Multiple Systems Atrophy (Shy-Drager) Autonomic Differential Diagnosis

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Multiple Systems Atrophy (Shy-Drager) <A

HREF= " http://www.neuro.wustl.edu/neuromuscular/autonomic.html#sd " >Autonomic

Differential Diagnosis</A>

oSporadic or Autosomal Dominant

o Nosolgoy: other similar or overlapping disorders

o Striatonigral Degeneration

o Adult-onset Sporadic Olivopontocerebellar Atrophy (opca)

o Shy Drager Syndrome

o Clinical features

o Onset: Adult, Early Parkinsonism & Bladder Dysfunction

o Autonomic

. Orthostatic Hypotension

. Bladder Incontinence

. Anhydrosis, Ptosis

. Bowel Dysfunction

o Extrapyramidal

. Parkinsonism (87%): Especially Rigidity, Postural

instability &

Bradykinesia

. Dystonic response to L-Dopa

o Upper motor neuron signs (50%) Spasticity, Brisk tendon

reflexes

o Bulbar

. Scanning speech

. Stridor: Laryngeal abductor paralysis (also see

Recessive ataxia )

o Cerebellar disorders (Especially OPCA variant): 50%

o Myoclonus: Stimulus-sensitive;Limbs & Face

o Peripheral nervous system

. Lower motor neuron signs (20 %)

. Polyneuropathy: Sensory-Motor (20%)

o Skin: Dusky discoloration of the extremities

o Course

. Variably progressive

. Median survival: 6 to 10 years

o Laboratory

o Bladder dysfunction ( 90%)

o EMG: Denervation (20%)

o Pathology

o Neuronal loss & Gliosis

. Extrapyramidal: Putamen;Substantia nigra;Locus

ceruleus

. Other brainstem: Inferior olive; Pontine nuclei

. Purkinje cells

. Spinal cord: Intermediolateral cell column

(Thoracic); Onuf's nucleus

o Glial cytoplasmic inclusions (Argyrophilic) :

Suprasegmental motor systems, Supraspinal autonomic system

o Autonomic pathology

. Spinal cord: Reduced sympathetic preganglinonic

neurons in intermediolateral cell column

. Medulla

o Reduced Catecholaminergic neurons (A

1/C1) in verntrolateral intermediate reticular formation

o Normal projections

. Descending: Intermediolateral

cell column in spinal cord

. Ascending: Vasopressin neurons

in hypothalamus

External link: eNeurology

[Guest guest]

Multiple Systems Atrophy (Shy-Drager) <A

HREF= " http://www.neuro.wustl.edu/neuromuscular/autonomic.html#sd " >Autonomic

Differential Diagnosis</A>

oSporadic or Autosomal Dominant

o Nosolgoy: other similar or overlapping disorders

o Striatonigral Degeneration

o Adult-onset Sporadic Olivopontocerebellar Atrophy (opca)

o Shy Drager Syndrome

o Clinical features

o Onset: Adult, Early Parkinsonism & Bladder Dysfunction

o Autonomic

. Orthostatic Hypotension

. Bladder Incontinence

. Anhydrosis, Ptosis

. Bowel Dysfunction

o Extrapyramidal

. Parkinsonism (87%): Especially Rigidity, Postural

instability &

Bradykinesia

. Dystonic response to L-Dopa

o Upper motor neuron signs (50%) Spasticity, Brisk tendon

reflexes

o Bulbar

. Scanning speech

. Stridor: Laryngeal abductor paralysis (also see

Recessive ataxia )

o Cerebellar disorders (Especially OPCA variant): 50%

o Myoclonus: Stimulus-sensitive;Limbs & Face

o Peripheral nervous system

. Lower motor neuron signs (20 %)

. Polyneuropathy: Sensory-Motor (20%)

o Skin: Dusky discoloration of the extremities

o Course

. Variably progressive

. Median survival: 6 to 10 years

o Laboratory

o Bladder dysfunction ( 90%)

o EMG: Denervation (20%)

o Pathology

o Neuronal loss & Gliosis

. Extrapyramidal: Putamen;Substantia nigra;Locus

ceruleus

. Other brainstem: Inferior olive; Pontine nuclei

. Purkinje cells

. Spinal cord: Intermediolateral cell column

(Thoracic); Onuf's nucleus

o Glial cytoplasmic inclusions (Argyrophilic) :

Suprasegmental motor systems, Supraspinal autonomic system

o Autonomic pathology

. Spinal cord: Reduced sympathetic preganglinonic

neurons in intermediolateral cell column

. Medulla

o Reduced Catecholaminergic neurons (A

1/C1) in verntrolateral intermediate reticular formation

o Normal projections

. Descending: Intermediolateral

cell column in spinal cord

. Ascending: Vasopressin neurons

in hypothalamus

External link: eNeurology