Fasciculation cramp syndrome preceding anterior horn cell
disease:an intermediate syndrome?
, who examined serial sections of anterior horn cells
using electron microscopy.
* Progressive muscle weakness, most severely affecting the legs and leading to paralysis, and identical to the pattern of weakness seen in children with spinal muscular atrophy (anterior horn cell
His neurological examination revealed wasting and weakness of distal muscles, left foot drop suggestive of anterior horn cell
These patients had asymmetric, acute weakness, pleocytosis, and in 14 patients with studies performed, results of electromyography/nerve conduction studies were consistent with motor axonopathy, anterior horn cell
involvement, or both.
In seven cases, the evaluation suggested involvement of spinal cord gray matter--specifically anterior horn cells
. All seven patients exhibited polio features.
Electrodiagnostic studies of all patients displayed markedly reduced compound motor axon potentials with preserved sensory nerve action potentials; the results were interpreted as consistent with a process primarily localized to the motor axons or, far more likely, given the clinical scenario and pathologic data from WNV-infected animals, the anterior horn cells
of the spinal cord.
Thus, in the context of WNV infection, electrodiagnostic studies previously interpreted as motor axonal polyneuropathy or motor axonopathy without sensory nerve involvement (4-6) are more suggestive of anterior horn cell
loss than of Guillain-Barre syndrome.
It's critical to use appropriate diagnostic testing--including cerebrospinal fluid evaluation, electrodiagnostics (indicative of anterior horn cell
damage or poliomyelitis), and neuroimaging--to avoid inappropriate treatments such as heparin or intravenous immunoglobulin, Dr.
It's critical to use appropriate diagnostic testing--including CSF evaluation, electrodiagnostics (indicative of anterior horn cell
damage or poliomyelitis), and neuroimaging--to avoid inappropriate treatments such as heparin or intravenous immunoglobulin, he advised.