constitute a homogenous group, distinct from typical PD. Besides their disproportionate gait disturbance, they are distinguished from PD patients by more rapid progression, higher incidence of hypertension, and a poor response to levodopa. Ischemic etiology for LBP is supported by abnormal neuroimaging studies.

Slowness of movement in Parkinson's disease.

Abstract: Loss of the ability to move is the most characteristic and fundamental motor deficit in Parkinson’s disease. Parkinsonian patients exhibit akinesia (inability to initiate movement), hypokinesia (reduced movement), and bradykinesia (slowness of the movement itself). (All these phenomena will be subsumed under the title akinesia.) Rigid muscles may contribute to akinesia, but they are not the cause. This is evident from the effects of both stereotactic surgery and treatment with levodopa. Stereotactic thalamotomy is an effective means of abolishing contralateral rigidity (and tremor), but does not relieve akinesia, which progresses to cause increasing disability. Levodopa therapy, on the other hand, dramatically relieves akinesia, even in those who have undergone previous stereotactic surgery. The akinesia of Parkinson’s disease represents a major negative symptom and, as such, probably gives the best clue as to what goes wrong with basal ganglia function in that condition (1,2). However, analysis of the pathophysiology of akinesia has proved difficult. One is dealing with higher-order motor function in all its complexity. Normal Movement Two approaches have been employed to investigate events in the brain before the initiation of movement in Parkinson’s disease; (a) the study of reaction times, and (b) the investigation of the Bereitschaftspotential.

Homolateral disappearance of essential tremor after cerebellar stroke.

Abstract: The case of a patient with essential tremor in whom tremor disappeared on the right side after a homolateral cerebellar infarct is reported. The pathophysiology of essential tremor remains unknown. Vascular and surgical lesions of dentorubrothalamic pathway have been reported to improve various kinds of tremor including essential tremor. In this patient, a lesion of this pathway related to the cerebellar infarct probably explains the homolateral cessation of essential tremor.

Tardive akathisia: an analysis of clinical features and response to open therapeutic trials.

Abstract: In recent years, there has been increasing recognition that akathisia occurs not only as an acute, self-limited complication of dopamine (DA) antagonist treatment, but also as a persistent form, called tardive akathisia. We represent a retrospective analysis of clinical features and therapeutic trials in 52 cases of this disorder. Although most patients developed this disorder after years of DA antagonist treatment (mean = 4.5 years), a significant proportion (34%) developed it within 1 year. The characteristic motor features included frequent, complex stereotyped movements. The legs were most frequently involved, showing marching in place and crossing/uncrossing. Trunk rocking, respiratory grunting and moaning, and complex hand movements such as face rubbing or scratching also occurred. In the 26 patients who were able to discontinue DA antagonists, akathisia persisted for years (mean = 2.7 years, range of 0.3-7 years) until abatement of symptoms or last follow-up. Younger patients were more likely to have remission or therapeutic suppression of akathisia at follow-up. In our experience, the catecholamine-depleting drugs reserpine and tetrabenazine were the most effective agents for suppressing symptoms, producing improvement in 87 and 58% of patients treated, respectively. However, improvement was limited in many patients, and at last follow-up only 33% of patients had complete abatement of their symptoms. In conclusion, tardive akathisia is a particularly disabling form of tardive dyskinesia, frequently persistent for years and often resistant to therapy.

The sz mutant hamster: a genetic model of epilepsy or of paroxysmal dystonia?

Abstract: Attacks of sustained dystonic postures of limbs and trunk can be initiated by mild environmental stimuli in an inbred line of Syrian hamsters. The trait is determined by an autosomal simple recessive genetic mutation, originally designated by the gene symbol sz, because the abnormal movements were thought to represent epileptic seizures. The attacks, which can be reproducibly initiated by placing the sz mutant hamsters in a new environment, begin with rapid twitches of the vibrissae, flattened ears, and flattened posture of the trunk while walking, followed by facial contortions, rearing, and sustained posturing of trunk and limbs, often resulting in falling over to the side or backwards. In the final stage, the hamsters became immobile, which can last for hours. An increased tone of limbs and trunk muscles can be palpated during the attack. Electromyographical recordings in awake, unrestrained mutant hamsters showed that the onset of the attack coincided with continuous tonic muscle activity and phasic bursts, which were present even when the animals did not move. During the attack, the animals continue to react to external stimuli. Bilateral electroencephalographic (EEG) recordings before and during motor disturbances in sz mutant hamsters showed no abnormalities. The severity of the dystonic syndrome in hamsters is age dependent with a peak at about 30-40 days of age. A score system for grading type and severity of dystonic attack was developed for use in drug activity studies. The severity of the attack was reduced or attacks were completely prevented by diazepam (1-2.5 mg/kg i.p.) and valproic acid (100-400 mg/kg i.p.) in a dose-dependent fashion. The latency to dystonic movements was significantly increased by diazepam but markedly reduced by subconvulsive doses of pentylenetetrazol (40 mg/kg s.c.). Diazepam antagonized the latency-reducing action of pentylenetetrazol in the hamsters. The pathophysiology and pharmacological sensitivity of the dystonic attacks in these animals remain to be further clarified, but the data indicate that the sz mutant hamsters might represent an interesting genetic model for paroxysmal dystonia. In view of these data, we propose that the hamster mutation should be re-named dystonic and that the new gene symbol should be designated dtsz.

