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Juvenile Paralysis Agitans of Hunt

Parkinsonian-Pyramidal Syndrome


Presentation

  • A distinction is made between paralysis and paresis, the latter being a condition in which motor functions are weakened but present.[encyclopedia2.thefreedictionary.com]
  • PMID: 26864383 (This article is OPEN ACCESS if you would like to read it) The researchers who wrote this article were presented with a 10 member Indian family from Aligarh, Uttar Pradesh.[scienceofparkinsons.com]
  • After 30 years under levodopa she still presents a moderate response but with severe fluctuations of the motor performance.[scielo.br]
  • The present study also revealed that in CPD cases significant numbers of LBs could be present in the cerebral cortex, amygdaloid and claustrum. These lesions can be in part responsible for dementia in CPD.[link.springer.com]
  • Abstract We report a case of juvenile Parkinson's disease which initially presented as bulbar incoordination at the age 12. The condition was characterized by dystonia of the upper extremities. The patient was a 14-year-old female.[ncbi.nlm.nih.gov]
Falling
  • A progressive, degenerative neurologic disease characterized by a TREMOR that is maximal at rest, retropulsion (i.e. a tendency to fall backwards), rigidity, stooped posture, slowness of voluntary movements, and a masklike facial expression.[curehunter.com]
  • Hypokalemic Periodic Paralysis September 27, 2018 by Peter Ciszewski Hypokalemic periodic paralysis (HOKPP) is characterized by episodes of muscle paralysis associated with a fall in blood potassium levels (hypokalemia).[checkrare.com]
  • Complications of Parkinson’s such as falling-related injuries or pneumonia.[medicinenet.com]
  • Linear fall 7 Anatomy-Basal Ganglia Internal capsule Putamen Caudate Nucleus Globus pallidus 8 PATHOPHYSIOLOGY 9 GLOBUS, PARS RETICULATA PATHOPHYSIOLOGY DA SUBSTANTIA NIGRA GLOBUS, PARS RETICULATA ACH GABA STRIATUM CAUDATE, PUTAMEN 10 Chemical Balance[slideplayer.com]
Inflammation
  • Paralyses must be distinguished from motor disorders associated with inflammation of muscles and lesions of bones and joints, which restrict the range of movement mechanically.[encyclopedia2.thefreedictionary.com]
  • With Polyangiitis (EPGA) January 17, 2019 by Peter Ciszewski Eosinophilic granulomatosis with polyangiitis (EGPA), also called Churg Strauss syndrome, is a rare, chronic disorder and a form of primary systemic autoimmune vasculitis characterized by inflammation[checkrare.com]
  • Genetic and pathological studies have revealed that various dysfunctional cellular processes, inflammation, and stress can all contribute to cell damage.[medicinenet.com]
  • “His application was on account of a considerable degree of inflammation over the lower ribs on the left side, which terminated in the formation of matter beneath the fascia.[scienceofparkinsons.com]
  • Reflecting therapeutic approaches of the early nineteenth century, Parkinson recommended venesection, specifically advocating bloodletting from the neck, followed by vesicatories to induce blistering and inflammation of the skin.[ncbi.nlm.nih.gov]
Goiter
  • Endocrine dysfunction, including hypergonadotropic hypogonadism, multinodular goiter, and diabetes mellitus, is present in affected adults.[mendelian.co]
  • […] of face with micrognathia -peromelia (severely deformed limbs) -small growth -normal intelligence -branchial arch deformities resulting in CHL -tongue deformities -carefully consider ear surgery due to abnormal course of facial nerve - appearance of goiter[cram.com]
Cerebral Palsy
  • Cerebral palsy cerebral palsy, disability caused by brain damage before or during birth or in the first years, resulting in a loss of voluntary muscular control and coordination.[encyclopedia2.thefreedictionary.com]
  • Dysarthria can either be: developmental - when it occurs as a result of brain damage before or during birth, such as in cerebral palsy acquired - when it occurs as the result of brain changes later in life, such as damage caused by a stroke, head injury[familydiagnosis.com]
Veterinarian
  • 例句 Dr. terry d. hunt a veterinarian from canada demonstrated the procedure used in canada for the antemortem postmortem inspection of a hog carcass before it is approved for human consumption 加拿大獸醫專家terry hunt博士,介紹了加拿大豬場質量保證計劃和加拿大屠宰場豬肉檢疫以及檢驗檢疫工作對食品安全的重要作用[dict.site]
Hypophonia
  • Hypomimia- “masked facies”,expressionless face, blinking Speech abnormalities- Hypophonia: soft voice Aprosody of speech: monotonous and lack of inflection Tachyphemia: do not separate syllables together, running words together Patients may eventually[slideplayer.com]
