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SYNDROMES OF THE MOTOR

Disorders Of the Neuron Of the Motor: Diagnosis Differentiated

• Asymmetric
  • Stop lower + Muestras Of the Neuron Of the Motor
    • Sclerosis De Lateral De Amyotrophic (ALS)
      • Sporadic ALS
      • Hereditary ALS
        • Dismutase De Superoxide; Chromosome 21; Dominant
        • The Other ALS: Recesivo And Dominante
    • Sporadic disorders of the neuron of the motor with dementia
      • Western Pacific ALS
      • The frontal dementia followed by disease of the system of the motor
        • Superior neuron of the motor: Specially to bulbar
        • Lower the neuron of the motor: Fasciculaciones; Smooth weakness
        • Abnormal syndromes of Creutzfeld-Jacob
    • Disorders of the multi-system
      • Extrapiramidales ALS with Ophthalmoplegia and disorders
      • Disease of the body of Polyglucosan
      • Motor neuronopathy with the skeletal cataracts and abnormalitys
      • Multiple Atrophy of the system
      • Syndrome of the AAA
  • Only lower the samples of the neuron of the motor (asymmetric)
    • Syndrome lower Distal Of the Neuron Of Motor (LMN)
      • IgM against ganglioside GM1
        
      • IgM against ganglioside of GalNAc-GD1a
        
      • Also it sees: Multifocal Neuropatía of the motor
    • Next lower syndromes Of the Neuron Of the Motor
      • Brachial Diplegia amyotrophic
      • Associated to IgM against asialo-GM1
      • Rare: Stop > lower members with the antibodies of anti-Hu
    • Lower the syndrome of the neuron of the motor without the antibodies (PMA)
    • Variants of ALS
      • Hereditary
      • Sporadic
    • Focal disease of the neuron of the motor
      • Monomelic Amyotrophy
      • Muscle of Paraspinous amyotrophy
      • Cervical Amyotrophy
    • Motor of Paraneoplastic neuro(no)pathy
      • Smooth weakness: With linfoma
      • Severe weakness: With the chest cancer
    • Syndrome of Hopkins: Acute post-asthmatic Amyotrophy
    • Syndrome of the poliomielitis and Poste-poliomielitis
    • SMN2 (canceladurasof SMN C)
    • Neurofibromatosis, Type 2 ⁵
• Symmetrical And Next : Hereditary Spinal Muscular Atrophy
  • SMN: Chromosome 5q; Recesivo
  • Receiver Of The Androgen (Bulb-Spinal Muscular Atrophy): X-bound; Recesivo
  • Hexosaminidase A (Tay-Sachs): Chromosome 15; Recesivo
• Weakness of the hand
  • Neuropathies of the disease and the motor of the neuron of the motor
  • Myopathies: Distal; Dystrophy De Myotonic; Myositis body Of the Inclusion
  • Myasthenia Gravis
  • Peripheral injury of the nerve
    • Midpoint: Recurrent branch of the motor; Previous Interosseus
    • Cubital: Channel of Guyon
    • Radial part: Later Interosseus
  • Brachial Plexopathy
• Implication to bulbar
  • Bulb-Spinal Muscular Atrophy
    • Receiver Of The Androgen: X-bound
    • Dominant
  • Sclerosis De Lateral De Amyotrophic (ALS)
    • Sporadic ALS
    • Hereditary ALS
    • ALS with beginning to bulbar, benign course and the bodies of Bunina
  • Syndromes to bulbar hereditary
    • Laere Brown-Vialetto-Van
    • Fazio-Londe
  • Syndrome to bulbar congenital
    • Syndrome of Worster-Sequi'a: Paralysis to suprabulbar congenital
• Painful : Diabetic Amyotrophy
• Fast Beginning
  • Acute Neuropatía Of The Motor De Axonal
    (with jejuni or the IgG serum of campylobacter against GM1)
  • Poliomielitis
  • Porphyria
    • 4 types neurological attacks of the cause
      
      • Intermittent acute
      • Vary Porphyria
      • Coproporphyria
      • δ- acid deficiency amino-amino-levulinic of dehydratase
    • It tinkles: All the types produce increased δ- amino-amino-levulinic acid during attacks
• Acquired
  • Toxic: Lead; Dapsone; Botulism; Paralysis Of the Signal
  • Infections
    • Poliomielitis
    • The Nile Of the west
    • Central European Encefalitis
      
    • Creutzfeld-Jacob
      • Amyotrophy
      • Polyneuropathy (± Demyelinating)
• Another one
  • SMA and syndromes of the neuron of the motor: Another hereditary one
  • Neuropatía Multifocal Of Motor (MMN)

Neuropatía Of The Motor De Paraneoplastic

• Beginning: After the diagnosis of the tumor
• Epidemiología: Man of the majority and > 50 years
• Clinical
  • Weakness
    • Asymmetric; Arms > Legs
    • Smooth
    • Only lower the neuron of the motor
    • Normal Bulbar
  • Cramps: Painful
  • Without pain in some patients
  • Course: The progressive later becomes stabilized or improves
• Associated a
  • Linfoma De Non-Hodgkin
  • Also others linfomas and disorders myeloproliferative ⁴
• Laboratory
  • Csf: No cells; Smoothly increasing protein
  • MRI: Normal spinal marrow
  • Neuronopathy
• Model animal: Similar disorder in virus leukemic murine of the mice 2°
• Also it sees: A Syndrome More under the Neuron Of the Motor De Paraneoplastic

