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Schmahmann's syndrome - identification of the third cornerstone of clinical ataxiology.

Manto M, Mariën P - Cerebellum Ataxias (2015)

Bottom Line: The syndrome was characterized by four clusters of symptoms including: (a) impairment of executive functions such as planning, set-shifting, verbal fluency, abstract reasoning and working memory, (b) impaired visuo-spatial cognition, (c) personality changes with blunting of affect or abnormal behaviour, and (d) language deficits including agrammatism, wordfinding disturbances, disruption of language dynamics and dysprosodia.This complex of neurocognitive and behavioural-affective symptoms was ascribed to a functional disruption of the reciprocal pathways that connect the cerebellum with the limbic circuitry and the prefrontal, temporal and parietal association cortices.With the introduction of Schmahmann's syndrome, clinical ataxiology has found its third cornerstone, the two others being the cerebellar motor syndrome (CMS) mainly delineated by the pioneer French and English neurologists of the 19(th) and early 20(th) century, and the vestibulo-cerebellar syndrome (VCS) consisting of ocular instability, deficits of oculomotor movements and ocular misalignment.

View Article: PubMed Central - PubMed

Affiliation: Unité d'Etude du Mouvement, FNRS-ULB Erasme, 808 Route de Lennik, 1070 Bruxelles, Belgium.

ABSTRACT
Schmahmann's syndrome represents a novel clinical condition consisting of a constellation of cognitive and affective deficits following cerebellar disease. The complex was first described in 1998 as cerebellar cognitive affective syndrome (CCAS) on the basis of a careful neurological examination, detailed bedside mental state tests, neuropsychological investigations and anatomical neuroimaging of a group of 20 patients with focal cerebellar disorders. The syndrome was characterized by four clusters of symptoms including: (a) impairment of executive functions such as planning, set-shifting, verbal fluency, abstract reasoning and working memory, (b) impaired visuo-spatial cognition, (c) personality changes with blunting of affect or abnormal behaviour, and (d) language deficits including agrammatism, wordfinding disturbances, disruption of language dynamics and dysprosodia. This complex of neurocognitive and behavioural-affective symptoms was ascribed to a functional disruption of the reciprocal pathways that connect the cerebellum with the limbic circuitry and the prefrontal, temporal and parietal association cortices. With the introduction of Schmahmann's syndrome, clinical ataxiology has found its third cornerstone, the two others being the cerebellar motor syndrome (CMS) mainly delineated by the pioneer French and English neurologists of the 19(th) and early 20(th) century, and the vestibulo-cerebellar syndrome (VCS) consisting of ocular instability, deficits of oculomotor movements and ocular misalignment.

No MeSH data available.


Related in: MedlinePlus

Illustration of an unfolded cerebellum with 10 lobules (I to X according to Larsell’s classification). Areas involved in the cerebellar motor syndrome (CMS), in Schmahmann’s syndrome and in the vestibulo-cerebellar syndrome (VCS) are indicated with orange ellipses, blue circles and green circles, respectively. The 3 fundamental syndromes underlying clinical ataxiology show a distinct profile in terms of symptom-lesion mapping. The 3 areas cover the entire cerebellum.
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Fig1: Illustration of an unfolded cerebellum with 10 lobules (I to X according to Larsell’s classification). Areas involved in the cerebellar motor syndrome (CMS), in Schmahmann’s syndrome and in the vestibulo-cerebellar syndrome (VCS) are indicated with orange ellipses, blue circles and green circles, respectively. The 3 fundamental syndromes underlying clinical ataxiology show a distinct profile in terms of symptom-lesion mapping. The 3 areas cover the entire cerebellum.

Mentions: The cerebellum is divided in three lobes: the anterior lobe, the posterior lobe and the flocculo-nodular lobe. The anterior fissure demarcates the first two lobes. The posterolateral fissure is located between the posterior lobe and the flocculo-nodular lobe. Larsell subdivided the cerebellum into 10 lobules (I to X; see Figure 1): the anterior lobe is composed of lobules I-V, the posterior lobe includes lobules VI-IX and the flocculo-nodular lobe corresponds to lobule X [3,4]. Although there is some variability in the shape of the cerebellum between subjects, the 10 lobules are always identified [5]. There is usually an asymmetry in size between the two cerebellar hemispheres, with the left hemisphere being larger than the right one in most cases. Larsell’s nomenclature has major impacts in terms of understanding symptom-lesion mapping in cerebellar disorders.Figure 1


Schmahmann's syndrome - identification of the third cornerstone of clinical ataxiology.

