Peri-mortem rigidity in a fatal case of severe salicylate poisoning.

INTRODUCTION: There has been a previous case report of peri-arrest muscle rigidity in the setting of severe salicylate poisoning (serum salicylate concentration 1,500 mg/L), described as paratonia or rapid rigor mortis. We present an image of rapid rigor mortis in another fatal salicylate poisoning. CASE SUMMARY: We report a 42-year-old male with severe salicylate poisoning (peak salicylate concentration 1,600 mg/L). During the peri-arrest period, the patient developed isotonic flexion of the upper and lower extremities, the clinical signs of rapid-occurring rigor mortis. Despite resuscitative efforts, the patient died. IMAGE: Our patient is exhibiting peri-arrest rigidity in the upper extremities. DISCUSSION: Peri-mortem rigidity is due to depletion of adenosine triphosphate. Severe salicylate poisoning causes uncoupling of oxidative phosphorylation which prevents the production of adenosine triphosphate, which is required to release myosin from actin to allow the muscle to relax. A limitation of our report is that we did not definitively exclude other uncouplers of oxidative phosphorylation, such as 2,4-dinitrophenol. However, the history of aspirin ingestion was provided by the patient and corroborated by his mother, and it was confirmed by measurement of his salicylate concentration. CONCLUSION: We hypothesize that in our patient, rapid-occurring rigor mortis likely resulted from depletion of adenosine triphosphate. This occurred as a result of uncoupling of oxidative phosphorylation in the mitochondria from severe salicylate poisoning, as adenosine triphosphate is required for muscle relaxation.

Survey of Canadian Physician Use of Botulinum Toxin A (BoNT-A) for Paratonia: Real World Insights.

Paratonia is a form of hypertonia characterized by an inability to relax muscles in the setting of cognitive impairment. Paratonia results in pain, refusal of care, and caregiver burden. We sent surveys to 67 Canadian physiatrists and neurologists regarding their experience treating paratonia with botulinum toxin A (BoNT-A). Twenty-seven survey respondents were included in the analysis. Thirteen percent of survey respondents treating paratonia with BoNT-A reported a significant clinically relevant improvement; 74% endorsed a moderately clinically relevant improvement; 13% endorsed a slight clinically relevant improvement. Ninety percent of survey respondents endorsed significant barriers in treating paratonia with BoNT-A.

From one to many: Hypertonia in schizophrenia spectrum psychosis an integrative review and adversarial collaboration report.

Different types of resistance to passive movement, i.e. hypertonia, were described in schizophrenia spectrum disorders (SSD) long before the introduction of antipsychotics. While these have been rediscovered in antipsychotic-naïve patients and their non-affected relatives, the existence of intrinsic hypertonia vs drug-induced parkinsonism (DIP) in treated SSD remains controversial. This integrative review seeks to develop a commonly accepted framework to specify the putative clinical phenomena, highlight conflicting issues and discuss ways to challenge each hypothesis and model through adversarial collaboration. The authors agreed on a common framework inspired from systems neuroscience. Specification of DIP, locomotor paratonia (LMP) and psychomotor paratonia (PMP) identified points of disagreement. Some viewed parkinsonian rigidity to be sufficient for diagnosing DIP, while others viewed DIP as a syndrome that should include bradykinesia. Sensitivity of DIP to anticholinergic drugs and the nature of LPM and PMP were the most debated issues. It was agreed that treated SSD should be investigated first. Clinical features of the phenomena at issue could be confirmed by torque, EMG and joint angle measures that could help in challenging the selectivity of DIP to anticholinergics. LMP was modeled as the release of the reticular formation from the control of the supplementary motor area (SMA), which could be challenged by the tonic vibration reflex or acoustic startle. PMP was modeled as the release of primary motor cortex from the control of the SMA and may be informed by subclinical echopraxia. If these challenges are not met, this would put new constraints on the models and have clinical and therapeutic implications.

Paratonia, Gegenhalten and psychomotor hypertonia Back to the roots.