Adductor laryngeal dystonia (spastic dysphonia): treatment with local injections of botulinum toxin (Botox).

Abstract: Adductor spastic dysphonia (SD) is a laryngeal dystonia characterized by a choked, constrained voice pattern with breaks in vocal flow. Treatment with a variety of therapies including speech and pharmacotherapy have minimal benefit; only one-third of patients undergoing recurrent laryngeal nerve section have benefitted at 3 years. We have used local injections of botulinum toxin (Botox) bilaterally into vocalis muscles in 42 patients with SD. Injections were through a teflon-coated hollow electromyography (EMG) recording needle. Unilateral small doses (2.5-3.75 U) were of no clinical benefit. Bilateral small doses resulted in sustained improvement lasting 84.4 +/- 9.3 days. The degree of improvement was 61.1 +/- 4.6%. Common side-effects included a brief period of breathy hypophonia (8.5 +/- 2.5 days) and a mild sensation of choking/aspiration of fluids (1.7 +/- 0.6 days); there were no serious adverse effects. Vocal cord paralysis was not necessary for benefit. Follow-up vocalis muscle EMGs revealed denervation. All patients responded to retreatment (longest follow-up 3.5 years). Patients with prior recurrent laryngeal nerve surgery and residual uncomplicated dysphonia had similar results. Our results indicate that local injection of low-dose Botox is the treatment of choice for SD.

Movement disorders and depression due to flunarizine and cinnarizine

Abstract: Over the last few years, cases of movement disorders induced by flunarizine and cinnarizine have been increasingly reported. We describe a series of 101 patients, whose ages ranged from 37 to 84 years (mean 69.1), developing abnormal movements frequently associated with depression, secondary to treatment with either or both drugs. Symptoms closely resembled those induced by neuroleptic drugs and remitted on drug discontinuance in all but five cases after 5-22 months' follow-up. Whether or not such undesirable side effects are attributable to calcium antagonism and/or dopamine receptor blockade, long-term treatment with flunarizine or cinnarizine should be discouraged, particularly in the elderly.

Eyelid movement abnormalities in progressive supranuclear palsy.

Abstract: We systematically videotaped eyelid movements in a community-based series of 38 patients with progressive supranuclear palsy (PSP). Ten patients (26%) had blepharospasm, "apraxia" of lid opening and/or "apraxia" of lid closing. These patients as a group had more severe upgaze paresis but no greater disease duration than the patients without supranuclear lid dysfunction. Patients used a variety of synkinetic movements to overcome lid-movement abnormalities. One patient displayed "slow blinks," a phenomenon not previously described in PSP. Blink rate in PSP, 3.0/min, was markedly lower than that in patients with Parkinson's disease (PD), 12.5/min, and patients with PSP but not PD increased their blink rate during command versional eye movements.

Volitional control of involuntary movements

Abstract: Voluntary suppressibility of abnormal movements is helpful in the classification of movement disorders because this ability appears to be a common component of tics. However, there has been no systematic study of voluntary suppressibility in other movement disorders. We have therefore assessed 146 patients with tremors and dyskinetic disorders as to their ability to suppress movements by mental concentration. Patients were videotaped while trying to stop their movements, and the length of time they could suppress their abnormal movements was recorded. One hundred percent (10 of 10) of patients with tics could suppress movements for an average of 2.5 min. Two percent (1 of 50) of essential tremor patients could suppress the tremor, and the tremor of 24% (12 of 50) was made worse by mental concentration. Eighty percent (4 of 5) of neuroleptic-induced tremor could be improved mentally. Seventy percent (35 of 50) of patients with parkinsonian tremor could voluntarily diminish their tremor for an average of 48 s. Fifty percent (8 of 16) of chorea (tardive dyskinesia, Huntington's disease, postencephalitic) was reduced. Dystonia was suppressible in 20% (3 of 15). It is concluded that movement disorders besides tics can be voluntarily suppressed and that suppressibility should not be used to classify movement disorders. Tics, however, are easier to suppress and can be suppressed for a longer time.

