Cerebellar cognitive affective syndrome due to cerebellar atrophy: case report / Síndrome cerebelar cognitivo-afetiva secundária à atrofia cerebelar: relato de caso

Cerebellar atrophy is a rare and challenging disease with few descriptions in the medical literature. Motor impairment is mild, but behavioral and linguistic alterations stand out, in what is known as the cerebellar cognitive affective syndrome secondary to cerebellar atrophy. We report the case of an older woman with early-onset (age 45) signs and symptoms of this syndrome, including impairment of executive functions and visuospatial cognition, personality changes, and language deficits, who was followed at a geriatric medical center for 14 years. Neuropsychological, imaging, and behavioral aspects during this period are discussed in light of scientific evidence. This case report contributes to the scientific literature by describing the progression of the signs and symptoms of cerebellar atrophy over the years, which can help guide medical management and support advice for patients and their families.

A atrofia cerebelar é uma doença rara, desafiadora e com poucas descrições na literatura médica. O prejuízo motor é discreto, mas as alterações comportamentais e de linguagem se destacam, caracterizando a síndrome cognitivo-afetiva cerebelar secundária à atrofia cerebelar. Apresentamos o relato de caso de uma paciente idosa, que apresentou sinais e sintomas dessa síndrome precocemente (aos 45 anos de idade) ­ tais como déficits na função executiva, prejuízo visuoespacial, alterações de personalidade e déficits de linguagem ­ e foi acompanhada em um centro médico geriátrico por um período de 14 anos. Aspectos neuropsicológicos, de imagem e comportamentais durante esse período são comentados à luz das evidências científicas. O caso relatado contribui com a literatura científica ao descrever a evolução dos sinais e sintomas da atrofia cerebelar ao longo dos anos, balizando as condutas médicas e amparando as orientações ao paciente e seus familiares.

Cadmium and Mercury Exposure: Oxidative, Neurobehavioural and Histological Alterations to the Cerebellum of Wistar Rats

Background: The unprecedented increase in metal exposure has been aided by modern industrialization and anthropogenic activities. Cadmium and mercury are recognized as two of the most common heavy metals with destructive impacts on most organ systems. The present study was designed to investigate and improve existing literature on the possible deleterious effects of cadmium and mercury exposure. Methods: Adult Wistar rats were treated with cadmium chloride (5 mg/kg/day) and mercury chloride (4 mg/kg/day) for 14 days. Body, brain and cerebellar weights, motor deficits, antioxidant and lipid peroxidation activities as well as histological alterations to the cerebellum were evaluated at the end of the experiment. Results: Findings showed a significant reduction in body and brain weights, dysregulation of antioxidant enzymes activity and impaired locomotion and exploratory activity in treated rats. Also, an increase in lipid peroxidation and degeneration of Purkinje cells of the cerebellum was observed in treated rats. Conclusion: Overall, these results corroborate previous findings that cadmium and mercury induce deleterious effects on the cerebellum and central nervous system. In addition, this study helps to provide an anatomical perspective and information on the exact cerebellar changes induced by cadmium and mercury in Wistar rats.

Cerebellar infarction after sneezing

SUMMARY Vertebral Artery Dissection (VAD) is a rare condition that can be caused by a wide amplitude of neck movement, which injures the vessel wall and can cause ischemia in the cerebellum. We present a 37-year-old man with herniated lumbar disc and allergic rhinosinusitis, which caused sneezing spells. After one of these bouts with a ricochet of the head, he presented C3 misalignment with local pain. Twenty-one days later, affected by a new crisis, he presented left temporal headache, nystagmus, and vertigo. After 3 days, Magnetic Resonance Imaging (MRI) identified 2 regions of cerebellar ischemia and filling failure of the right vertebral artery. After 2 days, Computed Angiotomography (CT Angiography) was performed and showed right VAD with a local thrombus, without aneurysmal signs. Transcranial Doppler did not indicate an increase in blood flow from this artery. The suggested treatment involved administration of anticoagulant Apixabana 5mg, 12/12h, for 3 months, until the condition was reevaluated with new Angio CT and MRI. It was recommended that the patient was released from work for 1 month and forbidden from doing intense physical exercises for 3 months; however, due to setbacks, these deadlines were extended until a new appointment, 4 months after the first visit. The new tests showed no changes, indicating that the condition was stable. This case aims to indicate the possible investigations of the diagnosis and therapeutic options of the rare association between VAD with cerebellar infarction in a well-documented case.