A lower Syndrome ⁸ Of the Neuron Of the Motor De Paraneoplastic

• Epidemiología: Single patient, old female of 72 years
• Beginning: 4 months before the diagnosis of the tumor
• Clinical
  • Weakness
    • Asymmetric in the beginning
    • Arms And Legs
    • Severe
    • Only lower the neuron of the motor
    • Bulbar: Hypophonia; Disfagia; Unilateral face Paresis
  • Without pain
  • Course
    • Excessive months of the progressive
    • Improvement after the retirement of the tumor
    • Residual incapacity in the long term
  • Sensation: Normal
  • Reflections of the sinew: Absent
  • Cns: Transitory vertigos and nistagma
• Associated a
  • Adenocarcinoma de Ductal of the chest
• Laboratory
  • Antibodies
    • Serum that ties βto spectrin of the intravenous one, isoform I (Wendish in MW 250kD and 140kD)
    • Serm that ties to the segments of the initial of axon and to the nodes of Ranvier in brain of the rat
  • Electrofisiología
    • EMG: Denervation
    • NCV: Small CMAPs; No block of the conduction
  • MRI: Spinal marrow with the high points of the signal in t2
  • Neuronopathy
• Inmunosupresivo treatment: No answer
• Also it sees: Neuropatía Of The Motor De Paraneoplastic

HOPKINS SYNDROME: Acute post-asthmatic Amyotrophy

• Age: 1 to 13 years
• Beginning
  • After acute asthmatic attack: Latency 1 to 18 days
  • Smooth pain: Member, neck or meningismus
  • Fast weakness of the beginning
• Weakness
  • Single member; Asymmetric; > can be next distal
  • Severity: Smooth to severe
  • Arm or leg
• Sensorial: Normal
• Csf
  • Pleocytosis
  • Protein: ± Increased
• MRI: Signal of the demonstration of May (t2) in spinal marrow
• Prognosis: Permanent paralysis

Syndrome Of the Poliomielitis Of the Post

• Criteria Of diagnosis: Clinical
  • History of the poliomielitis
    • Neurological recovery and functional partisan or completes
      
    • Stable function > 15 years
  • Beginning of
    • Fatigue
    • Pain the muscle
    • Functional loss
      • Generally disorder of 2° Musculoskeletal
      • The new weakness is rare
  • Neurological Examinación
    • Lower the syndrome of the neuron of the motor (confirmed by EMG or MRI)
      • The mensurable loss of force is rare
    • Reflections diminished or absent of the sinew
    • No sensorial loss
  • Another one
    • No other explanation for the symptoms
    • The common pain and the cold intolerancia can accompany the syndrome
• Characteristics of the laboratory
  • CK: Elevated
  • Electrofisiología
    • Very great units of the motor (up to 10 normal times)
    • Some fibrilaciones and positive acute waves even in stable patients
  • Pathology of the muscle
    • Near the complete recovery of the force and the stable
      • Type the predominance of the fiber of the muscle of I
    • Persistent or new weakness
      • type fibers of 50% of I
      • Angular fibers; Atrophy + hipertrofia
    • New high weakness and CK
      • Smaller angular fibers
      • Necrosis of the fiber of the muscle
• Recommended exercise
  • Smooth Paresis: Training of the resistance
    
  • Moderate Paresis: SubMaxima resistance
    
  • Severe Paresis: None; Common supporting

Monomelic Amyotrophy

• Eponyms
  • Disease of Hirayama: Progressive weakness on 1 to 4 years, then plateau
  • Syndrome of ÓSullivan-McLeod: Slow progression
• Beginning: Young adult; 15 to 25 years; Up to 40 years in India
• Epidemiología
  • Man > Female: Until 10:1
  • Familiar antecedents
    • Generally sporadic
    • Occasional familial occurrence
  • Field common in the India of east ⁷
• Weakness
  • Prisoner often to a single arm
  • Distal implication (97%): C7, C8 and the T1 innervated the muscles; Hand And Forearm
  • Next > Distal: 10%
  • Side: The Right = Gone
• Atrophy: "amyotrophy oblique"; To save brachioradialis
• Tremor (80%): In the extension of the finger; Irregular And Heavy (Minipolymyoclonus)
• Occasional other characteristics
  • Weakness
    • Shoulder of Ipsilateral
    • Progression to the opposite member (18%): A weakness generally smoother than 1r member
    • Worsening in contact with cold
  • Fasciculaciones: In the affected side (47% to 66%); It cannot be symptomatic
  • Cramps (16%)
  • Sensorial loss: Smooth
  • Hiperhidrosis
• Reflections of the sinew: Generally normal
• No craneal nerve, pyramidal leg or changes
• Course of the disease
  • Typical syndrome ¹⁰ of Hirayama
    • Progression: On 1 to 5 years; Once in a while while 8 years
    • Atmospheric disturbances after the phase of the progression: It can persist per decades
    • Incapacity: Smooth or none in 73%
  • Syndrome ⁶ of ÓSullivan-McLeod : Excessive decades of the progression
• Laboratory
  • EMG: Chronic Denervation
    • In limb(s) affected (100%)
    • Opposite arm or lower extremities in some patients
    • Samples of denervation acute in 45%
  • NCV: Small CMAPs in affected members
  • Comprehensive answer of the skin: It can be abnormal
  • MRI
    • Some patients with hard inelástico: Compression of the spinal marrow with flexion ¹ of the neck
    • Another one studies ³
      • No important spinal anomalies
      • Atrophy of the spinal marrow: C6 and C7
      • Signal of t2 in previous horns of the gray matter
      • Flexion-induced breakup smooth of the cord

