|Year : 2018 | Volume
| Issue : 3 | Page : 215-221
Uraga grahonmada: Extrapyramidal movement disorder?/Tourette syndrome-plus?
Prasad Mamidi, Kshama Gupta
Department of Kaya Chikitsa, Abhilashi Ayurvedic College & Research Institute, Abhilashi University, Mandi, Himachal Pradesh, India
|Date of Web Publication||25-Sep-2018|
Dr. Prasad Mamidi
Department of Kaya Chikitsa, Abhilashi Ayurvedic College & Research Institute, Abhilashi University, Mandi, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
Uraga/Sarpa/Bhujaga grahonmada (UG) is one among the 18 types of bhootonmada. Bhootonmada denotes a category of psychiatric/neuropsychiatric conditions assumed to be caused by affliction of evil spirits (bhuta/graha). Till date, no studies have been conducted on UG and it is unexplored. The present study is focused on the better understanding of UG and its clinical applicability. UG is characterized by features of Krodhanam (aggressiveness/impulsivity), Nishwasantam (hyperventilation/anxiety/phobia), Bhramantam (agitation/restlessness/hyperactivity), Trasyantam (startle response/hyperekplexia/anxiety/phobia), Raktaaksha (red eyes/Kayser–Fleischer rings), Stabdha drishtim (prolonged staring/abnormal eye movements), Jihwa lolayantam/Srikkinyau lihaan (facial tics), Sarpavat prasarati/Adhomukha shaayinam (athetosis/chorea/motor tics/opisthotonus), Chalam/Vakra gamanam (gait abnormalities), Ksheera, ghrita, guda, and madhu priyam (craving for sweets), Snaana maalya priyam (obsessive-compulsive features), Gaatraani kampayantam (tremors/motor tics/seizures), Dantai khaadantam (self-injurious behaviors/oromandibular dystonia/bruxism), Nidraalu (hypersomnia/excessive daytime sleepiness), etc.. The clinical picture of UG shows similarity with various “extrapyramidal movement disorders” and also with “Tourette syndrome-plus.”
Keywords: Ayurveda, extrapyramidal movement disorder, obsessive-compulsive features, tics, Tourette syndrome, Uraga grahonmada
|How to cite this article:|
Mamidi P, Gupta K. Uraga grahonmada: Extrapyramidal movement disorder?/Tourette syndrome-plus?. Indian J Health Sci Biomed Res 2018;11:215-21
| Introduction|| |
“Bhuta vidya” (Ayurvedic psychiatry) is one of the eight branches of Ayurveda which deals with incantation, mode of exorcising evil spirits, and making offerings to deva (divine beings), pishacha (class of demon fond of flesh), gandharva (class of demon fond of entertainment), yaksha (living super natural being/ghost), rakshasa (class of demon fond of violence), etc., for the cure of diseases originating due to their possession.Bhutonmada/grahonmada is a psychiatric/neuropsychiatric condition characterized by various types of abnormal behaviors. Acharya Vagbhata has described 18 types of bhutonmada. They are deva, asura, rushi, guru, vruddha, siddha, pitru, gandharva, yaksha, rakshasa, sarpa, brahma rakshasa, pishacha, kushmanda, nishada, preta, maukirana, and vetala. Sarpa/Bhujaga/Uraga grahonmada (UG) is one among these 18 types of grahonmada.
