Lesson of the month
Hypertrophy of multiple cranial nerves and spinal roots in chronic inflammatory demyelinating neuropathy
J Duartea, A Cruz Martinezb, F Rodrigueza, A Mendozaa, A P Semperea, L E Claveriaa
a Neurology Unit,
General Hospital, Segovia, Spain, b Electromyography Unit, Hospital La Luz, Madrid,
Spain
Correspondence to: Dr L E Claveria, Neurology Unit, General Hospital, Ctera de Avila s/n, Segovia 40001, Spain. Fax 00-34-921-419149.
Received 12 October
1998 and in revised form 4 February 1999;
Accepted 10
February 1999
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Introduction |
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Hereditary motor and sensory neuropathy (HMSN) types I and III and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in developed countries are the most frequent causes of enlargement of peripheral nerves, "hypertrophic neuropathy",1 with clinically palpable thickened nerves,2 visible on MRI.3-6 They may even be the cause of spinal cord compression.7 8
We report on a patient who developed clinical features that were interpreted elsewhere initially as a Guillain-Barré syndrome, and years later diagnosed as Dejerine-Sottas disease (HMSN III). We discuss the differential diagnosis between HMSN III and CIDP in this patient with a fluctuating but insidiously progressive 26 year history, in whom MRI demonstrated marked enlargement of multiple cranial nerves and spinal roots. We think that this is a dramatic example of cranial nerve and spinal root enlargement in a case of CIDP, and stress the importance of making a correct differential diagnosis when the disease presents in early childhood.
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Case report |
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A 30 year old
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[Abstract] [Full Text] -
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