Reversible chronic cerebellar ataxia after phenytoin intoxication

Botez, M.I.; Gravel, Jacqueline; Attig, Ezzedine; Vézina, Jean-Lorrain

doi:10.1212/wnl.35.8.1152

Cited by 153 publications

(47 citation statements)

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“…Patients with cerebellar cortical atrophy were found to have impaired executive function demonstrated by increased planning time on the Tower of Hanoi Test [Grafman et al 1992], and by poor performance on tests of fluency and the initiation/ perseveration subtest of the Mattis Dementia Rating Scale [Appollonio et al 1993]. Visual spatial deficits were described following excision of left cerebellar hemisphere tumors [Wallesch and Horn 1990], as well as in the setting of chronic phenytoin intoxication [Botez et al 1985], and after left superior cerebellar artery territory infarction [Botez-Marquard et al 1994]. Linguistic processing was impaired in patients with right cerebellar infarction, with symptoms including agrammatism [Silveri et al 1994] and impaired linguistic error detection [Fiez et al 1992].…”

Section: Current Literature On the Relationships Between Et And Cognimentioning

confidence: 99%

The cognitive side of essential tremor: what are the therapeutic implications?

Janicki

Cosentino

Louis

2013

Ther Adv Neurol Disord

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While essential tremor (ET) has traditionally been categorized as a pure motor disease, cross-sectional and longitudinal studies of cognition in ET have demonstrated that these patients may have cognitive dysfunction. Recent epidemiological studies demonstrate an association between ET (particularly with onset after age 65) and increased risk for cognitive impairment and dementia. Although existing studies have generally conceptualized cognitive changes in ET as consistent with a 'frontosubcortical' or 'corticocerebellar' profile, results from these same studies suggest that cognitive impairment in ET may in fact be heterogeneous. Furthermore, the underlying mechanisms remain uncertain. Cognitive changes could be a byproduct of the cerebellar dysfunction of ET itself; alternately, they may be a feature of concomitant neurodegenerative diseases that have been associated in several studies with ET, including Alzheimer's disease, Parkinson's disease or progressive supranuclear palsy. While the study of cognitive dysfunction in ET has received research attention in recent years, the results of these studies have not been translated into the clinical domain and clinical practice. This review first summarizes the current literature on the potential relationships between ET and cognitive change. We then suggest areas of further clinical evaluation and treatment; these suggestions are directed at physicians caring for ET patients who may demonstrate or complain of cognitive impairment. As we discuss, clinicians should ideally screen ET patients for possible signs or symptoms of cognitive impairment in addition to assessing for psychiatric comorbidity and quality of life. These recommendations are in contrast to most current clinical practice, which does not routinely include such assessment among ET patients. To our knowledge, there have been no pharmacotherapeutic trials to date of any agent for cognitive change associated with ET. We believe that studies for this indication are now called for. Future efforts in this direction will also need to take into account the pathobiology of cognitive changes in ET, which itself is an area that is ripe for future investigations.

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Section: Current Literature On the Relationships Between Et And Cognimentioning

confidence: 99%

The cognitive side of essential tremor: what are the therapeutic implications?

Janicki

Cosentino

Louis

2013

Ther Adv Neurol Disord

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“…The view that the cerebellum coordinates the direction of selective attention and as a consequence subserves the execution of cortically generated commands for the enhancement and inhibition of different sources of sensory information has been confirmed in large series of behavioral, neurophysiological, and neuroimaging studies (Courchesne, 1985;Thompson, 1986;Akshoomoff & Courchesne, 1994;Le & Hu, 1996;Akshoomoff, Courchesne, & Townsend, 1997). Botez, Gravel, Attig, and Vézina (1985) first underscored the role of cerebellofrontal and cerebelloparietal associative loops as neural substrates of mild frontal-and parietal-like symptoms encountered in a patient with reversible cerebellar ataxia after chronic phenytoin intoxication. Many of their subsequent studies further explored the role of the cerebellum in visuospatial and visuoconstructive procedures (Botez, Léveillé, Lambert, & Botez, 1991;Botez-Marquard & Botez, 1993;Botez-Marquard, Léveillé, & Botez, 1994;Botez-Marquard & Routhier, 1995;Botez-Marquard, Pedraza, & Botez, 1996).…”

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confidence: 92%

The Lateralized Linguistic Cerebellum: A Review and a New Hypothesis

et al. 2001

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“…For a long period of time, the scientific community considered the cerebellum to have an exclusive motor function. It is only recently that neuroanatomical and clinical evidence suggested that the cerebellum also plays an important role in cognitive function (in particular, Botez, Gravel, Attig, & Vezina, 1985;Leiner, Leiner, & Dow, 1986;Schmahmann, 1991). Schmahmann and Sherman (1998) further clarified the nonmotor role of the cerebellum, describing the cerebellar cognitive affective syndrome (CCAS).…”

Section: Introductionmentioning

confidence: 99%

Successful neuropsychological rehabilitation in a patient with Cerebellar Cognitive Affective Syndrome

Ruffieux¹,

Colombo²,

Gentaz

et al. 2016

Applied Neuropsychology: Child

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The objective of this case study was to describe the neuropsychological rehabilitation of a 16-year-old patient who presented a Cerebellar Cognitive Affective Syndrome (CCAS) following a bilateral cerebellar hemorrhage. The patient presented severe and diffuse cognitive deficits, massive behavioral disorders, and emotion regulation difficulties. The cognitive rehabilitation was performed in the chronic phase (one year after the onset of the hemorrhage) using a transdisciplinary neurobehavioral approach based on the patient's favorite interest (soccer). A significant behavioral and cognitive improvement was observed. The patient became progressively independent in all activities of daily living and was discharged home. The Functional Independence Measure at discharge was 124=126 (vs. 37=126 at entry). The patient was able to complete his schooling despite the mild cognitive and behavioral sequelae. This first description of the use of neurobehavioral therapy in a case of chronic CCAS suggests that (a) major clinical improvement can occur more than one year after the onset of the CCAS, showing the importance of long-term and intensive neurorehabilitation; and (b) when the cerebellum cannot properly play its regulator role in cognition, neuropsychological intervention through a behavioral and cognitive approach can be of great help by acting as an external modulator to help the patient regain control over himself.

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Reversible chronic cerebellar ataxia after phenytoin intoxication

Cited by 153 publications

References 0 publications

The cognitive side of essential tremor: what are the therapeutic implications?

The cognitive side of essential tremor: what are the therapeutic implications?

The Lateralized Linguistic Cerebellum: A Review and a New Hypothesis

Successful neuropsychological rehabilitation in a patient with Cerebellar Cognitive Affective Syndrome

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