Muscle Rigidity
  • It is clinically characterized by movement disorders such as muscle rigidity, akinesia and tremor.[books.google.de]
  • The diagnosis of Parkinson's disease is more likely if: At least two of the three major symptoms are present (tremor at rest, muscle rigidity, and slowness) The onset of symptoms started on one side of the body Symptoms are not due to secondary causes[medicinenet.com]
Thrombosis
  • Click the link for more information., in which hemorrhage, thrombosis, or obstruction of a cerebral vessel interferes with nerve function.[encyclopedia2.thefreedictionary.com]
  • […] syndrome (Vernet) plus Horner syndrome - more extensive involvement in the region of the jugular foramen, retroparotid area, and the lateral pharyngeal space Wallenberg Syndrome (lateral medullary syndrome) - the posterior-inferior cerebellar artery thrombosis[cram.com]
Jaundice
  • Alagille syndrome Inherited neonatal jaundice, cholestasis with peripheral pulmonic stenosis, unusual facies, and ocular, vertebral, and nervous system abnormalities, due to paucity or absence of intrahepatic bile ducts.[scribd.com]
Muscular Atrophy
  • Torsten syndrome G12 Spinal muscular atrophy and related syndromes G12.0 Infantile spinal muscular atrophy, type I [Werdnig-Hoffman]. Includes: Acute [inherited] infantile type I G12.1 Other inherited spinal muscular atrophy.[emprocell.com]
  • Peripheral paralysis is characterized by the complete absence of movement, decrease in muscle tone, loss of reflexes, and muscular atrophy.[encyclopedia2.thefreedictionary.com]
  • atrophy Amyotrophic lateral sclerosis Proximal amyotrophy Action tremor Generalized muscle weakness Abnormal cerebellum morphology Psychomotor deterioration Infantile onset Abnormality of the hair Neoplasm of the pancreas Neoplasm of the breast Abnormality[mendelian.co]
  • Atrophy And Friedreich Ataxia ,Combined Chiari Malformation Type I Chorea, Benign Hereditary Choreoacanthocytosis Choreoathetosis And Congenital Hypothyroidism With Or Without Pulmonarydysfunction Classic Galactosemia Cockayne Syndrome A Coenzyme Q10[familydiagnosis.com]
  • atrophy, distal, X-linked 3; Occipital horn syndrome 007623 Bloc1s4 Bloc1s4 cno cappuccino pigmentation, hematopoietic, vision/eye, behavior, cellular, integument BLOC1S4 Hermansky-Pudlak syndrome 005420 005421 Bmp5 Bmp5 cfe-se7J cauliflower ear-short[jax.org]
Fear
  • In the dream you may be “frozen with fear” and it is up to you decipher what that fear is and what it represents.[encyclopedia2.thefreedictionary.com]
  • Allergy relief Devitalizing viral, bacterial, parasitic, and fungal infections Unburdening and strengthening the immune system Relieving pain Detoxifying the body Calming the nervous system Releasing fears and phobias Uncovering and removing obstacles[healingbyquantumleaps.com]
  • Accepting one another without fear or judgment is essential for bringing back the passion.[blog.cognifit.com]
Hysteria
  • An unusual kind of paralysis occurs in hysteria. Paralyses must be distinguished from motor disorders associated with inflammation of muscles and lesions of bones and joints, which restrict the range of movement mechanically.[encyclopedia2.thefreedictionary.com]
Hypomimia
  • Hypomimia- “masked facies”,expressionless face, blinking Speech abnormalities- Hypophonia: soft voice Aprosody of speech: monotonous and lack of inflection Tachyphemia: do not separate syllables together, running words together Patients may eventually[slideplayer.com]
Tremor
  • Another disorder in which a resting tremor is one of the main symptoms, accompanied by slowness and poverty of movement, muscular rigidity, and postural instability is Parkinson's disease Parkinson's disease or Parkinsonism, degenerative brain disorder[encyclopedia2.thefreedictionary.com]
  • […] syndrome An intention tremor beginning in one extremity, gradually increasing in intensity, and subsequently involving other parts of the body. Synonyms: progressive cerebellar tremor.[mondofacto.com]
  • Typically, the tremor begins on one side of the body, usually in one hand. Tremors can also affect the arms, legs, feet, and face.[icdlist.com]
  • Tremors can also eventually occur in the head, lips, tongue, and feet. In younger patients tremor is usually predominant and often suggests a less aggressive form of the disease.[slideplayer.com]
  • The tremor is coarse and on occasions violent and is always maximal when the limb is at rest. In Case III it affects the head, which nowadays would raise suspicion of an essential or dystonic tremor.[academic.oup.com]
Bradykinesia
