Leg Rigidity Research Articles

Full-Term Baby with Cerebellar Hemorrhage Referred for Severe Jaundice Occurring within the First 3 Days of Life

Cerebellar hemorrhage in full-term infants is a rare condition recently recognized in high-risk newborns requiring intensive care with the availability of advanced neuroimaging techniques. Several aspects such as the incidence, pathophysiology, clinical features, and prognosis of cerebellar hemorrhage in full-term infants remain unknown. We present a case of cerebellar hemorrhage with subdural hemorrhage in a patient hospitalized for jaundice after birth without a history of traumatic delivery, such as breech presentation, prolonged labor or forceps delivery. A full-term female infant weighing 3,100 g at birth, with no complications during delivery, developed jaundice within 48 hours of birth and was admitted for intensive phototherapy in the first 3 days of life with a transcutaneous total bilirubin level of 18.1 mg/dL. Magnetic resonance imaging revealed cerebellar brain lesions with a subdural hemorrhage. At the age of 3 months, the infant exhibited leg rigidity and was referred for rehabilitation. The patient showed signs of improvement during treatment and was generally catching up well with her peers at the age of 9 months. Long-term follow-ups are required to evaluate the consequences on cognitive development, behavior, and motor performance subsequently in life.

Stellate Ganglion Blockade for Corticobasal Syndrome Pain: A Case Report and Potential New Discovery.

Corticobasal syndrome (CBS) is a neurodegenerative condition characterized by cortical and extrapyramidal dysfunction. It can manifest as painful asymmetric rigidity, spasticity, and dystonia. Treatments include muscle relaxants, analgesics, chemodenervation, and physical therapy.1 Some patient's pain is not alleviated by current treatments, demonstrating the need for new effective treatments. We report what may be the first case of CBS pain treated with stellate ganglion blockade. Stellate ganglion block is used to treat sympathetically mediated pain and complex regional pain syndrome (CRPS) of the upper extremity. We present a 63-year-old female diagnosed with CBS. Six years prior, she developed clumsiness, cramping, and difficulty completing tasks involving the right hand. This progressed to severe right upper extremity rigidity after 2 years. She was evaluated by movement and behavioral neurologists. She exhibited right upper extremity akinesis and rigidity, right sided predominate bradykinesia and leg rigidity, deficits with attention, language and recall, and speech apraxia. Magnetic resonance imaging, positron emission tomography, and dopamine transporter scan revealed volume loss of the left frontal and anterior parietal lobes, hypometabolism in the left posterior frontal region and mid-cingulate regions, and decreased radiotracer binding in the bilateral putamen, greater on the left. Laboratory studies including a frontotemporal dementia panel were unremarkable. As her condition progressed, she began experiencing constant pain in the right upper extremity. It was described as 8 of 10 in severity and only improved with botulinum toxin injections. At best, her pain was 4 of 10 after chemodenervation. However, this benefit often diminished weeks before her next dose. Despite chemodenervation treatment, she continued to describe her pain as “bone-breaking” and “intense.” Her pain did not improve with baclofen, tizanidine, clonazepam, levodopa, fluoxetine, ibuprofen, naproxen, acetaminophen, cannabidiol, topical diclofenac, or physical therapy. She was referred to pain medicine and a sympathetic block was proposed as a palliative measure. A right stellate ganglion block was performed with a 5 mL mixture of 10 mg of dexamethasone and 0.5% bupivacaine with epinephrine 1:200,000. She experienced relief after the procedure and continued to endorse a 0/10 pain at 2.5, 4, and 7 weeks. However, she did not experience improvement in her rigidity, dystonia, or limb akinesia. There has been at least one prior case of a patient with CBS who developed CRPS. Both conditions are associated with bradykinesia, dystonia, immobility, and sensory abnormalities.2 Both exhibit loss of inhibitory input to the cortex, increased cortical excitability,1, 3 and thalamic degeneration.1, 4 CRPS and CBS share the same pain generating mechanism as central post-stroke pain. In each condition, there is sensory deafferentation and reduction in thalamic and sensory input to the cortex, which produces pain.5 There are limited studies on the mechanism of pain generation in CBS. In her case, we suspect it is a combination of both peripherally and centrally mediated processes. The exact explanation for why she benefited from the procedure remains unclear. Sympathetic blockade may be effective in treating CBS-related pain. CBS and CRPS share similar pathophysiologic and phenotypic features. More research is needed to better treat pain associated with CBS and other neurodegenerative disorders. (1) Research project: A. Conception, B. Organization, C. Execution; (2) Statistical Analysis: A. Design, B. Execution, C. Review and Critique; (3) Manuscript: A. Writing of the First Draft, B. Review and Critique. L.H.: 1A, 1B, 1C, 3A. R.F.: 1A, 1C, 3B. E.C.S.: 1C, 3B. Ethical Compliance Statement: The authors confirm that this case is exempt from Institutional Review Board review per University of Nebraska Medical Center policy. Informed consent was discussed verbally with the patient, and then informed consent was obtained in written form. All core elements of Health Insurance Portability and Accountability Act of 1996 (HIPPA) and the additional informed consent requirements of this journal was addressed, obtained, and documented in the patient consent form. The patient consent form can be available upon request. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines. Funding Sources and Conflicts of Interest: No specific funding was received for this work. The authors declare that there are no conflicts of interest relevant to this work. Financial Disclosures for the Previous 12 Months: The authors declare that there are no additional disclosures to report.