There is no description found regarding UG in Charaka samhita.Acharya Sushruta has used the term “Bhujanga/bhujaga grahonmada” for UG. According to Acharya Sushruta, UG is characterized by features such as sarpavat prasarati (athetosis/chorea/motor tics/opisthotonus), srikkinyau vilikhati jihvaya (facial tics), nidraalu (hypersomnia/excessive daytime sleepiness [EDS]), and guda, madhu, dugdha, paayasepsu (fond of jiggery, honey, milk, and sweets). In Ashtanga samgraha (written by vriddha Vagbhata) along with the above features, krodhanam (aggressiveness/impulsivity), nishwasantam (hyperventilation/anxiety/phobia), bhramantam (agitation/restlessness/hyperactivity), trasyantam (startle response/hyperekplexia [HPX]/anxiety/phobia), raktaaksha (red eyes/Kayser–Fleischer rings), stabdha drishtim (prolonged staring/abnormal eye movements), jihwa lolayantam/srikkinyau lihaan (oral tics), adhomukha shaayinam (athetosis/chorea/motor tics/opisthotonus), chalam/vakra gamanam (gait abnormalities), ksheera, ghrita, guda and madhu priyam (craving for sweets), snaana maalya priyam (obsessive-compulsive features), gaatraani kampayantam (tremors/motor tics/seizures), dantai khaadantam (self-injurious behaviors [SIBs]/oromandibular dystonia [OMD]/bruxism), etc., have been added. A similar description (as in Ashtanga samgraha) has been found in Ashtanga hridaya (written by Vagbhata). The description of UG in “Madhava nidaana” is similar to the description of Acharya Sushruta.
Till date, no studies have been conducted on UG and it is an underexplored topic in Ayurvedic psychiatry. The present study is focused on the better understanding of UG and its clinical applicability. The clinical picture of UG shows similarity with various “extrapyramidal movement disorders” (EPMDs) and also with “Tourette syndrome-plus” (TS-Plus) (is a comorbid condition of TS + attention-deficit/hyperactivity disorder [ADHD] + obsessive-compulsive disorder [OCD] + other associated behavioral and/or mood disorders). This similarity has been explored in the following sections.
| Extrapyramidal Movement Disorder|| |
The control of voluntary movement is affected by the interaction of the pyramidal, cerebellar, and extrapyramidal systems. The extrapyramidal system consists of paired subcortical masses or nuclei of gray matter basal ganglia. The effects of disease of the extrapyramidal system on movement can be regarded as negative (hypokinetic) and positive (hyperkinetic). Parkinson's disease (PD), Huntington's disease (HD), dystonias, Wilson's disease (WD), and various other psychiatric/neuropsychiatric conditions come under EPMD category. Tremors, chorea (irregular, repetitive, jerking movements), athetosis (irregular, repetitive, writhing movements), dystonia (slow, sustained, abnormal movements), ballismus (explosive, violent movements), and myoclonus (shock-like jerks) are the involuntary abnormal movements observed in various EPMDs. Chorea is a sudden, quick, involuntary movement that can affect any skeletal muscle including the diaphragm. It is not rhythmic-like tremor and purposeless or patterned as tic. Athetosis is a slower movement, often called writhing or undulating that resembles deliberate stretching of a muscle group. It is most noticeable in the shoulders, fingers, and lower face. Tic is also sudden, quick, and not rhythmic, but more patterned and purposeful in outlook. Various abnormal movements described in UG such as “sarpavat prasarati,” “adhomukha shaayinam,” “gaatraani kampayantam,” and “bhramantam” indicate various abnormal involuntary movements seen in EPMD.
| Tourette Syndrome-Plus|| |
TS is a childhood-onset neuropsychiatric disorder and it is characterized by a combination of multiple motor and vocal tics. It is a chronic disorder with repetitive cycles of waxing and waning and distinguished by erratic movements of the whole body. Patients with TS commonly have additional neuropsychiatric disorders such as ADHD, OCD, and mood disorders. Increased incidence of anxiety, insomnia, hostility, coprolalia, SIB, and personality disorders has also been observed in TS. TS is characterized by simple and complex tics. The incidence of tics in the absence of other associated features and comorbidities occurs in only around 10% of cases (pure TS), while the remainder have a number of associated comorbidities (TS-Plus). In addition to the core clinical features that are integral to TS, a wide range of other behavioral, emotional, and psychiatric conditions have been reported to occur more commonly in TS (e.g., ADHD in 60% of the TS population and OCD in 30%–50% of the TS population). A number of co-existent psychopathologies have also been described in TS including anxiety, depression, learning difficulties, personality disorder, impulse control, aggression, oppositional defiant disorder, and conduct disorder.
| Etiology, Pathogenesis, and Prognosis of Uraga Grahonmada|| |
No separate description is available in Ayurvedic texts regarding the etiology, pathogenesis, and prognosis for UG. The samaanya nidaana, sampraapti, and saadhyaasaadhyata (common etiology, pathogenesis, and prognosis) explained for grahonmada are applicable for UG also. Grahavesha (affliction by evil spirit) and prajnaparadha/karma (cognitive blasphemy/deeds of present or previous life) are explained as causative factors for grahonmada. In bhutonmada, the symptoms occur suddenly without any visible or known reason. The course and prognosis of grahonmada is also unpredictable in nature.