  • Slowness of motion (bradykinesia) is one of the classic symptoms of Parkinson's disease.[slideplayer.com]
  • Diseases related with Melanoma and Bradykinesia In the following list you will find some of the most common rare diseases related to Melanoma and Bradykinesia that can help you solving undiagnosed cases.[mendelian.co]
  • The hallmark symptoms of Parkinson's disease (PD) are asymmetric tremors at rest, rigidity, and bradykinesia (slowness in movement).[rosevillepsg.weebly.com]
  • It is characterised by tremor, bradykinesia, dysarthria, rigidity, fixed facies and a tendency to faint. Mental functions are intact and there are no reflex changes.[whonamedit.com]
  • Other characteristic symptoms of Parkinson disease include rigidity or stiffness of the limbs and torso, slow movement (bradykinesia) or an inability to move (akinesia), and impaired balance and coordination (postural instability).[icdlist.com]
Postural Instability
  • Another disorder in which a resting tremor is one of the main symptoms, accompanied by slowness and poverty of movement, muscular rigidity, and postural instability is Parkinson's disease Parkinson's disease or Parkinsonism, degenerative brain disorder[encyclopedia2.thefreedictionary.com]
  • Other characteristic symptoms of Parkinson disease include rigidity or stiffness of the limbs and torso, slow movement (bradykinesia) or an inability to move (akinesia), and impaired balance and coordination (postural instability).[icdlist.com]
  • Some of the most common symptoms of Parkinson's disease are: tremor of the hands, arms, legs, jaw and face rigidity or stiffness of the limbs and trunk bradykinesia or slowness of movement postural instability or impaired balance and coordination *from[rosevillepsg.weebly.com]
  • Postural instability Impaired balance and the tendency to fall without explanation, usually when pivoting; a common symptom in the later stages of Parkinson’s. or impaired balance and coordination People with YOPD may experience the same non-motor symptoms[parkinson.org]
  • A triad of features classically characterizes Parkinson disease: resting tremor rigidity bradykinesia (postural instability: sometimes added as a fourth cardinal feature 3 ) Dementia can be a late feature.[radiopaedia.org]
Resting Tremor
  • Another disorder in which a resting tremor is one of the main symptoms, accompanied by slowness and poverty of movement, muscular rigidity, and postural instability is Parkinson's disease Parkinson's disease or Parkinsonism, degenerative brain disorder[encyclopedia2.thefreedictionary.com]
  • A triad of features classically characterizes Parkinson disease: resting tremor rigidity bradykinesia (postural instability: sometimes added as a fourth cardinal feature 3 ) Dementia can be a late feature.[radiopaedia.org]
  • Major features Resting tremor in hands, arms, legs, jaw, and face Bradykinesia Rigidity- cogwheel or lead-pipe Minor features Bradyphrenia Speech abnormalities Depression Dysautonomia Dystonia Constipation Hallucinations Dysphagia 19 Parkinson’s disease[slideplayer.com]
  • tremor Neoplasm Rare Symptoms - Less than 30% cases Hypogonadism Cognitive impairment Decreased testicular size Cutaneous photosensitivity Falls Cryptorchidism Cataract Sensory neuropathy Short stature Sensorineural hearing impairment Strabismus Pes[mendelian.co]
  • Buzzard endorsed Charcot’s view that although rest tremor was an important component of the shaking palsy it was not specific or invariable and that postural limb tremor and quivering of the lips could also be seen.[academic.oup.com]
Akinesia
  • Associated, reflex, and voluntary movements are absent (akinesia). Muscle tone is plastic, and the extremity can move only passively. Electromyography and other special methods of examination are important for differential diagnosis.[encyclopedia2.thefreedictionary.com]
  • Resting and action tremor, akinesia, stooped posture, distortion of the trunk, dystonia of the upper extremities, oculogyric crisis, and impairment of the postural reflex were seen.[ncbi.nlm.nih.gov]
  • It is clinically characterized by movement disorders such as muscle rigidity, akinesia and tremor.[books.google.de]
  • Other characteristic symptoms of Parkinson disease include rigidity or stiffness of the limbs and torso, slow movement (bradykinesia) or an inability to move (akinesia), and impaired balance and coordination (postural instability).[icdlist.com]
  • Developed as an antiviral agent, it was used widely in nursing home populations, and Schwab noted its unexpected benefit on tremor, balance, and akinesia in both Parkinson's disease and postencephalitic parkinsonian patients ( Schwab et al. 1969 ).[ncbi.nlm.nih.gov]

