Thursday, January 14, 2010

Stiff man syndrome (stiff person syndrome) Pearls

1. Insidious onset, then robotic appearance to walking and exaggerated lumbar lordosis in middle aged. Attempted passive movement causes a unique rock like immobility.

2. Trismus does not occur, and eye movements are not affected. Noise may precipitate an attack. Reflexes are normal, and lumbar spine is stiff on exam.

3. "Stiff limb" in one leg, spreading to other, has same antibodies (anti GAD) is similar to lcoalized tetanus.

4. EMG is normal and spasms disappear during sleep and anesthesia, differentiating from myokymia and Isaac's syndrome.

5. Rare paraneoplastic form usually accompanies breast cancer with antibodies to amphiphysin or gephyrin (proteins related to synaptic GABA receptors) and may be accompanied by opsoclonus or encephalopathy.

6. Differential diagnosis includes tetanus, Isaac's syndrome and myoclonic spinal neuronitis.

7. An infantile form starts at age 2 months and disappears by age 2.

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