Parkinsonism‐plus syndromes

Abstract: PARKINSONISM-PLUS SYNDROMES ......................................................................................................... 1 PROGRESSIVE SUPRANUCLEAR PALSY (PSP, S. STEELE-RICHARDSON-OLSZEWSKI SYNDROME) ........... 1 CORTICOBASAL GANGLIONIC DEGENERATION (CBGD) ........................................................................ 3 MULTIPLE SYSTEM ATROPHY (MSA) .................................................................................................... 3 LYTICO-BODIG DISEASE (PARKINSONISM-DEMENTIA-ALS COMPLEX OF GUAM) .................................. 5 HEREDODEGENERATIVE PARKINSONISM ................................................................................................ 5 HALLERVORDEN-SPATZ DISEASE .......................................................................................................... 5 FAMILIAL BASAL GANGLIA CALCIFICATIONS ........................................................................................ 5 WILSON DISEASE (HEPATOLENTICULAR DEGENERATION) ..................................................................... 5

Antiparkinsonian activity of CY 208-243, a partial D-1 dopamine receptor agonist, in MPTP-treated marmosets and patients with Parkinson's disease.

Abstract: The effect of stimulation of cerebral dopamine D-1 receptors by CY 208-243 on motor disability was tested in MPTP-treated parkinsonian marmosets and patients with Parkinson's disease. CY 208-243 (0.5–1.25 mg/kg s.c.) produced a dose-related reversal of akinesia and rigidity in the marmosets, lasting some 2 h. Single morning doses of CY 208-243 (5–40 mg) were compared with the usual morning dose of levodopa in eight patients with Parkinson's disease on long-term levodopa therapy who had developed motor fluctuations from immobility with akinesia and rigidity (off) to mobility often with dyskinesias (on). CY 208-243 alone was capable of switching such patients from off to on; five of the eight patients responded to the highest dose (40 mg), sometimes with dyskinesias. The response to CY 208-243 was comparable to that produced by levodopa in these cases. Drugs designed to stimulate both dopamine D1 D2 receptors in the brain may improve the therapy of Parkinson's disease.

Pure akinesia due to lewy body Parkinson's disease: a case with pathology

Abstract: The case of a patient with levodopa-responsive pure akinesia and freezing of gait for 17 years, whose brain showed the classical changes of Lewy body Parkinson's disease at postmortem is presented. A short trial of DL-threo-DOPS was ineffective. Intellectual function was preserved despite the presence of Lewy bodies and mild cell loss in subcortical cholinergic nuclei. Pure akinesia is likely to be a heterogeneous condition, with most cases having little or no response to levodopa therapy. However, this case demonstrates that this clinical picture may be caused by Lewy body Parkinson's disease.

Ataxia telangiectasia: a reappraisal of the ocular motor features and their value in the diagnosis of atypical cases.

Abstract: The eye movements of four patients with ataxia telangiectasia (AT), three of whom had an unusual neurological presentation, were studied. All had striking abnormalities of saccadic generation with markedly hypometric saccades, increased saccadic latency, but normal saccadic velocity. Three patients used head thrusts to aid refixation. In addition, there was absence of smooth pursuit and optokinetic nystagmus, and hyperactive vestibular responses. Two of the four patients had, in addition, periodic alternating nystagmus. This combination of an ocular motor apraxia with superadded cerebellar ocular motor abnormalities, and possibly periodic alternating nystagmus, should strongly suggest the diagnosis of AT, even if the clinical syndrome is otherwise atypical.

Betel nut-induced extrapyramidal syndrome: an unusual drug interaction.

Abstract: Two cases are described of chronic schizophrenic patients maintained on depot neuroleptics, who developed severe extrapyramidal symptoms following a period of heavy betel nut consumption. A mechanism for this effect is proposed based on the pharmacological antagonism of the anticholinergic agent, procyclidine, by the active alkaloid ingredient of the betel, arecoline.

Clinical and CT scan findings in a case of cyanide intoxication.

Abstract: A 39-year-old man showed a combination of severe parkinsonism and progressive dystonia following attempted suicide with sodium cyanide. Computed tomography (CT) scan showed bilateral lucencies in the putamen and external globus pallidus. The topography of lesions on CT scan closely correlated with the pathological findings described in a previous report of cyanide-induced parkinsonism. This is the first reported case of cyanide intoxication with delayed-onset dystonia.

Paraneoplastic degeneration of the substantia nigra with dystonia and parkinsonism.