RESUMO A Dissecção da Artéria Vertebral (DAV) é quadro raro que pode ser causado por movimentação de grande amplitude do pescoço, que lesiona a parede desse vaso, podendo provocar isquemia no cerebelo. Apresentamos um homem de 37 anos, com hérnia de disco e rinossinusite alérgica que lhe causava crises de espirros em salva (CE). Após uma dessas crises com ricocheteamento da cabeça, apresentou desalinhamento de C3 com dor local. Vinte e um dias depois, acometido por nova crise, apresentou cefaleia temporal esquerda, nistagmo e vertigem. Decorridos 3 dias, o paciente foi submetido a Ressoânncia Magnética (RM), que identificou 2 regiões de isquemia cerebelar e enchimento comprometido da artéria vertebral direita. Após 2 dias, foram feitos Angiotomografia Computadorizada (Angio TC), que constatou DAV direita com trombo local, sem sinais aneurismáticos, e Doppler Transcraniano, que não indicou aumento do fluxo sanguíneo dessa artéria. O tratamento sugerido envolvia administração de anticoagulante Apixabana 5mg, 12/12h, por 3 meses, até que o quadro fosse reavaliado com novas Angio TC e RM. Foi recomendado que o paciente ficasse afastado do trabalho por 1 mês e de exercícios físicos intensos por 3 meses, porém devido a contratempos, esses prazos foram prorrogados até nova consulta, 4 meses após a primeira. Os novos exames não apresentaram alterações, indicando que o quadro estava estável. Esse caso tem como objetivo indicar as possíveis investigações do diagnóstico e opções terapêuticas da rara associação entre DAV com infarto cerebelar em caso bem documentado.

Severe Cerebellar Degeneration and Chiari I Malformation - Speculative pathophysiology based on a systematic review

SUMMARY BACKGROUND Symptomatic Chiari Type I Malformation (CM) is treated with posterior fossa decompression with or without duroplasty. We have noticed some cases with concomitant severe cerebellar ataxia due to cerebellar atrophy. The aim of this study is to review the literature of CM associated with severe cerebellar atrophy and discuss its potential physiopathology. METHODS A systematic literature review in the Pubmed Database was performed using the following key-terms: "cerebellar atrophy Chiari", and "cerebellar degeneration Chiari". Articles reporting the presence of cerebellar degeneration/atrophy associated with CM were included. RESULTS We found only six studies directly discussing the association of cerebellar atrophy with CM, with a total of seven cases. We added one case of our own practice for additional discussion. Only speculative causes were described to justify cerebellar atrophy. The potential causes of cerebellar atrophy were diffuse cerebellar ischemia from chronic compression of small vessels (the most mentioned speculative cause), chronic raised intracranial pressure due to CSF block, chronic venous hypertension, and association with platybasia with ventral compression of the brainstem resulting in injury of the inferior olivary nuclei leading to mutual trophic effects in the cerebellum. Additionally, it is not impossible to rule out a degenerative cause for cerebellar atrophy without a causative reason. CONCLUSIONS Severe cerebellar atrophy is found in some patients with CM. Although chronic ischemia due to compression is the most presumed cause, other etiologies were proposed. The real reasons for cerebellar degeneration are not known. Further studies are necessary.

RESUMO OBJETIVO A Malformação de Chiari (MC) tipo I sintomática é tratada através da descompressão da fossa posterior com ou sem duroplastia. Observamos alguns casos com ataxia cerebelar grave concomitante devido à atrofia cerebelar. O objetivo deste estudo é revisar a literatura sobre MC associada à atrofia cerebelar grave e discutir sua possível fisiopatologia. METODOLOGIA Conduzimos uma revisão sistemática da literatura no banco de dados Pubmed utilizando as seguintes palavras-chave: "cerebellar atrophy Chiari", e "cerebellar degeneration Chiari". Artigos sobre a presença de degeneração/atrofia cerebelar associada à MC foram incluídos. RESULTADOS Encontramos apenas seis estudos que discutiam diretamente a associação entre atrofia cerebelar e MC, com um total de sete casos. Nós adicionamos um caso da nossa própria prática para ampliar a discussão. Apenas causas especulativas foram descritas para justificar a atrofia cerebelar, entre elas: isquemia cerebelar difusa devido à compressão crônica de pequenos vasos (a causa especulativa mais citada), pressão intracraniana elevada crônica devido ao bloqueio de LCR, hipertensão venosa crônica e associação com platibasia com compressão ventral do tronco cerebral, resultando em lesão do núcleo olivar inferior e levando a efeitos tróficos mútuos no cerebelo. Além disso, não é possível descartar uma causa degenerativa para atrofia cerebelar sem motivos claros. CONCLUSÃO A atrofia cerebelar grave é encontrada em alguns pacientes com MC. A isquemia crônica causada por compressão é a causa mais apontada como suspeita, porém outras etiologias foram propostas. As reais causas da degeneração cerebelar não são conhecidas. Mais estudos são necessários.

