UNIVERSIDAD REGIONAL DEL SURESTEFACULTAD DE MEDICINA Y CIRUGIACURSO DE NEUROLOGIAQUINTO AÑOCICLO LECTIVO: 2015-2016NOMBRE DEL ALUMNO: ERWIN GERMAN ORTEGA MARINDR. ROMEL PEREZ LOPEZ

ARTICULO CEREBELLAR COGNITIVE AFFECTIVE SYNDROME“Síndrome cerebeloso cognitivo-afectivo”

Arch. argent. pediatr. vol.113 no.5 Buenos Aires ene. 2015

Cerebellar cognitive affective syndromeCerebellar cognitive affective syndrome

SUMMARYIntroduction. 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.ABSTRACTIntroduction. 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 cognitive-affective 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 CASE16 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.

DISCUSSIONIn 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.

REFERENCES1. Tavano A, R Grasso, Gagliardi C, Triulzi F, et al. Disorders of cognitive and affective development in cerebellar malformations. Brain 2007; 130 (Pt 10): 2646-60.2. Schmahmann JD, Sherman JC. The cerebellar cognitive affective syndrome. Brain 1998; 121 (Pt 4): 561-793. Dominguez Carral-J, Sáez Carreras-I, JJ García-Peñas, Fournier-Del Castillo C, et al. Cerebellar cognitive affective syndrome secondary to cerebellar tumor. An Pediatr (Barc) 2015; 82 (1): e117-214. De Smet HJ, P Paquier, Verhoeven J, Mariën P. The cerebellum: its role in language and related cognitive and affective functions. Brain Lang 2013; 127 (3): 334-42.5. Durston S, Van Belle J, De Zeeuw P. frontostriatal Differentiating and fronto-cerebellar circuits in attention-deficit / hyperactivity disorder. Biol Psychiatry 2011; 69 (12): 1178-1184.6. Bledsoe JC, Semrud-Clikeman M, Pliszka SR. Neuroanatomical and neuropsychological correlates of the cerebellum in Children with attention-deficit / hyperactivity disorder-combined type. J Am Acad Child Adolesc Psychiatry 2011; 50 (6): 593-601. 7. Gottwald B, B Wilde, Mihajlovic Z, HM Mehdorn. Evidence for distinct cognitive deficits after focal cerebellar lesions. J Neurol Neurosurg Psychiatry 2004; 75 (11): 1524-1531. 8. BeatonA, Mariën P. Language, cognition and the cerebellum: Grappling With an enigma. Cortex 2010; 46 (7): 811-20.9. Tedesco AM, Chiricozzi FR Clausi S, Lupo M, et al. The cerebellar cognitive profile. Brain 2011; 134 (Pt 12): 3672-86. 10. Leggio MG, Tedesco AM, Chiricozzi FR Clausi S, et al. Cognitive impairment in patients sequencing With or atrophic focal cerebellar damage. Brain 2008; 131 (pt 5): 1332-43.11. Molinari M, L Petrosini, Misciagna S, MG Leggio. Visuospatial abilities in cerebellar disorders. J

Neurol Neurosurg Psychiatry 2004; 75 (2): 235-40. 12. Hernáez-Goñi P, Tirapu-Ustárroz J, Iglesias-Fernández L, Luna-Lario P. role of the cerebellum in the regulation of affect, emotion and behavior. Rev Neurol 2010; 51 (10): 597-609. 13. Stoodley CJ, Schmahmann JD. Evidence for topographic organization in the cerebellum of motor control versus cognitive and affective processing. Cortex 2010; 46 (7): 831-44.