Neuromechanical Assessment of Activated vs. Resting Leg Rigidity Using the Pendulum Test Is Associated With a Fall History in People With Parkinson's Disease.

Leg rigidity is associated with frequent falls in people with Parkinson’s disease (PD), suggesting a potential role in functional balance and gait impairments. Changes in the neural state due to secondary tasks, e.g., activation maneuvers, can exacerbate (or “activate”) rigidity, possibly increasing the risk of falls. However, the subjective interpretation and coarse classification of the standard clinical rigidity scale has prohibited the systematic, objective assessment of resting and activated leg rigidity. The pendulum test is an objective diagnostic method that we hypothesized would be sensitive enough to characterize resting and activated leg rigidity. We recorded kinematic data and electromyographic signals from rectus femoris and biceps femoris during the pendulum test in 15 individuals with PD, spanning a range of leg rigidity severity. From the recorded data of leg swing kinematics, we measured biomechanical outcomes including first swing excursion, first extension peak, number and duration of the oscillations, resting angle, relaxation index, maximum and minimum angular velocity. We examined associations between biomechanical outcomes and clinical leg rigidity score. We evaluated the effect of increasing rigidity through activation maneuvers on biomechanical outcomes. Finally, we assessed whether either biomechanical outcomes or changes in outcomes with activation were associated with a fall history. Our results suggest that the biomechanical assessment of the pendulum test can objectively quantify parkinsonian leg rigidity. We found that the presence of high rigidity during clinical exam significantly impacted biomechanical outcomes, i.e., first extension peak, number of oscillations, relaxation index, and maximum angular velocity. No differences in the effect of activation maneuvers between groups with clinically assessed low rigidity were observed, suggesting that activated rigidity may be independent of resting rigidity and should be scored as independent variables. Moreover, we found that fall history was more common among people whose rigidity was increased with a secondary task, as measured by biomechanical outcomes. We conclude that different mechanisms contributing to resting and activated rigidity may play an important yet unexplored functional role in balance impairments. The pendulum test may contribute to a better understanding of fundamental mechanisms underlying motor symptoms in PD, evaluating the efficacy of treatments, and predicting the risk of falls.

Lower Limb Rigidity Is Associated with Frequent Falls in Parkinson's Disease.

The role of muscle rigidity as an etiological factor of falls in Parkinson's disease (PD) is poorly understood. Our objective was to determine whether lower leg rigidity was differentially associated with frequent falls in PD compared to upper limb, neck, and total rigidity measures. We examined the associations between Unified Parkinson's Disease Rating Scale-Part III (motor) rigidity subscores and the history of monthly or more frequent falls in 216 individuals with PD (age, 66 ± 10 years; 36% female; disease duration, 7 ± 5 years) with logistic regression. A total of 35 individuals were frequent fallers. Significant associations were identified between lower limb rigidity and frequent falls (P = 0.01) after controlling for age, sex, PD duration, total Unified Parkinson's Disease Rating Scale- Part III score, and presence of freezing of gait. No significant associations (P ≥ 0.14) were identified for total, arm, or neck rigidity. Lower limb rigidity is related to frequent falls in people with PD. Further investigation may be warranted into how parkinsonian rigidity could cause falls.

