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Case Reports
. 2020 Apr 23;8(7):1223-1225.
doi: 10.1002/ccr3.2899. eCollection 2020 Jul.

Bulbospinal muscular atrophy (Kennedy disease) responsive to immunoglobulins?

Katharina Poustka  1 , Sabine Pollanz-Petrovic  1 , Elisabeth Lindeck-Pozza  1 , Josef Finsterer  2
Affiliations
Case Reports

Bulbospinal muscular atrophy (Kennedy disease) responsive to immunoglobulins?

Katharina Poustka et al. Clin Case Rep. .

Abstract

A 61 year old man with facial diplegia, quadruparesis, tongue atrophy/fasciculations, bulbar speech, muscle weakness/wasting, hypotonia, tremor, dysdiadochokinesia, absent tendon reflexes, fasciculations, and gynecomastia, received immunoglobulins for suspected immune-neuropathy with limited benefit. After reconsideration, Kennedy disease was diagnosed upon 44 CAG repeats in AR. In conclusion, immunoglobulins exhibit limited benefit on immune-neuropathy in patients with coexisting KD.

Keywords: Kennedy disease; immunoglobulins; motor neuron disease; phenotype; polyglutamine disease; polyneuropathy.

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Conflict of interest statement

There are no conflicts of interest.

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