EPMDs are common in psychiatric patients, and psychiatric syndromes are quite common in patients with extrapyramidal disorders. The most important hyperkinetic movement disorders in psychiatry feature chorea and athetosis. They can also be caused by HD, Sydenham's chorea (associated with a history of streptococcal infection), WD (a disorder of copper metabolism, usually of adolescent onset), vascular lesions, and numerous other rare disorders. The tic disorders are another type of hyperkinetic movement disorder, dominated by motor and vocal tics. These are named TS and are often comorbid with OCD and ADHD. Tics are a feature of pediatric autoimmune neuropsychiatric disorder associated with streptococcal infection. Multiple lesions affecting thalamus, caudate head, putamen, globus pallidus, midbrain, and the internal capsule were related with various generalized involuntary movements such as chorea, hemichorea, hemiballismus, and choreo-athetoid movements. The onset of TS shows genetic heritability with differing penetrance, and the pathological mechanisms of TS are still unclear. The pathophysiology of EPMDs is also still unknown. Such type of lesions in basal ganglia or extrapyramidal structures of the brain can be visualized or identified only by radiography or some other invasive procedures but may not be visible or traceable to the external eye (similar to the description of bhootonmada/grahonmada in which the etiology and pathogenesis are untraceable/idiopathic).
| Similarity between Lakshanas (Signs and Symptoms) of Uraga Grahonmada and Extrapyramidal Movement Disorder/tourette Syndrome-Plus|| |
Compared to other Ayurvedic texts, Vriddha Vagbhata in “Ashtanga samgraha” has described the lakshanas (signs and symptoms) of UG in a detailed way. EPMD and TS-plus resemble UG in signs and symptoms. Each and every lakshana of UG along with its relevant modern psychiatric/neuropsychiatric conditions is correlated as follows.
Poor impulse control is one of the features of TS. Antisocial behavior, aggressive behavior, discipline problems, and SIB have been reported in a significant proportion of TS patients. Impulsivity is defined as the failure to resist an impulse, drive, or temptation that is potentially harmful to self or others. Impulsivity is evidenced behaviorally as carelessness, an underestimated sense of harm, extroversion, impatience, inability to delay gratification, tendency toward risk taking, and pleasure and sensation seeking. It has been found that aggressive outbursts are found to be higher in patients of TS with ADHD and OCD (70.6%) compared to TS with ADHD (56.7%) or TS alone (36.7%). SIB occurs in up to 60% of patients with TS. A variety of SIBs have been reported in individuals with TS, including compulsive skin picking, self-hitting, lip and other self-biting, filing of the teeth, head banging, and eye damage from self-poking. Impulse dysregulation is seen in a number of neuropsychiatric disorders that affect children with TS, including ADHD, depression, and bipolar disorder.Krodhanam of UG is similar to the aggressive behavior or impulsivity seen in various EPMD or TS-plus.
Trasyantam and Nishwasantam (startle response/hyperekplexia/anxiety/phobia and hyperventilation)
“Trasyantam” of UG indicates excessive fear, whereas “Nishwasantam” indicates hyperventilation due to excessive fear. The startle response is a normal primitive reaction which consists of a stereotyped pattern of movements such as blinking, facial grimacing, and flexion of the head, shoulders, elbows, trunk, and knees. It may be elicited by any unexpected stimuli and decrease in magnitude on repeated stimulation. Response magnitude is influenced by individual factors such as personality, anxiety or fatigue, sleep, sensory deprivation, and the presence of a range of neurological and psychiatric conditions. The exaggerated startle response is a feature of many organic and psychiatric disorders. The association between excessive startle and sensitive personality or neurosis has been made. The relationship between startle and epilepsy has been found. The startle reaction may be so intense as to precipitate an epileptic seizure.
