Paroxysmal dysarthria and ataxia in multiple sclerosis

Andermann, Frédérick; Cosgrove, James L.; Lloyd-Smith, Donald; Walters, Allan M.

doi:10.1212/wnl.9.4.211

Cited by 61 publications

(22 citation statements)

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“…Episodes that involve more complex abnormalities are not common but have been described. The episodes of brainstem dysfunction in our patient resembled similar events reported by Andermann et al 10 and Espir et al 11 One of the 2 patients described by Andermann et al had as many as 200 attacks a day that lasted 10 to 15 seconds and subsided over months; these episodes were thought to result from a disturbance in the excitability of demyelinated axons. Our patient's episodes exhibited several brainstem abnormalities suggesting mass synchronous firing as a result of ephaptic transmission (see Smith and McDonald 12 for a review).…”

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confidence: 88%

An Elderly Patient With Bickerstaff Brainstem Encephalitis and Transient Episodes of Brainstem Dysfunction

et al. 2008

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Section: Commentsupporting

confidence: 88%

An Elderly Patient With Bickerstaff Brainstem Encephalitis and Transient Episodes of Brainstem Dysfunction

et al. 2008

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“…The primary cause is inherited episodic ataxia (EA), mostly due to mutations of ion-channel genes [1] with seven identified loci (EA1-EA7) [2]. Paroxysmal episodes have been comprehensively studied, but episodes of symptomatic PDA are poorly documented [3][4][5][6][7][8][9][10][11][12][13][14], and there are no video reports. It is unclear whether symptomatic PDA is characteristic of multiple sclerosis (MS) or can be encountered in other neuropathological settings [5,9,10,13,14].…”

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confidence: 99%

Symptomatic paroxysmal dysarthria-ataxia in demyelinating diseases

et al. 2010

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Paroxysmal dysarthria-ataxia syndrome (PDA) is a rare neurological disorder that can be either primary or symptomatic of acute neurological dysfunction. Episodes of symptomatic PDA are poorly documented and there are no video reports. We describe the cases of two patients with symptomatic PDA related to demyelinating diseases. Detailed studies of the patients' speech disorders showed that the dysarthria and gait disorders were of the ataxic type in both cases. Both patients had midbrain lesions at or below the level of the red nucleus, confirming that this area is critically involved in PDA. The best clinical signs for distinguishing between symptomatic and primary PDA are adult onset and short (<1 min) episodes in the former. If these signs are present, brain MRI should be used to identify a cause of symptomatic PDA.

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“…Epilepsy occasionally occurs in MS (Matthews, 1962) and trigeminal neuralgia is well documented but neither presents any distinctive features. In contrast, tonic seizures (Matthews, 1958), paroxysmal dysarthria (Andermann et al, 1959), and certain sensory symptoms (Espir et al, 1966) are highly characteristic of MS, being seldom identifiable in other conditions. I propose to review, from personal experience, the range of paroxysmal phenomena encountered and their significance.…”

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confidence: 99%