Abstract: A 42-year-old woman suffered unexplained weight loss followed by action tremor and difficulty initiating gait. Three months after onset of symptoms, infiltrating ductal carcinoma of the breast, metastatic to liver and lymph nodes, was diagnosed and treated briefly with cyclophosphamide, methotrexate, and 5-flourouracil (5FU). Severe symmetric action and postural tremor with a myoclonic component developed, with minimal rest tremor, severe dysarthria and dysphagia, small-stepped and slightly ataxic gait progressing to a bedbound state, and severe widespread dystonic posturing. The latter began as a typical parkinsonian posture of trunk and upper extremities and progressed to a fixed and painful flexion of the elbows and wrists and extension of fingers and neck. Sinemet, anticholinergics, baclofen, diazepam, and plasmapheresis gave no benefit. The patient died of complications of immobility 5 months after neurologic symptom onset. Autopsy revealed many pigment-laden macrophages in substantia nigra and moderate loss of pigmented neurons. Inflammation, Lewy bodies, and tumor were absent. Cerebellar Purkinje cells were moderately depleted. Mild neuronal loss and gliosis were present in globus pallidus and cerebellar cortex. Stains for anti-human IgG, IgM, kappa, and lambda were negative. This, to our knowledge, is the first report of paraneoplastic degeneration of substantia nigra or paraneoplastic parkinsonism.

Treatment of movement disorders with trihexyphenidyl.

Abstract: The clinical efficacy of the trihexyphenidyl was investigated in 100 patients with movement disorders. The study group consisted of 54 women and 46 men. Their ages ranged from 18 to 70 years, and their duration of illness varied from a few months to 36 years. Each patient had a videotape of the movements and a neurological examination, before administration of the drug, at the time of maximum or effective dosage, and one week after withdrawal from trihexyphenidyl. The drug was administered at an initial total daily dose of 2 mg and gradually increased to a total daily dose of 60 mg over a period of 4-6 weeks. Improvements were rated both clinically and from the videotapes. Three groups of movement disorders demonstrated a significant response to trihexyphenidyl: (1) dystonia 37%; tonic torticollis demonstrated a significantly better response than the clonic variant (80% vs. 22%). (2) rhythmic-oscillatory movements of brainstem-cerebellar origin (palatal myoclonus, pendular nystagmus, facial myokymia) 90%; (3) cerebellar tremor 75%. Among 32 responders, 17 (56%) continued taking trihexyphenidyl beyond 24 months. Side effects consisted of dryness of the mouth, jitteriness, stomatitis, blurred vision, and forgetfulness.

Off-period belching due to a reversible disturbance of oesophageal motility in Parkinson's disease and its treatment with apomorphine.

Abstract: Two L-dopa-treated patients with Parkinson's disease who developed distressing belching during "off" periods are reported. In each case, contrast cine radiography revealed disturbed oesophageal motility which disappeared after injection of the dopamine receptor agonist apomorphine. It is suggested that central dopaminergic abnormalities may be important in the aetiology of "off period belching."

"On-off"-induced lethal hyperthermia.

Abstract: Neuroleptic malignant syndrome (NMS) is being increasingly recognized as a potential complication of neuroleptic therapy. Similar hyperthermic episodes have also been described in other settings of abrupt cessation of dopaminergic stimulation such as levodopa withdrawal. We report a fatal NMS-like episode occurring as a complication of an "off" period in a patient with Parkinson's disease.

Hemichorea-hemiballismus associated with acquired immune deficiency syndrome and cerebral toxoplasmosis

Abstract: A young woman had hemichorea-hemiballismus subsequently found to be secondary to a cerebral toxoplasmosis infection complicating human immunodeficiency virus infection. This patient had the sixth reported case of acquired immune deficiency syndrome (AIDS) with hemichorea-hemiballismus, and each has been secondary to cerebral toxoplasmosis. The presence of hemichorea-hemiballismus in a young patient should suggest a diagnosis of AIDS and in particular the diagnosis of secondary cerebral toxoplasmosis. Other movement disorders that occur in AIDS are discussed.

Spasmodic torticollis due to a midbrain lesion in a case of multiple sclerosis

Abstract: A case of multiple sclerosis is described in which spasmodic torticollis occurred abruplty and abated after 1 year. Magnetic resonance imaging (MRI) demonstrated a lesion in the mesencephalon. Other symptoms and physical signs that developed at the same time as the spasmodic torticollis were compatible with the lesion that had not been present on MRI 18 months previously. There are very few reports of spasmodic torticollis due to an identified focal lesion; there is evidence form experimental work on animals that midbrain lesions may cause spasmodic torticollis but there has been no previous human example.