Startle syndromes are divided into three categories, namely HPX, stimulus-induced disorders, and neuropsychiatric disorders. There is no loss of consciousness during startle responses. Startle-induced or reflex tics may occur as a part of TS as well as an independent phenomenon. Tics are typically provoked by internal, but occasionally by external stimuli, such as startle. The stimulus-induced disorders and neuropsychiatric startle syndromes are diverse, heterogeneous with overlapping features, paroxysmal in nature, and frequently provocated by an external stimulus. Anxiety is also common in TS patients. Anxiety symptoms found in TS patients are sleep difficulties, tension habits, motor unrest, phobias, worries, and poor concentration. The symptoms of “Trasyantam aatapatraat” (afraid of the umbrella), “jalaugha ghanadu nibhisvanai hrishyantam” (becoming excited by the sound of water), etc., indicate “startle response” or “phobia” or “anxiety” which are commonly seen in or associated with various EPMD and TS-plus.
The essential feature of ADHD is a persistent pattern of inattention and/or hyperactivity impulsivity that is more frequently displayed and more severe in individuals. Hyperactivity may be manifested by fidgetiness, squirming, unable to sit at one place, excessive running or climbing, often “on the go,” seems like “driven by a motor,” tapping hands, and shaking feet or legs excessively and restlessness. ADHD appears at least 50% of clinic-referred individuals with TS. When the two disorders (ADHD and TS) coexist, the onset of the ADHD often precedes the onset of the TS. “Bhramantam” of UG indicates hyperactivity of ADHD comorbid with TS.
Raktaaksha and Stabdha drishtim (red eyes/Kayser–Fleischer rings and prolonged staring/abnormal eye movements)
Red eyes and abnormal eye movements are found in various EPMDs. WD is an autosomal recessive inborn error of metabolism that results in excessive copper deposition in the liver, kidney, and other vital organs. A case report has documented congested red eyes and itching in both eyes in a patient with WD. Characteristic copper-colored Kayser–Fleischer (K-F) rings, encircling with the peripheral cornea, adjacent to the limbus in both eyes, has been found in a case of WD. In a report of WD, one-third of patients present with liver disease, another third with neurologic manifestations, and others with psychiatric and behavioral manifestations. Movement disorders are increasingly diagnosed as a potential complication of acquired immunodeficiency syndrome (AIDS) and may sometimes represent the initial manifestation of human immunodeficiency virus infection. A case report has revealed bilateral papilledema on fundus examination with generalized choreo-athetoid movements and bilateral clonus in a patient of AIDS with encephalopathy. “Raktaaksha” of UG may indicate K-F rings or some other ophthalmic pathology due to the underlying EPMD or TS-plus.
Cardinal signs of PD include “low spontaneous blink rate” and blunted affect. Supranuclear gaze palsy, cerebellar ataxia, pyramidal tract signs, myoclonus, etc., indicate the other parkinsonian diseases. Simple motor tics include various eye movements such as blinking, squinting, rolling, and opening eyes wide. Various complex motor tics related to eye movements are “looking surprised” or “looking to one side for a brief period of time,” etc. Limitation of upward gaze is more common than limitation of downward gaze in neurodegenerative disorders. Dysfunction of voluntary eyelid movements is a characteristic feature of progressive supranuclear palsy and includes reduced blinking, voluntary eyelid motility impairment, and even full-blown blepharospasm. Slowing of eye opening or eye closure are late signs. “Stabdha drishtim” of UG indicates prolonged staring or various abnormal eye movements seen in various EPMDs.
Jihwa lolayantam/Srikkinyau lihaan (facial simple or complex motor tics)
Tic disorders newly presenting during adulthood have occasionally been described in the neurological literature, mostly in relation to an acquired brain lesion, or as incidental tics in the context of another neurological or psychiatric disease. In a patient, stereotyped tongue movements consisted of a tendency for the tongue to curl back and press itself against the upper teeth have been observed. Single tongue-thrusting tics are found in a patient suffering with tic disorder. Simple motor tics of face are characterized by biting, chewing, licking the lip and teeth grinding. Sticking out the tongue is a complex motor tic of the face. Patients with TS often demonstrate various complex movements such as licking. Licking things with tongue or idiosyncratic mannerisms (e.g., of the lips and tongue) are the features of TS with OCD. Thus, “Jihwa lolayantam/Srikkinyau lihaan,” i.e., abnormal movements of the tongue and licking the corners of the mouth with tongue resemble tics or idiosyncratic mannerisms commonly seen in TS and/or OCD.