Manto M, Mariën P - Cerebellum Ataxias (2015)

Illustration of an unfolded cerebellum with 10 lobules (I to X according to Larsell’s classification). Areas involved in the cerebellar motor syndrome (CMS), in Schmahmann’s syndrome and in the vestibulo-cerebellar syndrome (VCS) are indicated with orange ellipses, blue circles and green circles, respectively. The 3 fundamental syndromes underlying clinical ataxiology show a distinct profile in terms of symptom-lesion mapping. The 3 areas cover the entire cerebellum.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4552302&req=5

Fig1: Illustration of an unfolded cerebellum with 10 lobules (I to X according to Larsell’s classification). Areas involved in the cerebellar motor syndrome (CMS), in Schmahmann’s syndrome and in the vestibulo-cerebellar syndrome (VCS) are indicated with orange ellipses, blue circles and green circles, respectively. The 3 fundamental syndromes underlying clinical ataxiology show a distinct profile in terms of symptom-lesion mapping. The 3 areas cover the entire cerebellum.
Mentions: The cerebellum is divided in three lobes: the anterior lobe, the posterior lobe and the flocculo-nodular lobe. The anterior fissure demarcates the first two lobes. The posterolateral fissure is located between the posterior lobe and the flocculo-nodular lobe. Larsell subdivided the cerebellum into 10 lobules (I to X; see Figure 1): the anterior lobe is composed of lobules I-V, the posterior lobe includes lobules VI-IX and the flocculo-nodular lobe corresponds to lobule X [3,4]. Although there is some variability in the shape of the cerebellum between subjects, the 10 lobules are always identified [5]. There is usually an asymmetry in size between the two cerebellar hemispheres, with the left hemisphere being larger than the right one in most cases. Larsell’s nomenclature has major impacts in terms of understanding symptom-lesion mapping in cerebellar disorders.Figure 1

Bottom Line: The syndrome was characterized by four clusters of symptoms including: (a) impairment of executive functions such as planning, set-shifting, verbal fluency, abstract reasoning and working memory, (b) impaired visuo-spatial cognition, (c) personality changes with blunting of affect or abnormal behaviour, and (d) language deficits including agrammatism, wordfinding disturbances, disruption of language dynamics and dysprosodia.This complex of neurocognitive and behavioural-affective symptoms was ascribed to a functional disruption of the reciprocal pathways that connect the cerebellum with the limbic circuitry and the prefrontal, temporal and parietal association cortices.With the introduction of Schmahmann's syndrome, clinical ataxiology has found its third cornerstone, the two others being the cerebellar motor syndrome (CMS) mainly delineated by the pioneer French and English neurologists of the 19(th) and early 20(th) century, and the vestibulo-cerebellar syndrome (VCS) consisting of ocular instability, deficits of oculomotor movements and ocular misalignment.

View Article: PubMed Central - PubMed

Affiliation: Unité d'Etude du Mouvement, FNRS-ULB Erasme, 808 Route de Lennik, 1070 Bruxelles, Belgium.

ABSTRACT
Schmahmann's syndrome represents a novel clinical condition consisting of a constellation of cognitive and affective deficits following cerebellar disease. The complex was first described in 1998 as cerebellar cognitive affective syndrome (CCAS) on the basis of a careful neurological examination, detailed bedside mental state tests, neuropsychological investigations and anatomical neuroimaging of a group of 20 patients with focal cerebellar disorders. The syndrome was characterized by four clusters of symptoms including: (a) impairment of executive functions such as planning, set-shifting, verbal fluency, abstract reasoning and working memory, (b) impaired visuo-spatial cognition, (c) personality changes with blunting of affect or abnormal behaviour, and (d) language deficits including agrammatism, wordfinding disturbances, disruption of language dynamics and dysprosodia. This complex of neurocognitive and behavioural-affective symptoms was ascribed to a functional disruption of the reciprocal pathways that connect the cerebellum with the limbic circuitry and the prefrontal, temporal and parietal association cortices. With the introduction of Schmahmann's syndrome, clinical ataxiology has found its third cornerstone, the two others being the cerebellar motor syndrome (CMS) mainly delineated by the pioneer French and English neurologists of the 19(th) and early 20(th) century, and the vestibulo-cerebellar syndrome (VCS) consisting of ocular instability, deficits of oculomotor movements and ocular misalignment.

No MeSH data available.


Related in: MedlinePlus