Disease Information for Motor neuron disease

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Clinical Manifestations
Signs & Symptoms
Akinetic rigid frozen Posture
Dysphagia Swallowing Difficulties
Tongue fasciculations
Tongue muscle atrophy
Tongue muscle/hemiatrophy
Focal wasting muscle groups
Hypotonia
Interosseous muscle wasting/hand
Intrinsic hand muscle wasting/sign
Loss of muscle mass
Muscle Atrophy
Muscle fasciculations
Muscle stiffness/rigidity
Muscle tone/decreased
Muscle twitches
Muscle weakness
Thenar muscle wasting
Twitchy/muscle spasms
Weak/Weak as a kitten (symptom)
Weakness, pharyngeal muscles/signs
Abdominal reflex decreased
Absent knee reflexes
Ankle reflexes absent
Arm reflexes, bilateral decrease
Cremasteric reflex decreased
Flaccid arm/shoulder paralysis
Flaccid muscle paralysis
Grip strength decreased
Hyporeflexia/DTRs decreased
Intelligence normal
Jaw jerk decreased
Limb weakness/signs
Lower motor neuron lesion/signs
Paralysis tongue/Aphasia/Aphonia
Signs shoulder girdle/LM neuron disease
Spares sensory modalities/exam
Weakness/Diffuse motor loss
Voice Alteration
Voice Alteration in Elderly
Voice change/disturbance/Unusual
Enervated/extreme acute fatigue
Muscle Wasting/Diffuse
Weakness
Weakness non-neuromuscular/systemic
Weakness, Gradual Onset
Demographics & Risk Factors
Past History
Past history/Trophic ulcer
Diagnostic Test Results
Pathology
BX/Muscle Biopsy Abnormal
Electrodiagnosis
EMG/Abnormal findings
EMG/Denervation potentials
Associated Diseases & Rule outs
Rule Outs
Amyotrophic lateral sclerosis
Dermatomyositis
Myotonia atrophica
Associated Disease & Complications
Motor neuron disease
Myelopathy manifestations/involvement
Progressive Bulbar palsy
Pseudobulbar palsy syndrome
Akinetic Rigid Syndrome
Disease Mechanism & Classification
Class
CLASS/Motor neuron/unit disorder (ex)
CLASS/Anterior horn cell disease (ex)
CLASS/Neurologic (category)
Process
PROCESS/Atrophic disorders (ex)
PROCESS/Reference organ/system (category)
Treatment
Drug Therapy - Indication
RX/Lithium (Lithobid)
RX/Myotropin injection
RX/Riluzole (Rilutek)
Definition

Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex; Clinical subtypes are distinguished by the major site of degeneration; In AMYOTROPHIC LATERAL SCLEROSIS there is involvment of upper, lower, and brainstem motor neurons; In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected; With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem; In primary lateral sclerosis, the cortical neurons are affected in isolation; (Adams et al, Principles of Neurology, 6th ed, p1089)---------------------------------------.

Motor Neuron Disease; Motoneuron Disease; Motoneurone Disease; Motor Neuron Syndrome; Motor Neurone Disease; ALS Included; Amyotrophic Lateral Sclerosis; Benign Focal Amyotrophy others: Duchenne"s Paralysis; Infantile Spinal Muscular Atrophy (all types); Juvenile Spinal Muscular Atrophy; Kugelberg-Welander Syndrome; Lou Gehrig"s Disease, Included; Progressive Bulbar Palsy; Spinal Muscular Atrophy, All Types; Werdnig-Hoffmann Disease;Motor Neuron Disease comprises a group of severe disorders of the nervous system characterized by progressive degeneration of motor neurons (neurons are the basic nerve cells that combine to form nerves); Motor neurons control the behavior of muscles; Motor Neuron Diseases may affect the upper motor neurons, nerves that lead from the brain to the medulla (a part of the brain stem) or to the spinal cord, or the lower motor neurons, nerves that lead from the spinal cord to the muscles of the body, or both; Spasms and exaggerated reflexes indicate damage to the upper motor neurons; A progressive wasting (atrophy) and weakness of muscles that have lost their nerve supply indicate damage to the lower motor neurons; [NORD]

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Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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