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Pure sensory chronic inflammatory polyneuropathy: rapid deterioration after steroid treatment.

Chroni E, Veltsista D, Gavanozi E, Vlachou T, Polychronopoulos P, Papathanasopoulos P - BMC Neurol (2015)

Bottom Line: Steroids were then substituted by rituximab in the first patient and intravenous immunoglobulin in the second patient resulting in gradual decrement of symptoms and signs.Two-year follow-up showed no further deterioration.Caution should be exercised when treating cases of pure sensory polyneuropathy with high dose steroids since an unfavorable outcome is possible.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, School of Medicine, University of Patras, Patras, Greece. echroni@yahoo.com.

ABSTRACT

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) as a pure sensory variant is rarely encountered. Therefore the best treatment option is hard to define.

Case presentations: We reported two middle-aged patients of Caucasian origin, one female and one male, who over a period of several months presented limbs and gait ataxia. Clinical and neurophysiological examination revealed only sensory abnormalities. A diagnosis of atypical CIDP was suggested, considering the elevated CSF protein level and the presence of anti-gangliosides antibodies. Ten and 15 days respectively after initiation of prednisolone treatment both patients experienced exacerbation of sensory symptoms and emerging of muscle weakness. Steroids were then substituted by rituximab in the first patient and intravenous immunoglobulin in the second patient resulting in gradual decrement of symptoms and signs. Two-year follow-up showed no further deterioration.

Conclusion: Caution should be exercised when treating cases of pure sensory polyneuropathy with high dose steroids since an unfavorable outcome is possible.

No MeSH data available.


Related in: MedlinePlus

Clinical assessment scores of a 55 year old man are plotted against treatment regime at different time-points.
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Fig2: Clinical assessment scores of a 55 year old man are plotted against treatment regime at different time-points.

Mentions: The second patient was started on oral prednisolone (60 mg daily) but from day 15 he experienced exacerbation of ataxia and spreading of the numbness. Steroid tapering was scheduled 20 days from treatment onset since the patient developed diplopia, numbness in the face, inability to perform fine hand’s movements, stand or walk 1 meter (expanded MRC score 64; ONLS arm grade 4, leg grade score 5). Within the next month the patient’s condition improved significantly; his cranial nerve deficits and muscle weakness disappeared and he was able to walk again with support for a few meters. Monthly infusions of IVIg at an initial dose of 0.4 g/kg daily for 5 days and maintenance dose of 0.5 g/kg for 2 days) had a beneficial but short-lived effect on this patient, requiring monthly treatment repetitions (Figure 2). Over the next two years the patient’s condition was further improved by IVIg infusions every 4–6 months and he was able to return to his work.Figure 2


Pure sensory chronic inflammatory polyneuropathy: rapid deterioration after steroid treatment.

Chroni E, Veltsista D, Gavanozi E, Vlachou T, Polychronopoulos P, Papathanasopoulos P - BMC Neurol (2015)

Clinical assessment scores of a 55 year old man are plotted against treatment regime at different time-points.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4359520&req=5

Fig2: Clinical assessment scores of a 55 year old man are plotted against treatment regime at different time-points.
Mentions: The second patient was started on oral prednisolone (60 mg daily) but from day 15 he experienced exacerbation of ataxia and spreading of the numbness. Steroid tapering was scheduled 20 days from treatment onset since the patient developed diplopia, numbness in the face, inability to perform fine hand’s movements, stand or walk 1 meter (expanded MRC score 64; ONLS arm grade 4, leg grade score 5). Within the next month the patient’s condition improved significantly; his cranial nerve deficits and muscle weakness disappeared and he was able to walk again with support for a few meters. Monthly infusions of IVIg at an initial dose of 0.4 g/kg daily for 5 days and maintenance dose of 0.5 g/kg for 2 days) had a beneficial but short-lived effect on this patient, requiring monthly treatment repetitions (Figure 2). Over the next two years the patient’s condition was further improved by IVIg infusions every 4–6 months and he was able to return to his work.Figure 2

Bottom Line: Steroids were then substituted by rituximab in the first patient and intravenous immunoglobulin in the second patient resulting in gradual decrement of symptoms and signs.Two-year follow-up showed no further deterioration.Caution should be exercised when treating cases of pure sensory polyneuropathy with high dose steroids since an unfavorable outcome is possible.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, School of Medicine, University of Patras, Patras, Greece. echroni@yahoo.com.

ABSTRACT

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) as a pure sensory variant is rarely encountered. Therefore the best treatment option is hard to define.

Case presentations: We reported two middle-aged patients of Caucasian origin, one female and one male, who over a period of several months presented limbs and gait ataxia. Clinical and neurophysiological examination revealed only sensory abnormalities. A diagnosis of atypical CIDP was suggested, considering the elevated CSF protein level and the presence of anti-gangliosides antibodies. Ten and 15 days respectively after initiation of prednisolone treatment both patients experienced exacerbation of sensory symptoms and emerging of muscle weakness. Steroids were then substituted by rituximab in the first patient and intravenous immunoglobulin in the second patient resulting in gradual decrement of symptoms and signs. Two-year follow-up showed no further deterioration.

Conclusion: Caution should be exercised when treating cases of pure sensory polyneuropathy with high dose steroids since an unfavorable outcome is possible.

No MeSH data available.


Related in: MedlinePlus