Temblor de Holmes, como manifestación única o predominante en lesiones cerebelosas de origen vascular / Holmes's Tremor as isolated or predominant manifestation in cerebellar lesions of vascular origin

Resumen El temblor de Holmes o rúbrico es infrecuente de ver como manifestación única o predominante de un accidente vascular encefálico cerebeloso. "Grosso modo" el temblor es un trastorno del movimiento habitual en la practica medica, siendo el fisiológico exacerbado el más frecuentemente visto; y el temblor esencial el más común de los movimientos involuntarios patológicos. Los estudios de neuroimágenes han concluido la importancia del cerebelo y de toda la vía cerebelotalámo-cortical en la patogénesis del temblor en general; sin dilucidar aun todas las estructuras involucradas entre las que se menciona a nivel molecular, las células de Purkinje. El temblor de Holmes es una manifestación extrapiramidal frecuente en lesiones cerebelosas de origen vascular; asociándose habitualmente a otra sintomatología de circulación posterior. Presentamos dos casos de temblor rúbrico o de Holmes como manifestación predominante de accidente vascular encefálico. Los autores concluyen que es posible este tipo de presentación, especialmente en pacientes con temblor de acción de inicio ictal, o en aquellos casos de temblor de reposo no parkinsoniano con mala respuesta a la L-Dopa; y en dichos casos, con lesión no mescencefálica, los pacientes podrían beneficiarse del uso de anticolinérgicos.

Holmes's tremor; or rubric tremor is infrequent as predominantly or unique manifestation of cerebellum stroke. Grosso modo tremor is a movement's disorder common in medical practice, being the physiological exacerbated type the most frequently seen, and the essential tremor the most frequently seen in pathological involuntary movements. Neuroimaging studies have concluded the importance of the cerebellum and of the entire cortical-cerebellar- thalamic pathway in the pathogenesis of tremor in general; without specifically of all the structures involved; but among which is mentioned at the molecular level the Purkinje cells. Holmes's tremor, is one of the frequently extrapyramidal manifestations of cerebellar lesions of vascular origin; and it is common to be associated with another symptomatology of posterior circulation. We present two cases of rubric Holmes tremor, as a predominant manifestation of stroke. The authors conclude that this type of presentation is possible, especially in patients with Holmes tremor with ictal onset, or in those cases of non-parkinsonian resting tremor that responds poorly to L-Dopa; and suggest that in such cases, with non-mesencephalic injury, patients could benefit from the use of anticholinergics.

Isolated hemorrhage in the cerebellar vermis with vertigo and body lateropulsion to the contralesional side / 영남의대학술지

There have been several reports of patients with isolated lesions of the cerebellar vermis presenting with clinical features similar to those of peripheral vestibulopathy. We report a case of small, isolated hematoma in the cerebellar vermis in a patient who presented with vertigo, ipsilesional nystagmus, and body lateropulsion to the contralesional side without the usual signs or symptoms of cerebellar dysfunction. Although they present with symptoms that mimic those of peripheral vestibulopathy, and brain computed tomography shows no abnormality, as there may be a small, isolated hematoma or infarction in the cerebellar vermis. Thus, brain magnetic resonance imaging should be performed in elderly patients with vascular risk factors.

Acute cerebellitis caused by herpes simplex virus

Case presentation of acute cerebellitis caused by herpes simplex virus.