Sarpavat prasarati/Adhomukha shaayinam (athetosis/chorea/complex motor tics/opisthotonus)
TS is one of the common causes of motor and phonic tics. Complex motor tics produce more coordinated movements mimicking normal motor function. Complex motor tics often occur out of their normal context or in inappropriate situations and they are exaggerated, forceful, and repetitive in nature. Chorea, dystonia, myoclonus, etc., are other abnormal involuntary movements which may be misinterpreted as tics. Patients with TS often demonstrate various complex motor movements such as touching, licking, spitting, jumping, smelling, squatting, and abnormalities of gait and forced touching. Dystonic tics are more likely to be associated with premonitory sensations. Athetosis is a writhing, sinuous distal limb movement which is now often classified as dystonia and ballism linked with chorea. Opisthotonus is a state of extreme hyperextension of the head, neck, and spine that presents as severe, prolonged spasm characterized by a strongly arched and rigid back, hyperextended neck and arms, with the heels bent back. It is known to occur in tetanus, rabies, cerebral malaria, neurosyphilis, meningitis, encephalitis, cerebral palsy, partial seizures, strychnine poisoning, and adverse effects of medications. Choreoathetotic movements are an abrupt, irregular movement (chorea) that seems to launch a writhing or stretching movement (athetosis). Athetosis or choreoathetosis is a snake-like writhing or stretching movement which is commonly seen in various EPMDs and resembles “sarpavat prasarati“/“Adhomukha shaayinam” of UG.
Chalam/Vakra gamanam (gait abnormalities)
Extrapyramidal signs include increased motor tone, changes in the amount and velocity of movement, and involuntary motor activities. Cardinal signs of PD follow the mnemonic TRAP: tremor (rest), rigidity (lead pipe), akinesia (or bradykinesia), and postural instability. Other “parkinsonian” signs include hypophonia, micrographia, blunted affect, low spontaneous blink rate, stooped posture, short-stepped gait, motor restlessness (akathisia), and dystonia. Gait in PD is characteristic for short steps, diminished arm swing, and festination. Festination is a tottering forward leaning gait, in which fairly rapid but short steps fail to keep up with the center of gravity. The patient often looks unable to rotate at the waist, sometimes called “en bloc turning.” “Chalam” or “vakra gaaminam” indicates ataxia or festinating gait or akathisia which is commonly found in PD or EPMDs.
Ksheera, ghrita, guda, and madhu priyam (craving for sweets)
Consumption of diet low in carbohydrate tends to precipitate depression, since the production of brain chemicals such as serotonin and tryptophan that promote the feeling of well-being is triggered by carbohydrate-rich foods. Foods rich in high-quality protein include meats, milk and other dairy products, and eggs. Many of the neurotransmitters in the brain are made from amino acids. The neurotransmitter dopamine is made from the amino acid tyrosine and the neurotransmitter serotonin is made from the tryptophan. If there is a lack of any of these two amino acids, there will not be enough synthesis of the respective neurotransmitters, which is associated with low mood and aggression in the patients. Higher proportion of PD patients identified themselves as craving for sweets than controls, and a higher proportion scored over the 75th percentile on a score of craving. Craving sweets significantly correlated with duration of PD, but not with other factors. Craving for sweets in PD may be an expression of dopamine-mediated reward systems.Ksheera, ghrita, guda, and madhu priyam (fond of milk, ghee, jiggery, honey, and sweet foods) of UG indicate cravings toward food rich in carbohydrates/sugars/amino acids/proteins. Such type of cravings toward sweets can be found in PD-like EPMDs and other neurodegenerative conditions.