In the first half of the 20th century, well before the antipsychotic era, paratonia, Gegenhalten and psychomotor hypertonia were described as new forms of hypertonia intrinsic to particular psychoses and catatonic disorders. A series of astute clinical observations and experiments supported their independence from rigidity seen in Parkinson's disease. After World War II, motor disorders went out of fashion in psychiatry, with drug-induced parkinsonism becoming the prevailing explanation for all involuntary resistance to passive motion. With the 'forgetting' of paratonia and Gegenhalten, parkinsonism became the prevailing reading grid, such that the rediscovery of hypertonia in antipsychotic-naive patients at the turn of the 21st century is currently referred to as "spontaneous parkinsonism", implicitly suggesting intrinsic and drug-induced forms to be the same. Classical descriptive psychopathology gives a more nuanced view in suggesting two non-parkinsonian hypertonias: (i) locomotor hypertonia corresponds to Ernest Dupré's paratonia and Karl Kleist's reactive Gegenhalten; it is a dys-relaxation phenomenon that often needs to be activated. (ii) Psychomotor hypertonia is experienced as an admixture of assistance and resistance that partially overlaps with Kleist's spontaneous Gegenhalten, but was convincingly isolated by Henri Claude and Henri Baruk thanks to electromyogram recordings; psychomotor hypertonia is underpinned by "anticipatory contractions" of cortical origin, occurrence of which in phase or antiphase with the movement accounted for facilitation or opposition to passive motions. This century-old knowledge is not only of historical interest. Some results have recently been replicated in dementia and as now known to involve specific premotor systems.

Paratonía: un signo relevante en neuropsiquiatría / Paratonia: a relevant sign in neuropsychiatry

La paratonía es una alteración del tono motor, de interés en neuropsiquiatría por su presencia en la semiología de la demencia y la catatonía. En la paratonía, la resistencia a la manipulación pasiva es errática y depende de la intensidad del movimiento impuesto. Habitualmente se divide en un subtipo oposicionista (gegenhalten) y un subtipo facilitador (mitgehen). Desde el campo de las demencias, se han realizado esfuerzos para establecer una definición de consenso de la paratonía y para describir diferentes maniobras exploratorias, lo que contrasta con la indefinición y heterogeneidad conceptual que se encuentra en la literatura psiquiátrica alrededor de este término, que aún así, aparece en múltiples escalas y criterios diagnósticos de catatonía. Esto supone un problema en el contexto de las dificultades generales para definir y operacionalizar los signos característicos de la catatonía y la creación de instrumentos de evaluación aceptados universalmente. En el presente artículo se revisa la literatura científica de la paratonía con el objetivo de plasmar este problema, así como fomentar que los avances en su estudio desde otros campos puedan servir como referencia para su estudio en psiquiatría. (AU)

Paratonia is motor tone disturbance, of interest in neuropsychiatry due to its involvement in the semiology of dementia and catatonia. In paratonia, resistance to passive manipulation is erratic and depends on the intensity of the imposed movement. It is usually divided into an oppositional subtype (gegenhalten) and a facilitatory subtype (mitgehen). Efforts have been made in the field of dementia to establish a consensus definition of paratonia and to describe different exploratory manoeuvres. This contrasts with the conceptual ambiguity and heterogeneity found in the psychiatric literature regarding this term, which still appears in multiple scales and diagnostic criteria for catatonia. This is a problem given the general difficulties in defining and operationalising the characteristic signs of catatonia and in the creation of universally accepted assessment instruments. This article provides a review of scientific literature on paratonia to describe this problem, and encourage advances made in its research by other fields that could serve as a reference for its study in psychiatry. (AU)

Muscle Tone Physiology and Abnormalities.

