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Seizure control in a patient with Dravet syndrome and cystic fibrosis.

Schmalbach B, Moeller B, von Spiczak S, Muhle H, Stephani U, Lang N - Epilepsy Behav Case Rep (2013)

Bottom Line: Some success can be achieved with bromides, but cognitive side effects and disturbed vigilance may limit their use.Here, we present the case of a successfully treated patient with DS and remarkable features in the course of his disease: additionally to DS, the patient was diagnosed with cystic fibrosis (CF), another genetic channelopathy.Seizure freedom could be achieved under treatment with potassium bromide at the age of 15, but at the age of 20, adverse events made it necessary to stop bromide treatment.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Christian-Albrechts-University of Kiel, Arnold-Heller-Str. 3, Haus 41, 24105 Kiel, Germany.

ABSTRACT
Satisfactory treatment of patients with Dravet syndrome (DS) is often difficult. Some success can be achieved with bromides, but cognitive side effects and disturbed vigilance may limit their use. Here, we present the case of a successfully treated patient with DS and remarkable features in the course of his disease: additionally to DS, the patient was diagnosed with cystic fibrosis (CF), another genetic channelopathy. Seizure freedom could be achieved under treatment with potassium bromide at the age of 15, but at the age of 20, adverse events made it necessary to stop bromide treatment. After conversion to valproic acid, the patient remained seizure-free, and neuropsychological tests demonstrated sustained improvement of cognition.

No MeSH data available.


Related in: MedlinePlus

Comparison of neuropsychological assessment during (2009) and after (2011) therapy with potassium bromide. The figure shows z-values (m = 0, SD = 1), i.e., − 1 < x < 1; 1 is average, < − 1 is below average, and < − 2 is far below average. *Difference > 1 SD, **Difference > critical difference.
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f0010: Comparison of neuropsychological assessment during (2009) and after (2011) therapy with potassium bromide. The figure shows z-values (m = 0, SD = 1), i.e., − 1 < x < 1; 1 is average, < − 1 is below average, and < − 2 is far below average. *Difference > 1 SD, **Difference > critical difference.

Mentions: Psychomotor development gradually deteriorated in the first year after initial status had been normal. At the age of three, the patient went to a regular nursery school with a special group for mentally handicapped children followed by diverse supportive schools for physically and mentally disabled children and subsequent vocational preparation. After discontinuation of primidone, cognition improved for a short period; subsequently, the patient was able to learn to read. Nevertheless, due to the therapy with potassium bromide, cognitive abilities continued to be impaired. Neuropsychological assessment during ongoing bromide therapy included IQ screening, cognitive speed of processing, attention, verbal, and nonverbal memory, verbal and nonverbal fluency, construction and orientation, and the test batteries for the evaluation of attention derived from the Tests of Attentional Performance (TAP). The testing revealed deficits in cognitive speed of processing (Trail Making Test, TMT A), tonic alertness, phasic alertness, divided attention, and selective attention and verbal fluency with an estimated IQ of 90. A second neuropsychological assessment after discontinuation of bromide therapy and conversion to valproic acid confirmed the clinical impression of a clinically relevant improvement of alertness, divided attention, and cognitive speed of processing (TMT A) (Fig. 2).


Seizure control in a patient with Dravet syndrome and cystic fibrosis.

Schmalbach B, Moeller B, von Spiczak S, Muhle H, Stephani U, Lang N - Epilepsy Behav Case Rep (2013)

Comparison of neuropsychological assessment during (2009) and after (2011) therapy with potassium bromide. The figure shows z-values (m = 0, SD = 1), i.e., − 1 < x < 1; 1 is average, < − 1 is below average, and < − 2 is far below average. *Difference > 1 SD, **Difference > critical difference.
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Related In: Results  -  Collection

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f0010: Comparison of neuropsychological assessment during (2009) and after (2011) therapy with potassium bromide. The figure shows z-values (m = 0, SD = 1), i.e., − 1 < x < 1; 1 is average, < − 1 is below average, and < − 2 is far below average. *Difference > 1 SD, **Difference > critical difference.
Mentions: Psychomotor development gradually deteriorated in the first year after initial status had been normal. At the age of three, the patient went to a regular nursery school with a special group for mentally handicapped children followed by diverse supportive schools for physically and mentally disabled children and subsequent vocational preparation. After discontinuation of primidone, cognition improved for a short period; subsequently, the patient was able to learn to read. Nevertheless, due to the therapy with potassium bromide, cognitive abilities continued to be impaired. Neuropsychological assessment during ongoing bromide therapy included IQ screening, cognitive speed of processing, attention, verbal, and nonverbal memory, verbal and nonverbal fluency, construction and orientation, and the test batteries for the evaluation of attention derived from the Tests of Attentional Performance (TAP). The testing revealed deficits in cognitive speed of processing (Trail Making Test, TMT A), tonic alertness, phasic alertness, divided attention, and selective attention and verbal fluency with an estimated IQ of 90. A second neuropsychological assessment after discontinuation of bromide therapy and conversion to valproic acid confirmed the clinical impression of a clinically relevant improvement of alertness, divided attention, and cognitive speed of processing (TMT A) (Fig. 2).

Bottom Line: Some success can be achieved with bromides, but cognitive side effects and disturbed vigilance may limit their use.Here, we present the case of a successfully treated patient with DS and remarkable features in the course of his disease: additionally to DS, the patient was diagnosed with cystic fibrosis (CF), another genetic channelopathy.Seizure freedom could be achieved under treatment with potassium bromide at the age of 15, but at the age of 20, adverse events made it necessary to stop bromide treatment.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Christian-Albrechts-University of Kiel, Arnold-Heller-Str. 3, Haus 41, 24105 Kiel, Germany.

ABSTRACT
Satisfactory treatment of patients with Dravet syndrome (DS) is often difficult. Some success can be achieved with bromides, but cognitive side effects and disturbed vigilance may limit their use. Here, we present the case of a successfully treated patient with DS and remarkable features in the course of his disease: additionally to DS, the patient was diagnosed with cystic fibrosis (CF), another genetic channelopathy. Seizure freedom could be achieved under treatment with potassium bromide at the age of 15, but at the age of 20, adverse events made it necessary to stop bromide treatment. After conversion to valproic acid, the patient remained seizure-free, and neuropsychological tests demonstrated sustained improvement of cognition.

No MeSH data available.


Related in: MedlinePlus