Treatment

  • The prognosis and treatment are determined by the disease that caused the paralysis. Among the special methods used to restore the functions of the extremity are massage and remedial gymnastics. REFERENCES Krol’, M. B., and E. A. Fedorova.[encyclopedia2.thefreedictionary.com]
  • Therapy and Treatment When it comes to medical treatment, people with YOPD have a significantly greater risk of developing the following: Dyskinesias or involuntary movements (most commonly dystonia) as a side effect of carbidopa A medication used together[parkinson.org]
  • A patient with Juvenile PD developed PG soon after beginning treatment with pergolide, a mixed D1/D2 dopamine agonist that is also supposed to exhibit D3 activity. This behavior remitted upon the discontinuation of the drug.[journals.lww.com]
  • Co-treatment with carbidopa allows for a lower levodopa dose, thereby reducing side effects.[medicinenet.com]
  • Charcot’s British contemporary, WR Gowers, followed similar treatment strategies.[ncbi.nlm.nih.gov]

Prognosis

  • The prognosis and treatment are determined by the disease that caused the paralysis. Among the special methods used to restore the functions of the extremity are massage and remedial gymnastics. REFERENCES Krol’, M. B., and E. A. Fedorova.[encyclopedia2.thefreedictionary.com]
  • Treatment and prognosis The mainstay of treatment is medical. In patients with refractory symptoms, deep brain stimulation may be useful 15.[radiopaedia.org]
  • What is the prognosis and life expectancy for Parkinson's disease? The severity of Parkinson's disease symptoms and signs vary greatly from person to peson, and it is not possible to predict how quickly the disease will progress.[medicinenet.com]
  • Fahn (1991) Dopa-responsive dystonia: long term treatment, response and prognosis. Neurology, 41: 174-181. Ota, Y., M. Sawako, O. Ueda, T. Mukai and A. Maeda (1958) Familial paralysis agitans juvenilis. A clinical anatomical and genetic study. Fol.[kundoc.com]

Etiology

  • Drug induced parkinsonism Hypoxia Tumor Trauma Vascular:Multiinfarct Toxin:Mn, CO, MPTP and cyanide Post-encephalitic parkinsonism (von Economo’s encephalitis) Normal pressure hydrocephalus Wilson’s disease, Hutington’s disease 17 Classification and Etiology[slideplayer.com]
  • Acute retinal necrosis syndrome Necrotizing retinitis with uveitis and other retinal pathology, severe loss of vision, and often retinal detachment; of viral etiology.[scribd.com]
  • The etiology and linkage between these conditions are still controversial and unclear.[kundoc.com]
  • It may be argued that he neither discovered its etiology nor found a cure; but the disease has stood an open challenge to investigators for a full century.[searching4james.wordpress.com]

Epidemiology

  • Hereditary juvenile epithelial dystrophy of cornea meesmann corneal dystrophy 遺傳性青少年性角膜上皮營養不良 Analysis of phase - change related endogenous hormone changes in juvenile and mature of phellodendron amurense 黃波羅不同年齡材料微繁苗內源激素的比較分析 Epidemiological analysis[dict.site]
  • Current epidemiological and biochemical evidence leads to the hypothesis that the brothers have impaired ability to synthesize dopamine, possibly because of an inherited deficiency of tyrosine hydroxylase enzyme.[jamanetwork.com]
  • Clinical, pharmacological, evolutive, and epidemiological data were collected on all patients.[onlinelibrary.wiley.com]
  • Introduction Parkinson’s disease is a chronic neurodegenerative movement disorder affecting voluntary and emotional movements and most commonly seen in the elderly, but is also found in the young and inexorably progresses leading to significant disability. 5 Epidemiology[slideplayer.com]
Sex distribution
Age distribution

Pathophysiology

  • 9 GLOBUS, PARS RETICULATA PATHOPHYSIOLOGY DA SUBSTANTIA NIGRA GLOBUS, PARS RETICULATA ACH GABA STRIATUM CAUDATE, PUTAMEN 10 Chemical Balance in Corpus Striatum Excitatory Cholinergic pathway Inhibitory Dopaminergic pathway BALANCE 11 Chemical Balance[slideplayer.com]

Prevention

  • Genetic factors may contribute to the pathogenesis of Parkinson's disease (PD) by several mechanisms, such as preventing normal development of dopaminergic neurons, delayed programming or accelerated cell death, interfering with normal protective mechanisms[neurologyindia.com]
  • Therefore, parkin loss of function, as happens in autosomal recessive juvenile Parkinson (AR-JP), prevents degradation of parkin substrates (i.e. GPR37), thus resulting in their toxic accumulation.[ub.edu]
  • Preventing neurodegeneration in the Drosophila mutant bubblegum . Science 284 , 1985–1988 (1999). 52. Van Geel, B. M. et al.[nature.com]
  • Concussion Care Excela Health's Concussion Clinic has a double-sided objective of providing education to prevent concussion and using the latest techniques to treat and evaluate recovery.[excelahealth.org]

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