The simple definition of tone as the resistance to passive stretch is physiologically a complex interlaced network encompassing neural circuits in the brain, spinal cord, and muscle spindle. Disorders of muscle tone can arise from dysfunction in these pathways and manifest as hypertonia or hypotonia. The loss of supraspinal control mechanisms gives rise to hypertonia, resulting in spasticity or rigidity. On the other hand, dystonia and paratonia also manifest as abnormalities of muscle tone, but arise more due to the network dysfunction between the basal ganglia and the thalamo-cerebello-cortical connections. In this review, we have discussed the normal homeostatic mechanisms maintaining tone and the pathophysiology of spasticity and rigidity with its anatomical correlates. Thereafter, we have also highlighted the phenomenon of network dysfunction, cortical disinhibition, and neuroplastic alterations giving rise to dystonia and paratonia.

The role of inflammaging and advanced glycation end products on paratonia in patients with dementia.

Impaired motor function is a prominent characteristic of aging. Inflammatory processes and oxidative stress from advanced glycation end-products are related to impaired motor function and could plausibly be a contributing factor to the pathogenesis of paratonia, a specific motor disorder in people with dementia. Severe paratonia results in a substantial increase of a caretaker's burden and a decrease in the quality of life. The pathogenesis of paratonia is not well understood, and no effective interventions are available to combat it. Intensive glycaemic control, reducing oxidative stress, possibly combined with a low AGE diet and AGE targeting medication may be the key method for preventing advanced glycation end-product accumulation and reducing the inflammatory burden as well as possibly postponing or preventing paratonia.

Paratonia in Dementia: A Systematic Review.

BACKGROUND: Paratonia is a dementia-induced motor abnormality. Although paratonia affects virtually all people with dementia, it is not well known among clinicians and researchers. OBJECTIVE: The aim of this study was to perform a systematic review of the literature on the definition, pathogenesis, diagnosis, and intervention of paratonia as well as to propose a research agenda for paratonia. METHODS: In this systematic review, the Embase, PubMed, CINAHL, and Cochrane CENTRAL databases were searched for articles published prior to December 2019. Two independent reviewers performed data extraction and assessed the risk of bias of the studies. The following data were extracted: first author, year of publication, study design, study population, diagnosis, assessment, pathogenesis, therapy and interventions. RESULTS: Thirty-five studies met the inclusion criteria and were included. Most studies included in the review mention clinical criteria for paratonia. Additionally, pathogenesis, method of assessment, diagnosis, and paratonia severity as are interventions to address paratonia are also discussed. CONCLUSION: This systematic review outlines what is currently known about paratonia, as well as discusses the preliminary research on the underlying mechanisms of paratonia. Although paratonia has obvious devastating impacts on health and quality of life, the amount of research to date has been limited. In the last decade, there appears to have been increased research on paratonia, which hopefully will increase the momentum to further advance the field.

Managing Paratonia in Persons With Dementia: Short-term Effects of Supporting Cushions and Harmonic Techniques.

OBJECTIVES: Paratonia, a form of hypertonia typically seen in dementia, is often associated with difficulties in positioning and daily care. No evidence-based therapy or clinical guideline for management is available. In this study, the short-term effect of harmonic techniques (HT) and supporting cushions (SC) on paratonia was explored. DESIGN: This was a multicenter interventional clinical trial with AB/BA crossover design. Each intervention (SC or HT) was subsequently implemented over 1 week in each of the participants. SETTING AND PARTICIPANTS: The study included 22 participants with moderate to severe paratonia from 9 different nursing homes in Flanders, Belgium. METHODS: Measurements of biceps brachii and rectus femoris muscle tone (MyotonPRO), maximal elbow and knee extension (goniometer), and pain (Pain Assessment Checklist for Seniors With Limited Ability to Communicate) were performed on 3 different days within 1 week. The effect of HT on nursing care was evaluated with the Pain Assessment Checklist for Seniors With Limited Ability to Communicate and visual analog scale ratings of discomfort items. RESULTS: After 30 minutes of positioning with SC, participants had lower biceps brachii muscle tone (P = .041) and higher maximal elbow extension (P = .006) than without SC. After a 30-minute session of HT, a significant increase in biceps brachii muscle tone (P = .032) and maximal extension of elbow (P < .001) and knee (P = .028) was found. Pain (P = .003) and discomfort (P = .001 to P = .019) during morning care were significantly lower when care was preceded by 30 minutes of HT. CONCLUSIONS/IMPLICATIONS: This explorative study revealed beneficial short-term effects on range of motion for both SC and HT and a positive effect of SC on upper limb muscle tone. Beneficial effects of HT were found on resident's pain and caregiver's discomfort during care. The results of the present study are encouraging and can contribute to the development of evidence-based interventions for paratonia.