Abnormal Eye Movements in Parkinsonism and Movement Disorders

Abnormal eye movements are commonly observed in movement disorders. Ocular motility examination should include bedside evaluation and laboratory recording of ocular misalignment, involuntary eye movements, including nystagmus and saccadic intrusions/oscillations, triggered nystagmus, saccades, smooth pursuit (SP), and the vestibulo-ocular reflex. Patients with Parkinson's disease (PD) mostly show hypometric saccades, especially for the self-paced saccades, and impaired SP. Early vertical saccadic palsy is characteristic of progressive supranuclear palsy-Richardson's syndrome. Patients with cortico-basal syndrome typically show a delayed onset of saccades. Downbeat and gaze-evoked nystagmus and hypermetric saccades are characteristic ocular motor findings in ataxic disorders due to cerebellar dysfunction. In this review, we discuss various ocular motor findings in movement disorders, including PD and related disorders, ataxic syndromes, and hyperkinetic movement disorders. Systemic evaluation of the ocular motor functions may provide valuable information for early detection and monitoring of movement disorders, despite an overlap in the abnormal eye movements among different movement disorders.

Gluten and Neuroimmunology. Rare association with Myasthenia Gravis and Literature Review

SUMMARY As the celiac disease (CD), the non-celiac gluten sensitivity (NCGS) has also been associated with several autoimmune manifestations. It is rarely associated with myasthenia gravis (MG). This paper shall introduce the case of a young female patient, initially presenting a peripheral neuropathy framework. During clinical and neurological follow-up, she began to present symptoms of various immune-mediated morbidities. Diseases related to gluten represent a clinical spectrum of manifestations with a trigger in common, the ingestion of gluten. CD is the most well-known and serious disease of the spectrum, also called gluten-sensitive enteropathy. The NCGS is diagnosed from clinical evidence of improvement in symptoms followed by a Gluten Free Diet (GFD) in patients without signs of enteropathy in duodenal biopsy. There are indications that, although rare, with a prevalence of 1 in 5000, myasthenia gravis (MG) may occur more often when CD is also present. Between 13 to 22% of the patients with MG have a second autoimmune disorder. However, it is often associated with dermatomyositis or polymyositis, lupus erythematosussystemic lupus erythematosus, Addison's disease, Guillain-Barré syndrome and juvenile rheumatoid arthritis. Thus, the symptoms of neuromuscular junction involvement may give a diagnostic evidence of this rare association.

A Case of Posterior Inferior Cerebellar Artery Infarction after Cervical Chiropractic Manipulation / 대한신경손상학회지

We describe the case of a patient who had infarction of the posterior inferior cerebellar artery (PICA) after a chiropractic cervical manipulation. A 39-year-old man visited the emergency room with signs of cerebellar dysfunction, presenting with a 6-hour history of vertigo and imbalance. Two weeks ago, he was treated by a chiropractor for intermittent neck pain. At the time of admission, brain computed tomography, magnetic resonance imaging, and angiography revealed an acute infarction in the left PICA territory and occlusion of the extracranial vertebral artery (VA; V1/2 junction) as a result of the dissection of the VA. Angiography revealed complete occlusion of the left PICA and arterial dissection was shown in the extracranial portion of the VA. He was treated with antiplatelet therapy. Three weeks later, he was discharged without any sequelae. The possibility of VA dissection should be considered at least once in patients presenting with cerebellar dysfunctions with a recent history of chiropractic cervical manipulation.

Malformación arteriovenosa cerebelosa en paciente pediátrico. Presentación de autopsia / Cerebellar arteriovenous malformation in a pediatric patient. Autopsy presentation

Se presenta un paciente masculino de 6 años de edad, quien consultó por cefalea de inicio súbito posterior a un acceso de tos y asociado con síntomas comportamentales. Comentan los padres que en los dos días previos se quejó de odinofagia y tos seca ocasional, sin alteraciones al examen físico de ingreso ni antecedentes clínicos de importancia con posterior fallecimiento. A continuación se presentan los hallazgos de autopsia tanto macroscópica como microscópicamente y una breve discusión teórica del tema.

The current article present a 6-year-old male patient, who consults for sudden onset headache after coughing with behavioral symptoms associated with normal physical exam at admission and no important clinical record, subsequent death without identifiable cause. The parents report a previous two-day symptoms of odynophagia and occasional dry cough. Below are the findings of autopsy both macroscopically and microscopically and a brief theoretical discussion.

Symptomatic Relief of Idiopathic Infratentorial Superficial Siderosis with Maintaining Supine Position

Superficial siderosis results from the deposition of hemosiderin in subpial layers of the central nervous system following hemorrhage in subarachnoid spaces. Infratentorial superficial siderosis (ISS) presents with unique clinical features including progressive hearing loss, ataxia, and myelopathy, and the most common cause of idiopathic ISS is dural abnormality. Here we report a case of idiopathic ISS with radiological findings of spontaneous intracranial hypotension, whose clinical symptoms of ISS including cerebellar dysfunction improved after supine position was maintained for 2 months.

