UNIVERSIDAD REGIONAL DEL SURESTE

FACULTAD DE MEDICINA Y CIRUGIA

CURSO DE NEUROLOGIA
QUINTO AO
CICLO LECTIVO: 2015-2016
NOMBRE DEL ALUMNO: ERWIN GERMAN ORTEGA MARIN
DR. ROMEL PEREZ LOPEZ

ARTICULO CEREBELLAR COGNITIVE AFFECTIVE SYNDROME

Sndrome cerebeloso cognitivo-afectivo
Arch.argent.pediatr.vol.113no.5BuenosAiresene.2015

Cerebellar cognitive affective syndrome

Cerebellar cognitive affective syndrome
SUMMARY
Introduction. The diagnosis of cerebellar cognitive affective syndrome should be performed in
patients with cerebellar lesions and cognitive deficits associated with neuropsychological deficits and
visuospatial executive, expressive language disorders and affective disorders. Case report. 16 year
old diagnosed with attention deficit disorder and hyperactivity at age 7, which has emotional
instability, apathy and speech and reading some fluids. Visuospatial deficits are seen in the
neuropsychological tests. A brain MRI is performed to present impaired coordination and fine motor
skills, and atrophy of the cerebellar vermis is evident. The symptoms are consistent with cerebellar
cognitive affective syndrome. Classically, the cerebellum is known for its engine role. However, it is
involved in higher cognitive functions, emotional expression and behavioral regulation. The cerebellar
cognitive affective syndrome is a poorly understood entity must include in the differential diagnosis of
neuropsychiatric disorders with cerebellar injury.
Keywords: Cerebellum; Cognitive disorders; Affective disorders; Attention deficit hyperactivity
disorder; Cerebellar cognitive affective syndrome.
ABSTRACT
Introduction. The diagnosis of Cerebellar Cognitive Affective Syndrome Should be Considered With
cerebellar lesions in patients suffer cognitive deficits WHO Also Associated With executive or
visuospatial neuropsychological disorders, expressive language disorders and affective disorders.
Clinical case. A 16 year old adolescent diagnosed With Attention Deficit Hyperactivity Disorder at the
age of 7 presents With emotional instability, apathy, and speech and reading Difficulties.
Neuropsychological tests show visuospatial Difficulties. A brain magnetic resonance imaging is
performed due to impaired coordination and fine Movements and shows atrophy of the cerebellar
vermis motor. The clinical picture Suggests a diagnosis of Cerebellar Cognitive Affective Syndrome.
The cerebellum is mostly Known for STI engine role. However, It Is Also Involved in higher cognitive
functions, expression of emotion and behavioral regulation. Cerebellar Cognitive Affective Syndrome
is a Relatively unknown diagnosis and Should be included in the differential diagnosis of
neuropsychiatric disorders With cerebellar lesion.
Key words: Cerebellum; Cognitive disorders; Affective disorders; Attention deficit; Hyperactivity
disorders; Cerebellar cognitive affective syndrome.