Bringing order to higher order motor disorders.

Majority of movements in everyday situations are complex and involve volition, planning of the movement and selection of the motor programme, all occurring before movement execution. Higher order motor disorders may be defined as abnormal motor behaviours resulting from disruption of any of the cortical processes that precede execution of the motor act. They are common in patients with neurodegenerative disorders, psychiatric diseases and structural brain lesions. These abnormal behaviours may be overlooked in the clinic, unless specifically evoked by the examiner. We discuss clinical and pathophysiological aspects of higher order motor disorders including: (1) disorders of disinhibition, such as grasp reflex and grasping behaviour, utilisation and imitation behaviour, motor preservations and paratonia; (2) disorders of motor intention such as motor neglect and motor impersistence; (3) alien limb syndrome; and (4) motor overflow phenomena, such as mirror movements and synkinesias. A video illustration of each phenomenon is provided. We place the findings from recent neurophysiological studies within the framework of theories of motor control to provide better insight into pathophysiology of different disorders.

Paratonia in Flemish Nursing Homes: Current State of Practice.

BACKGROUND: Paratonia is a major underlying motor problem impeding functionality and locomotion in dementia. Despite its undeniable impact on patient's quality of life and daily care, there is a lack of evidence-based therapy on patients with this condition. METHODS: We surveyed physiotherapists working in nursing homes in Flanders (Belgium) concerning the use and perceived effect of therapeutic strategies and positioning methods/aids. RESULTS: Positioning and soft passive mobilization were the most applied and positively appraised therapeutic interventions. Highest ratings for positioning were found for C-shaped positioning cushions and multi-position wheelchair. According to the respondents, key points for paratonia approach were relaxation, positioning, active movement stimulation, and-to some extent-passive mobilization. Indispensable for any success however is multidisciplinary cooperation. CONCLUSION: These findings might provide an inspirational path for research to verify possible (evidence based) beneficial treatments that could be applied to improve current and future treatment of patients with paratonia.

Psychometric Properties of the MyotonPRO in Dementia Patients with Paratonia.

BACKGROUND: Paratonia is a distinctive form of hypertonia, causing loss of functional mobility in early stages of dementia to severe high muscle tone and pain in the late stages. For assessing and evaluating therapeutic interventions, objective instruments are required. OBJECTIVE: Determine the psychometric properties of the MyotonPRO, a portable device that objectively measures muscle properties, in dementia patients with paratonia. METHODS: Muscle properties were assessed with the MyotonPRO by 2 assessors within one session and repeated by the main researcher after 30 min and again after 6 months. Receiver operating characteristic curves were constructed for all MyotonPRO outcomes to discriminate between participants with (n = 70) and without paratonia (n = 82). In the participants with paratonia, correlation coefficients were established between the MyotonPRO outcomes and the Modified Ashworth Scale for paratonia (MAS-P) and muscle palpation. In participants with paratonia, reliability (intraclass correlation coefficient) and agreement values (standard error of measurement and minimal detectable change) were established. Longitudinal outcome from participants with paratonia throughout the study (n = 48) was used to establish the sensitivity for change (correlation coefficient) and responsiveness (minimal clinical important difference). RESULTS: Included were 152 participants with dementia (mean [standard deviation] age of 83.5 [98.2]). The area under the curve ranged from 0.60 to 0.67 indicating the MyotonPRO is able to differentiate between participants with and without paratonia. The MyotonPRO explained 10-18% of the MAS-P score and 8-14% of the palpation score. Interclass correlation coefficients for interrater reliability ranged from 0.57 to 0.75 and from 0.54 to 0.71 for intrarater. The best agreement values were found for tone, elasticity, and stiffness. The change between baseline and 6 months in the MyotonPRO outcomes explained 8-13% of the change in the MAS-P scores. The minimal clinically important difference values were all smaller than the measurement error. CONCLUSION: The MyotonPRO is potentially applicable for cross-sectional studies between groups of paratonia patients and appears less suitable to measure intraindividual changes in paratonia. Because of the inherent variability in movement resistance in paratonia, the outcomes from the MyotonPRO should be interpreted with care; therefore, future research should focus on additional guidelines to increase the clinical interpretation and improving reproducibility.