Reduced glutamate decarboxylase activity in the subthalamic nucleus in patients with tardive dyskinesia.

Abstract: Glutamate decarboxylase (GAD) activity was measured in the nuclei of the basal ganglia in patients with neuroleptic-induced tardive dyskinesia (TD) and controls matched for age and premortem state. In five TD patients, who all had a sudden death, a significant decrease in GAD activity was found in the subthalamic nucleus (STN). The lowered GAD activity in the STN may represent a biochemical substrate for neuroleptic-induced TD.

Comparison of Sinemet CR4 and standard Sinemet: double blind and long-term open trial in parkinsonian patients with fluctuations.

Abstract: "Wearing-off" effect, the most common form of levodopa-induced fluctuations, seems to be related to the short plasma half-life of the drug. More sustained plasma levodopa levels may be achieved with a new controlled-release formulation of carbidopa/levodopa, Sinemet CR4. We studied 20 patients, 12 men and 8 women, with Parkinson's disease complicated by "wearing-off" phenomenon. Mean age was 61.1 +/- 8.1 years, duration of symptoms 8.3 +/- 2.4 years, and the Hoehn-Yahr stage 3.0 +/- 0.9. In a 12-week double-blind study, the average number of tablets administered per day decreased from 5.7 +/- 1.2 to 3.8 +/- 0.7 when Sinemet CR4 (50/200) was substituted for the standard Sinemet (25/100) (p less than 0.001). However, this was at the expense of reducing the "on" time (without dyskinesia) from 9.3 +/- 4.6 to 7.5 +/- 4.3 (p less than 0.05), although the total "on" time did not significantly change. In a long-term follow-up of 18 patients, the "on" time with dyskinesia and morning dystonia significantly increased (p less than 0.05). There was no significant change in the total daily dosage of levodopa, but the daily number of doses and tablets significantly decreased (p less than 0.001). Despite increased dyskinesia, most patients preferred taking fewer tablets and have elected to continue taking Sinemet CR4 instead of standard Sinemet. Sinemet CR4 seems to offer a new and effective strategy for the management of levodopa-related fluctuations.

Dopamine and memory function in Parkinson's disease.

Abstract: Response fluctuations in motor function, complicating long-term dopaminomimetic therapy of Parkinson's disease, may extend to the cognitive realm. To evaluate the effect of levodopa treatment both on attention as well as acquisition and retrieval of memory tasks, parkinsonian patients were examined neuropsychologically both while medicated with levodopa/carbidopa ("on") and when the medication's antiparkinsonian effect had worn off ("off"). Significant cognitive differences emerged only on the delayed recall of complex verbal materials, where patients when "on" performed better compared with their "off" state. Comparison of change scores across states (administration or withholding of levodopa/carbidopa between acquisition and retrieval, "off" to "on" or "on" to "off"), revealed no substantial differences as a function of dopaminomimetic therapy. These results support the view that slight changes in cognition are associated with dopaminomimetic therapy of Parkinson's disease, but that these changes may be task-specific.

A case of post-traumatic tic disorder.

Abstract: We present the case of a 27-year-old man who developed multiple motor tics following closed head trauma with loss of consciousness. Age of onset, lack of family history of tics, and atypical progression of tic severity and location make it unlikely that the patient had Gilles de la Tourette syndrome. Previous reports of post-traumatic tic syndrome are discussed.

Spasms of amputation stumps: a report of 2 cases

Abstract: The occurrence of involuntary movements of stumps following amputation is described in two patients. Although recognised for over 100 years, this phenomenon has received little attention in the modern literature. The condition appears to represent a variant within the spectrum of movement disorders induced by injury to the peripheral nervous system.

Stability of the head in pitch (neck flexion‐extension): Studies in normal subjects and patients with axial rigidity

Abstract: The dynamic stability of the head in pitch during normal upright posture has been studied in normal subjects and patients with neurological disease affecting neck muscle tone by examining angular head acceleration responses to unpredictable linear motion of the trunk in the direction of surge. Within the frequency range of natural head movements the transfer function between head and trunk for both normal subjects and patients approximated a second-order linear differential equation involving inertia and coefficients of viscosity and elasticity. The degree of neck rigidity was determined by the damping ratio (viscosity:elasticity), which averaged .35 for normal subjects and ranged from 0.6 to 0.96 for patients with rigid syndromes. A patient with absent labyrinthine function and a "floppy" head had a damping ratio 0.18. The technique gives a numerical measurement of neck rigidity, which could be of value in characterising severity of disorder and response to therapy.