Amyotrophy: Benign lower extremity ¹¹

• Epidemiología
  • 16 described cases
  • Man > Female: 5:1
• Beginning: Age mediates 41 years
• Clinical
  • Weakness: Later legs
    • Generally: Later yearling calf; Peronei; Sinews of the arched one
    • Rare: Quadrangles
    • Often bilateral (50%)
      • Clinical or subclinical
      • Asymmetry: Some
  • No sensorial, to bulbar or superior samples of the neuron of the motor
  • Course
    • Insidioso beginning
    • Slow progression: Few years
    • Stabilization
• Laboratory
  • MRI
    • Loss of muscle with the fat replacement
    • Distribution: Lower the extremities
      • Distal leg: Later compartment
      • Thigh: Long head of the biceps
  • EMG: Denervation
  • Biopsy of the muscle: Denervation; Grouped Atrophy

"Primary Muscular Atrophy" (PMA)

• Extensive lower syndrome Of the Neuron Of the Motor
  • Weakness: Distribution
    • Distal And Pro'ximo: Anyone can be more prominent
    • Asymmetric
    • Paraspinous often implies respiratory muscles and
    • It often saves the musculatura to bulbar
  • Spontaneous activity of motor
    • Cramps: Field common in legs, at night
    • Fasciculaciones
  • No samples of the neuron of the motor of the stop
  • Pain: Related to the inmovilidad
  • Course of the time
    • Progressive
    • Similar to, faster, or slower than, typical ALS
• Laboratory
  • Pathology of the muscle: Atrophy > type grouped grouping of the fiber
  • No antibodies of the serum
  • No block of the conduction
• No evidence for the answer to the treatment
• Differentiated Diagnosis
  • Next lower syndrome of the neuron of the motor
  • ALS
    • Sporadic
    • Hereditary
      • Mutations of the TURF: A4V, Leu84Val, D101N
• Pathology
  • Loss of neurons of the motor in previous horn of the spinal marrow
  • Contraction of the remaining neurons of the motor
  • Bodies of the inclusion: Intracitoplásmico, Hyaline

Primary Lateral Sclerosis ²

• Discreet syndrome against ALS variant
• Clinical
  • Beginning
    • Slowly progressive
    • Legs before arms
    • The more commonly possible in 5ta decade
    • No familiar antecedents
  • Disfunción of the motor
    • Disfunción ± to corticobulbar of the zone of Corticospinal
      • Spasticity: Legs > Arms
      • Reflections of the sinew: Energetic
      • Reflection of Planting: Normal or upgoing
      • Samples of Pseudobulbar: Specially with beginning of the disease > 45 years
    • Symmetrical
    • No change more under the neuron of the motor
  • Sensorial: Normal
  • Disfunción of lóobulo frontal: Smooth
  • Progression
    • Gradual
    • Slow
    • > 3 years; Up to 3 decades
  • Function of vejiga: Normal until late inside disease
• Laboratory
  • Magnetic stimulus: The cortical absentee or prolonged motor evoked latencies
  • MRI: Focal Atrophy of the convolución of the precentral brain
  • Exploration of the DOMESTIC ANIMAL: Reduced Consumición of the glucose in the pericentral region
  • Central times of the conduction of the motor: Prolonged
  • Normal: Serum; Csf; EMG; Projection of image of the spinal marrow; Serum CK; Protein of the CSF
• Association of the disease: Cnacer of the chest
• Pathology
  • Zone of Corticospinal: Loss of Axonal
  • Normal: Previous cells of Betz ± of the cells of the horn
• Differentiated Diagnosis
  • Structural disorders: Spinal; Double bottle of the hole; Hidrocefalia
  • Hereditary spinal disorders
  • Infections

• Mutations of gene
  • PLS: Canceladura in exon 9
  • ALS2: Mutation in another region of gene of alsin
  • Espástico Paraparesis of Familial, infantile beginning
• Clinical
  • Beginning: Childhood
  • Spasticity: Bulbar; Extremities
  • Watch the paresis
  • Normal: Cognition; Sensation
• Laboratory
  • Central times of the conduction of the motor: Retrasado or Unrecordable
  • EMG: None denervation