Electromyographic assessment of paratonia.

Many years after its initial description, paratonia remains a poorly understood concept. It is described as the inability to relax muscles during muscle tone assessment with the subject involuntary facilitating or opposing the examiner. Although related to cognitive impairment and frontal lobe function, the underlying mechanisms have not been clarified. Moreover, criteria to distinguish oppositional paratonia from parkinsonian rigidity or spasticity are not yet available. Paratonia is very frequently encountered in clinical practice and only semi-quantitative rating scales are available. The purpose of this study is to assess the feasibility of a quantitative measure of paratonia using surface electromyography. Paratonia was elicited by performing consecutive metronome-synchronized continuous and discontinuous elbow movements in a group of paratonic patients with cognitive impairment. Goniometric and electromyographic recordings were performed on biceps and triceps brachii muscles. Facilitatory (mitgehen) and oppositional (gegenhalten) paratonia could be recorded on both muscles. After normalization with voluntary maximal contraction, biceps showed higher paratonia than triceps. Facilitatory paratonia was higher than oppositional on the biceps. Movement repetition induced increased paratonic burst amplitude only when flexion and extension movements were performed continuously. Both facilitatory and oppositional paratonia increased with movement repetition. Only oppositional paratonia increased following faster movements. This is the first study providing a quantitative and objective characterization of paratonia using electromyography. Unlike parkinsonian rigidity, oppositional paratonia increases with velocity and with consecutive movement repetition. Like spasticity, oppositional paratonia is velocity-dependent, but different from spasticity, it increases during movement repetition instead of decreasing. A quantitative measure of paratonia could help better understanding its pathophysiology and could be used for research purposes on cognitive impairment.

How to assess tendon reflexes of the lower limb in the elderly.

BACKGROUND: Clinicians frequently experience difficulty in eliciting the reflexes of elderly patients using standard methods due to paratonia/frontal rigidity. If reflexes are incorrectly thought to be absent, important diagnostic errors may be made. Neurologists use alternative methods when technical difficulties require them, but these are not widely used by non-neurologists. METHODS: A neurologist and a medical student both used standard and non-standard techniques to assess reflexes of the lower limb in geriatric inpatients, aged over 65, to determine which method permitted the most confident assessment of the presence of knee and ankle reflexes. RESULTS: 45 patients were assessed. The consultant found that in 20 patients (44%) all three knee reflex methods examined produced similar results. When the methods produced different results, the "superior patellar supine" method was the best single method overall (best or equal best in 19 patients (42%)). For the ankle reflex all four reflex methods examined produced similar results in only 7 patients (16%). When the methods produced different results the "Achilles strike elevated" method was best or equal best in 32 patients (71%) and the "plantar strike" method in 29 patients (64%). If the student had relied on standard methods alone, reflexes would have been incorrectly called absent in 28 limbs (37%) for knee jerks and 52 limbs (84%) for ankle jerks. Supplementing standard methods with alternative methods reduced these error rates to 19% and 21% respectively. CONCLUSIONS: Our findings indicate that a reasonable practical approach is to assess the knee reflex with the standard method and then, if a definite reflex has not been recorded, move on to use the "superior patellar supine" method; and for the ankle reflex begin with the "plantar strike method" and then, if necessary, move on to use the "Achilles strike elevated" method.