A parkinsonian movement disorder with brain iron deposition and a haemochromatosis mutation

A 60-year-old, right-handed, Caucasian woman presented to the outpatient department with a three-month history of clumsiness and resting tremor in her right hand, associated with micrographia. She had no significant past medical history. Her family history included a brother who died at the age of 50 after a gastrointestinal bleed, attributed to alcoholic liver disease. Examination showed hypomimia, right hand resting tremor, and right arm and leg rigidity with bradykinesia. There was reduced arm swing during gait, and retropulsion on pull testing. The provisional diagnosis was idiopathic Parkinson’s disease. She was started on dopamine agonists and later on levodopa. However, the symptoms progressed rapidly, with worsening slowness and stiffness, development of postural hypotension and incomplete bladder emptying. She also reported daytime somnolence and snoring at night. As such, the diagnosis was revised to a Parkinson’s-plus syndrome: multiple system atrophy. Investigations revealed mildly raised serum alanine aminotransferase (63 IU/L), but the other liver function tests, full blood count, and copper studies (including serum ceruloplasmin) were all normal. No iron studies were performed. A 24-h cardiac monitor showed no arrhythmias. MRI-head (T1, T2, FLAIR and Gradient echo) at the time of presentation was normal. The patient did not attend for planned autonomic and respiratory function tests. Thirteen months after the first clinic appointment she died in her sleep. A post-mortem examination showed extraand intra-cellular deposition of iron in the globus pallidus, putamen, substantia nigra, locus coeruleus, red nuclei and dentate nuclei. This iron was visualised by Perl’s stain (Figs. 1 and 2), and was in fine granules, in moderate amounts. In addition, there was marked and diffuse neuronal loss from the substantia nigra and locus coeruleus with pale bodies within their neurons. However, no Lewy bodies were seen (alpha-synuclein negative). Axonal spheroids were present, dispersed through brain white matter tracts, consistent with secondary axonal damage. The liver was strongly positive for iron throughout (Perl’s stain). A DNA sample from post-mortem tissue was homozygous for the C282Y Hereditary Haemochromatosis mutation. This is the first case reported of a patient with a movement disorder and a haemochromatosis mutation, in which clear iron deposition in relevant parts of the brain has been seen at postmortem. Several idiopathic neurological conditions (including Parkinson’s disease) are associated with increased brain iron [1], but this is at the level of percentage changes in overall concentration, rather than the gross iron deposition seen in the case reported here. Very high brain iron does occur in conjunction with extrapyramidal disorders in neurodegeneration with brain iron accumulation (NBIA), a group of inherited diseases [2]. Our patient’s age, family history, liver iron, and C282Y mutation all go against this diagnosis. Hereditary haemochromatosis (HH) involves pathological iron deposition in a variety of organs [3]. However, central nervous system disorders are not traditionally listed among the sequelae of the disease. Previous postmortem studies in HH patients have shown excess iron in choroid plexus and pituitary, but not elsewhere in the brain [4], suggesting the blood brain barrier is protective. S. Williams (&) M. R. Vinjam A. Ismail A. Hassan Leeds Teaching Hospitals NHS Trust, Leeds, West Yorkshire, UK e-mail: stefanwilliams@doctors.org.uk

Lateralized alpha-motoneuron excitabilities during lying and standing of healthy individuals in relation to Parkinsonian rigidity

Objectives: To elucidate mechanisms of Parkinsonian rigidity by assessing excitability of alpha-motoneurons innervating right and left soleus muscles in healthy controls and Parkinson’s disease (PD) patients with rigidities in the right, left and both legs.Methods: One group of 45 controls was recruited and 60 PD patients in three groups: rigidities, predominantly in the right, left and both legs. H-reflex (H) and muscle response (M) were recorded from right and left soleus muscles during stimulations of the posterior tibial nerve at the popliteal fossa while lying and standing. The H/M ratio was taken as an index for motoneuron excitability.Results: Mean H/M ratios were significantly different on the right and left sides, modified by postural changes in controls and PD patients. Analysis of variance showed that in healthy subjects the H/M ratio was: standing>lying (right), lying>standing (left). In right leg rigidity patients, the H/M ratio was greatest during standing, and smallest during lying. In left leg rigidity patients, the H/M ratios on the right and left sides were equally independent of posture. In controls, left H/M>right while lying, <right while standing. In right leg rigidity patients, right H/M>left, but <right in left leg rigidity patients, independent of posture. There was no side difference in patients with rigidity in both legs.Conclusions: (i) motoneuron excitability may show side and postural differences in healthy individuals and PD patients; (ii) posture may be associated with lateralized motoneuron excitability in these subjects; and (iii) Parkinsonian rigidity may have spinal motor origins.