INTRODUCTION
The cerebellum, traditionally studied as a center of coordination, is also involved in cognitive and
affective functions. Several studies have analyzed the clinical manifestations of cognitive and
affective disorders have been linked to cerebellar functional neuroanatomy and neuroimaging. The
cerebellar cognitive affective syndrome (SCCA) is characterized by the presence of cognitive deficits
associated with neuropsychological deficits and visuospatial executive, expressive language
disorders and affective disorders in patients with cerebellar lesions.
Visuospatial impairment is typically manifested in the form of disorganization and changes in the
generation and manipulation of mental images. Deficiencies in planning, abstract reasoning, flexibility,
verbal fluency and working memory are what characterizes impaired executive function. Expressive
language disorders present in this syndrome are dysprosody, agrammatism and anomie. Affective
disorders can manifest with personality changes, inappropriate behavior, emotional dysregulation,
disinhibition, impulsivity, anxiety, dysphoria and uncontrolled behavior.
That is, patients with cerebellar involvement may present simultaneously motor and cognitiveaffective condition known as SCCA symptoms. However, in clinical practice, are usually diagnosed
with cognitive-affective disorders and cerebellar disorder and it is unusual that a relationship between
them, therefore, receive the diagnosis of suspected SCCA.
CLINICAL CASE
16 year old patient with a history of reflex myoclonic epilepsy noise at 16 months of age, which was
treated with valproic acid for 10 months. At age 7, he was diagnosed with disorder attention deficit
hyperactivity disorder (ADHD), by presenting symptoms compatible (where restlessness and
distraction stands) and get consistent results on the scale of inattention test Conners Parent and
teachers (percentile score 76 and 89, respectively). In the diagnostic interview Kiddie-SADS-PL, he
showed criteria for ADHD combined type. Also presented anxious-phobic temper, apathy, poor
emotional reactivity, rigid behavior and lack of flexibility, so that, through social communication
questionnaire SCQ an autism spectrum disorder was discarded. Methylphenidate treatment was
started, with proper response.
At age 9, a magnetic resonance imaging (MRI) was performed to present alternative movements
incoordination of gait and fine motor deficits. Mild atrophy of the cerebellar vermis was evident. At age
16, the brain MRI and physical examination of fine and gross motor skills have not changed. The
patient finished with graduate school educational program for primary and secondary.
In the psychopathological examination, apathy, poor emotional reactivity and facial expressiveness,
mild psychomotor retardation, speech and poor little fluid content has said, without meeting criteria for
an affective disorder. He presented deficiencies in reading fluency with an unsuitable for their age.
Before the alterations found in the psychopathological examination, it was decided to extend the
study to the administration of a neuropsychological battery. , Working memory, verbal understanding
(100 15 HP: average 78) in estimating the intellectual level was evaluated by the scale of Wechsler
Intelligence for Children (WISC-IV), a discrepant cognitive profile observed (MT: 102; average: 100;
DE: 15) and processing speed (VP: 85; average: 100; DE: 15) were within acceptable limits of
normal, and perceptual reasoning (RP: 64; average: 100,: 15), well below normal. Complex figure of
Rey-Osterrieth (remember: percentile score 1; average: 50) shortcomings in the encoding and
retrieval of visual information and visuospatial orientation is highlighted, Memory Scale Wechsler
(WMS-R: immediate recall : typical score of 23, I remember delayed: typical score of 26 [50 10]),
perceptual reasoning scale (cubes: 4 scale score). He also presented deficiencies in psychomotor
speed processing (Key: 6 scale score (10 3), and Trail Making Test -A: typical score 20) and
sustained attention (d2: Total effectiveness in test -TOT- : percentile score 15; -CON- concentration
index: percentile score 20).
Therefore, he presented affective-cognitive symptoms, visuospatial deficiencies, language disorder
and ADHD associated with a lesion of the vermis, all compatible with SCCA.
At present, he performs a program of professional training. It presents little social activity by
difficulties in social skills, possibly due to their anxious-phobic temperament and cognitive difficulties.

DISCUSSION
In SCCA, the existence of Diaschisis (distal effects of neuronal injury, beyond the area directly
affected) cerebro-cerebellar has been proposed as the organic base to justify the appearance of
symptoms characteristic of cortical regions in patients with cerebellar lesions. The frontal cortex and
the basal ganglia, involved in cognition and affective processing might be inhibited due to cerebellar
injury induce changes in the concentration of neurotransmitters from cerebellum to supratentorial
regions via through-ponto-cerebellar thalamus -cortical. Several studies find cerebellar atrophy and
less activation in the prefrontal lobe functional imaging evidence of ADHD patients compared with
controls, ie, cerebellar injury associated with symptoms characteristic of the prefrontal cortex
(changes in behavior, impulsivity, inattention).
The lateral and posterior regions of the cerebellum are related to cognition, while the cerebellar
vermis abnormalities have been associated with mood disordersThe cerebellar lesions associated
with verbal deficits such as difficulties in formulation and semantics correct syntax, articulation and
speech fluency in reading and writing. Some authors relate these verbal deficiencies right cerebellar
hemisphere and others find no evidence to say that there lateralization. Nor are related to the type
cerebellar lesion (atrophic or focal). Moreover, the cerebellar damage has been linked to cognitive
impairment (such as impairment in visuospatial skills, execution and planning, abstract reasoning and
working memory) and conductoafectivas ( behavioral disinhibition or inhibition, inappropriate
behavior). The cerebellum, through their higher cognitive functions, its involvement in emotional
expression and behavioral regulation, participates in various neuropsychiatric disorders such as
schizophrenia spectrum disorder autism, bipolar disorder, major depression, anxiety disorders, ADHD
and disorders pathological crying.
Although there is insufficient evidence to say that the cerebellum is involved in many functions in
addition to coordination, SCCA remains an underdiagnosed entity. Therefore, we believe it is clinically
relevant report it: in a patient with a cerebellar lesion, cognitive-affective symptoms,
neuropsychological deficits and language disorders, we have to include the SCCA in the differential
diagnosis.
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