RESUMO
Higher brain dysfunctions commonly interfere with functional reconstruction during rehabilitation. Most culprit lesions causing higher brain dysfunctions are observed in the cerebral cortex. However, recently, higher brain dysfunction caused by cerebellar lesions, termed cerebellar cognitive affective syndrome (CCAS), has attracted attention. Here, we report a case of CCAS in a patient with cerebellar infarction exhibiting aphasia, where arterial spin labeling (ASL) method of magnetic resonance imaging (MRI) was used to confirm decreased blood flow due to crossed cerebello-cerebral diaschisis (CCCD). The patient was a 5x years old, left-handed female. She was initially admitted to a neurosurgical hospital for dizziness. MRI demonstrated cerebellar infarction in the left posterior inferior cerebellar artery area. The next day, she was admitted to our hospital for surgery after the diagnosis of hemorrhagic cerebellar infarction due to progressive loss of consciousness. Craniotomy was performed to remove the hematoma. Neurological examination revealed fluent aphasia and ataxia in the left upper and lower extremities and trunk. ASL demonstrated decreased cerebral blood flow in the left cerebellar hemisphere and right front-temporal lobe. Therefore, we detected that CCCD resulted in higher brain dysfunction. After 3 months of inpatient rehabilitation, the patient's auditory comprehension, word conversion, and word recall improved. The patient was discharged unaided. This study used ASL to confirm the diagnosis and rehabilitation of the CCAS incidence effectively.
RESUMO
Higher brain dysfunctions commonly interfere with functional reconstruction during rehabilitation. Most culprit lesions causing higher brain dysfunctions are observed in the cerebral cortex. However, recently, higher brain dysfunction caused by cerebellar lesions, termed cerebellar cognitive affective syndrome (CCAS), has attracted attention. Here, we report a case of CCAS in a patient with cerebellar infarction exhibiting aphasia, where arterial spin labeling (ASL) method of magnetic resonance imaging (MRI) was used to confirm decreased blood flow due to crossed cerebello-cerebral diaschisis (CCCD). The patient was a 5x years old, left-handed female. She was initially admitted to a neurosurgical hospital for dizziness. MRI demonstrated cerebellar infarction in the left posterior inferior cerebellar artery area. The next day, she was admitted to our hospital for surgery after the diagnosis of hemorrhagic cerebellar infarction due to progressive loss of consciousness. Craniotomy was performed to remove the hematoma. Neurological examination revealed fluent aphasia and ataxia in the left upper and lower extremities and trunk. ASL demonstrated decreased cerebral blood flow in the left cerebellar hemisphere and right front-temporal lobe. Therefore, we detected that CCCD resulted in higher brain dysfunction. After 3 months of inpatient rehabilitation, the patient's auditory comprehension, word conversion, and word recall improved. The patient was discharged unaided. This study used ASL to confirm the diagnosis and rehabilitation of the CCAS incidence effectively.
RESUMO
Introducción. El diagnóstico de síndrome cerebeloso cognitivo afectivo se debe realizar en aquellos pacientes con lesiones cerebelosas y con déficit cognitivo asociado a deficiencias neuropsicológicas visoespaciales o ejecutivas, trastornos del lenguaje expresivo y trastornos afectivos. Caso clínico. Adolescente de 16 años diagnosticada con trastorno por déficit de atención e hiperactividad a los 7 años, que presenta inestabilidad emocional, apatía y discurso y lectura poco fluidos. Se observan deficiencias visoespaciales en los tests neuropsicológicos. Se realiza una resonancia magnética cerebral por presentar alteración de la coordinación y motricidad fina, y se evidencia atrofia de vermis cerebeloso. La sintomatología es compatible con síndrome cerebeloso cognitivo afectivo. Clásicamente, el cerebelo es conocido por su rol motor. Sin embargo, está implicado en funciones cognitivas superiores, en la expresión emocional y en la regulación conductual. El síndrome cerebeloso cognitivo afectivo es una entidad no bien conocida que debemos incluir en el diagnóstico diferencial de trastornos neuropsiquiátricos con lesión cerebelar.
Introduction. The diagnosis of Cerebellar Cognitive Affective Syndrome should be considered in patients with cerebellar lesions who also suffer cognitive deficits associated with visuospatial or executive 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 motor movements and shows atrophy of the cerebellar vermis. The clinical picture suggests a diagnosis of Cerebellar Cognitive Affective Syndrome. The cerebellum is mostly known for its motor 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.
Assuntos
Humanos , Feminino , Adolescente , Transtorno do Deficit de Atenção com Hiperatividade , Doenças Cerebelares/diagnóstico , Transtornos Cognitivos/diagnóstico , Transtornos do Humor/diagnóstico , CerebeloRESUMO
Paragraphia due to cerebellar cognitive affective syndrome (CCAS) has not been reported previously. This report presents a patient with cerebellar hemorrhage, showing cognitive dysfunctions including paragraphia. A 74-year-old right-handed man was hospitalized with sudden dizziness. CT demonstrated a small hemorrhage in the right cerebellar dentate nucleus. He also exhibited gait disturbance due to mild truncal ataxia and became aware of errors while writing his dairy. Neuropsychological assessments showed phonological paragraphia with Kana characters and semantic paragraphia with Kanji characters, as well as mild auditory comprehension and verbal memory disorders. Although MRI did not detect any abnormality in the cerebral hemispheres, SPECT demonstrated decreased cerebral blood flow in the left angular gyrus, frontal lobe and parieto-occipital lobe, predominantly on the left side. On neuropsychological assessment 3 months after stroke, the patient's paragraphia and auditory comprehension disorder had subsided, but his verbal memory disturbance remained. The clinical features in this case differed from those of CCAS cases previously reported. In particular, the lesion considered responsible for the cognitive dysfunctions in this case involved the right dentate nucleus, which is supplied by the superior cerebellar artery, but not the posterior inferior cerebellar artery, and especially the presence of dis-orders in writing ability, such as paragraphia. The possible mechanism of paragraphia in this case might be a reduction of cerebral blood flow in the angular gyrus due to a crossed cerebello-cerebral diaschisis. However, this case suggests that the right dentate nucleus contributes to the neuropsychological mechanism of writing.
RESUMO
A number of brain imaging and human lesion studies suggest an involvement of the cerebellum in various cognitive functions. A 60-year-old woman developed dizziness due to cerebellar infarction. One month later, she developed cognitive decline, delusion, irritability, impulsive and violent behavior. Cognitive and behavioral symptom onset, neuroimaging findings and neuropsychological test led us to the diagnosis of cerebellar cognitive affective syndrome.