Suspected congenital pseudomyotonia in two Scottish beef herds

Pseudomyotonia is clinically characterized by a lifelong history of exercise-induced muscle contracture. This case report describes four crossbred calves with suspected congenital pseudomyotonia from two beef suckler herds in Scotland connected by a common sire. Affected calves would lag behind the others and after being forced to walk quickly for 50 metres or so would suddenly develop hind leg rigidity propelling themselves forwards with the nose touching the ground and then fall into lateral recumbency. There was no seizure activity and the calves would stand within 10–20 seconds and walk away albeit with a stilted gait.

Development of a gas chamber for detecting broiler chicken halothane sensitivity and PSE (Pale, Soft, Exudative) meat formation

The objective of this work was to investigate the use of a halothane to screen broiler chickens prone to develop PSE (Pale, Soft, Exudative) meat through a special gas chamber connected to a veterinarian anaesthetic apparatus developed in our laboratory. Anaesthesia was induced with approx. 3.0% halothane at a flow rate of 6.0 Lm-1 in pure oxygen for 5 min. Commercial male broilers (n=342) aged 46 days old were evaluated and classified as either halothane sensitive (HAL+) or insensitive (HAL-), depending on the leg rigidity response. Approximately 27.5% (n=94) of broilers were HAL+ and 72.5% (n=248) were HAL-. This is a simple and rapid technique to evaluate broiler sensitivity to halothane and identify broilers prone to develop PSE meat. The occurrence of PSE meat in HAL insensitive broiler chicken samples suggests that other factors are related to PSE occurrence.

Muscle strength is significantly associated with hip bone mineral density in women with Parkinson’s disease: A cross-sectional study

To study the influence of physical impairments on hip bone mineral density in women with Parkinson's disease. Cross-sectional study. Thirty-four women with Parkinson's disease and 30 age-matched healthy controls. Patients with Parkinson's disease underwent a hip scan using dual-energy X-ray absorptiometry and total hip bone mineral density values were obtained. Motor Examination III of the Unified Parkinson Disease Rating Scale was used to assess leg tremor, leg agility, leg rigidity and postural stability. In addition, all subjects were evaluated for walking speed, walking endurance, and leg muscle strength. Based on the hip bone mineral density values, 12 patients with Parkinson's disease (35%) had osteopaenia and another 3 patients (9%) had osteoporosis. Patients with Parkinson's disease had significantly lower walking velocity (p = 0.002), walking endurance (p < 0.001) and leg muscle strength (p = 0.047) than controls. Multiple regression revealed that leg muscle strength alone accounted for 8.8-10.6% of the variance in hip bone mineral density among patients with Parkinson's disease, after controlling for body mass index, post-menopausal years, Hoehn and Yahr stage, and postural stability (p < 0.05). Hip bone mineral density is independently associated with leg muscle strength in women with Parkinson's disease.

Apotemnophilie : une mise en forme contemporaine de la souffrance psychique ?