Snaana maalya priyam (obsessive-compulsive features)
“Snaana maalya priyam” of UG denotes that the patient of UG has been developed interest in wearing garlands, using perfumes, and preferring hygienic environment. This sudden change of behavior (excessive hygiene/rituals/religious/fear of contamination) without any visible or known cause denotes OCD. Excessive showering, bathing, grooming, cleaning, or washing compulsions, excessive ritualized hand washing, fear of contamination, etc., are features of OCD  that resemble “Snaana maalya priyam” of UG.
Gaatraani kampayantam (tremors/motor tics/seizures)
The tremor of Parkinsonism More Details is of the rest variety. Rest tremor is noted in a limb or other body part at rest, particularly with distraction. It can be intensified by anxiety. When the person extends the hands unsupported, rest tremor ceases, but it may resume after several seconds maintaining the posture. When rest tremor is prominent in the second digit, so that it rubs against the palmar surface of the thumb, it is called “pill-rolling tremor.” Tremor, dystonia, chorea, and myoclonus are dyskinesias recognized to reflect various neurological diseases. Tics and stereotypies are distinct motor behaviors seen commonly in neuropsychiatric practice and are frequent in movement disorder clinics. Other odd motor behaviors seen in neurological practice include akathisia, compulsions and rituals, apraxias, cramps, geniospasm, mannerisms, hemifacial spasms, and myokymias. When the tics are present for longer than a year and are accompanied by abnormal vocalizations, the diagnosis of TS can be made. “Gaatraani kampayantam” of UG indicates tremors of PD or tics of TS or other abnormal movements seen in various EPMDs.
Dantai khaadantam (self-injurious behaviors/oromandibular dystonia/bruxism)
The terms OMD, craniocervical dystonia, or Meige syndrome describe a focal or segmental dystonia whereby repetitive sustained spasms of the masticatory, facial, or lingual muscles result in painful, involuntary movement of the jaws. OMD is considered as a focal dystonia involving mouth, jaw, and tongue, manifested by involuntary muscle contractions producing repetitive, patterned movements of the involved structures. Dystonia is the most common prevalent movement disorder next only to PD and essential tremor. Tourette is defined as part of spectrum of tic disorder, which includes provisional, transient, and persistent (chronic) tics. Various facial abnormal involuntary movements are the most common tics seen in tic disorders. SIB occurs in up to 60% of patients with TS, although estimates vary depending on the definition of self-injury. A variety of SIBs have been reported in individuals with TS, such as compulsive skin picking, self-hitting, lip and other self-biting, filing of the teeth, head banging, and eye damage from self-poking. SIB in other neuropsychiatric disorders appears to be related to problems with impulse control. Impulse dysregulation is seen in a number of neuropsychiatric disorders that affect children with TS, including ADHD. Biting, chewing, teeth baring, or teeth grinding are simple motor tics related to face.Dantai khaadantam of UG denotes bruxism or OMD or SIB commonly seen in various EPMDs or TS-plus.
Nidraalu (hypersomnia/excessive daytime sleepiness)
Sleep disorders are one of the common comorbid conditions for TS. Approximately 20%–45% of the TS patients have comorbid sleep disorders. Their etiology seems to be multifactorial. In ADHD, commonly associated with sleep disorders, there is initial insomnia, sleepwalking, and hypersomnia. ADHD and TS are characterized by prominent and specific changes in polysomnographic parameters, indicating that the neural substrates of ADHD and TS are involved in sleep regulation dissimilarly. In patients with ADHD + TS, both ADHD- and TS-related changes in sleep persist independently, and no sleep alterations specific for comorbidity were found. Consequently, coexisting ADHD and TS may be a combination of two independent pathologies, and therefore, TS and its comorbidities may lead to sleep disorders. Sleep disorders are the most prevalent nonmotor symptoms in PD. EDS and “sleep attacks” affect half of the patients with PD and can also precede disease onset. A sudden onset of sleep during the day is a phenomenon in PD which resembles narcolepsy. “Nidraalu” as mentioned in UG may not directly indicate any feature found in EPMD or TS-plus, but it may indirectly suggest EDS which may be due to insomnia/parasomnia/other sleep disorders seen in TS-plus.
| Conclusion|| |
“UG” is one among the 18 types of grahonmada/bhootonmada. The clinical picture of UG has shown similarity with various EPMDs and also with TS-plus.
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