The word <<apotemnophilia>> was created in 1977. It was first used to describe an extreme paraphilia concerning both the search for amputees as sexual partners, and the fantasies and wishes to be amputated linked to a sexual arousal. More recently, the number of self-demand amputations appears to have raised. Some amputations of healthy legs have even been performed in hospital settings, raising important ethical issues. A new category of trouble has been described: the Body Integrity Identity Disorder (BIID). Criterias for its diagnosis have been developed and submitted to DSM V task force. According to clinicians who support the existence of BIID, this disorder is not a paraphilia, don't overlap with other psychiatric disorders and could be in some ways compared to transexualism. The patient's health would therefore require the amputation of healthy limbs in order to <<achieve>> themselves and to help them to become <<able-bodied>> according to their <<true self>>. Still according to those clinicians, psychiatric symptoms would be either a consequence of the shame resulting from this condition, or a consequence of the doctor's refusal to perform these amputations. An ever growing <<grey>> litterature on the web support this opinion, but the scientific litterature is still very scarce. In this paper, we analyse available scientific datas. This review does not support the existence of BIID as a discrete nor a specific condition. In a second part of this paper, we describe the case of a young woman who started asking for above-the-knee leg amputation after a minor knee trauma, while complaining for pain and leg rigidity. Her medical state worsened, probably because of repeated self-inflicted lesions and food restriction, leading to a severe undernutrition and life-threatening hypokaliemia. After some time, her medical state required amputation. This surgery did not resolve her psychological suffering. She never talked about amputation as a way to achieve herself. She did not meet the criterias for BIID. We consider her trouble as an association of a factitious disorder with a borderline personality disorder. In the last part of this paper, we discuss the <<grey>> scientific litterature about apotemnophilia and BIID. We support the idea that BIID can be considered as a culture-bound syndrome, a contemporary frame for psychological suffering. We think that BIID does not have neither intrinsic nor unequivocal psychopathological meaning. It is a <<new way to be mad>> (Elliott), a common pathway for the expression of very different kinds of psychological suffering. Apotemnophilia and BIID are raising important ethical and practical issues for psychiatrists: their opinion will probably be requested by patients and surgical teams having to deal with patient asking for healthy limbs amputation.

Mirror movements in parkinsonism: evaluation of a new clinical sign

Background: Mirror movements (MM) are not widely appreciated in parkinsonism and no report has evaluated this clinical sign in detail. Objectives: To define the parkinsonian clinical features associated with MM...

Specific antagonism of behavioral action of "uncommon" amino acids linked to motor-system diseases.

Beta-N-methylamino-L-alanine (BMAA) and beta-N-oxalylamino-L-alanine (BOAA) are chemically related amino acids present in the seeds of Cycas circinalis and Lathyrus sativus, respectively. Consumption of these seeds has been linked to Guam amyotrophic lateral sclerosis (BMAA) and lathyrism (BOAA; a form of primary lateral sclerosis). A single large dose of BOAA or BMAA causes seizures in newborn mice and postsynaptic neuronal edema and degeneration in CNS explants. We report that the acute neurotoxic actions of these amino acids are blocked selectively by specific glutamate-receptor antagonists (administered intracerebroventricularly) (i.c.v.) prior to the amino acid. Administration of BOAA i.c.v. to neonatal mice (ED100 = 50 micrograms) elicits a spectrum of time-dependent behavioral states including arm and leg rigidity, convulsions, and resting tremor. These are blocked in a dose-dependent manner by cis-2,3-piperidine dicarboxylic acid (PDA), an antagonist of quisqualate (QA)-preferring (A2) and kainate (KA)-preferring (A3) glutamate receptors (ED50s; 2.8 micrograms, rigidity; 1.4 micrograms, convulsions; 2.4 micrograms, resting tremor). BMAA induces a transitory hyperexcitable state followed by a long-lasting whole-body shake/wobble (ED100 = 1,000 micrograms, i.c.v.). These responses are antagonized selectively and dose-dependently by 2-amino-7-phosphonoheptanoic acid (AP7), an N-methyl-D-aspartate (NMDA) or A1 glutamate-receptor antagonist (ED50 = 0.45 microgram). Taken collectively, our data indicate that the acute neuronotoxic actions of BOAA and BMAA (or a metabolite) operate through different glutamate-receptor species. BMAA likely exerts most of its action indirectly via the A1 glutamate receptor, while BOAA acts principally at the A2 and/or A3 receptor.

Effects of striatal and pallidal lesions and intrapallidal GABA and opiate drugs on tremorine-induced rigidity in the rat

Tremorine-induced hindlimb rigidity was prevented by the neurotoxin kainic acid in one neostriatum. A unilateral globus pallidus (GP) electrolesion reduced the tone of the contralateral leg (CL) and differentially modified tremorine rigidity. The ipsilateral leg rigidity was reduced and the CL rigidity was increased. Resting tone and tremorine-induced rigidity were measured in CL after injection in one GP of drugs that modify pallidal GABA function. The GABA drugs tested in GP had no consistent effects on resting limb tone. By contrast, baclofen and muscimol, agonists for GABA receptors, both prevented tremorine rigidity, whereas the antagonists bicuculline and picrotoxin increased rigidity. Opiate receptor agonists in GP did not affect tone or rigidity but naltrexone prevented rigidity in CL leg. The findings suggest a close involvement of GP in mediating cholinergic limb rigidity.