Abiotrofia cerebelar em um gato: relato de caso / Cerebellar abiotrophy in a feline: case report

Um caso de abiotrofia cerebelar em um gato com 45 dias de idade foi diagnosticado no Laboratório de Patologia Animal, Hospital Veterinário da Universidade Federal de Campina Grande. O animal apresentava, havia 15 dias, apatia, anorexia, desidratação, ataxia, hipermetria, espasticidade dos membros torácicos e pélvicos, tremores de intenção, nistagmo, opistótono, déficit proprioceptivo e ausência de resposta de ameaça. Clinicamente, havia a suspeita de hipoplasia cerebelar, e, devido ao prognóstico desfavorável, o animal foi eutanasiado. Na necropsia, não foram observadas alterações macroscópicas. Microscopicamente, as lesões estavam restritas ao cerebelo e caracterizavam-se por alterações neurodegenerativas e necróticas, com desaparecimento segmentar dos neurônios de Purkinje. Nessas áreas, também se verificaram espaços em branco, denominado aspecto de cesto vazio, resultantes da perda dos neurônios de Purkinje, além de raros esferoides axonais e proliferação dos astrócitos de Bergmann. Em algumas áreas, a camada granular estava hipocelular e havia moderada gliose multifocal na camada molecular. O diagnóstico de abiotrofia cerebelar foi realizado com base nos dados epidemiológicos, clínicos e, principalmente, pelas alterações histopatológicas dos neurônios de Purkinje características da doença.(AU)

The aim of this report was to describe a case of cerebellar abiotrophy in cat with 45-year-old diagnosed at the Animal Pathology Laboratory, Veterinary Hospital of the Federal University of Campina Grande. The animal had presented 15-day apathy, anorexia, dehydration and neurological signs, characterized by ataxia, hypermetria, spasticity of fore and hindlimbs, intention tremor, nystagmus, opisthotonos, proprioceptive deficits, and absence of threat response. Clinically, cerebellar hypoplasia was suspected and the animal was euthanized due to poor prognosis. During necropsy, gross lesions were not observed. Microscopically the lesions were restricted to the cerebellum and were characterized by neurodegenerative and necrotic damage with segmental disappearance of the Purkinje cells. In these areas, there were also empty spaces, called the empty basket aspect, resulting from the loss of Purkinje cells, as well as rare axonal spheroids and proliferation of Bergmann's astrocytes. In some areas, the granular layer was hypocellular and there was moderate multifocal gliosis in the molecular layer. The diagnosis of cerebellar abiotrophy was based on epidemiological, clinical and mainly on histopathological changes in neurons of Purkinje disease characteristics.(AU)

Cryptococcal cerebellitis in no-VIH patient / Cerebelitis criptococócica en paciente VIH negativo

Abstract Introduction: Cryptococcosis is an opportunistic fungal infection whose etiology is Cryptococcus neofromans / C. gattii, complex which affects immunocompromised patients mainly. Meningeal infection is one of the most common presentations, but cerebellar affection is rare. Case Description: Male patient with 65 old years, from an area of subtropical climate with chronic exposure to poultry, without pathological antecedents, who presented clinical picture consistent with headache, fever, seizures and altered mental status. Clinical findings and diagnostic methods: Initially without menigeal signs or intracranial hypertension and normal neurological examination. Later, the patient developed ataxia, dysdiadochokinesia and limb loss. By lumbar punction and image of nuclear magnetic resonance (NMR) cerebellitis cryptococcal was diagnosticated. Treatment: Antifungal therapy with amphotericin B and fluconazole was performed, however the patient died. Clinical Relevance: The cryptococcosis has different presentations, it´s a disease whose incidence has been increasing since the advent of the HIV / AIDS pandemy, however the commitment of the encephalic parenchyma and in particular the cerebellum is considered rare. In this way we are facing the first case of cryptococcal cerebellitis in our midst.

Resumen Introducción: La Criptococosis es una infección micótica oportunista cuya etiología es el complejo Cryptococcus neofromans/C. gattii, el cual principalmente afecta pacientes inmunocomprometidos. La afección meníngea es una de las formas más frecuentes pero el compromiso cerebeloso es raro. Descripción del Caso: Paciente masculino de 65 años, procedente de un área rural con exposición crónica a aves de corral, sin antecedentes patológicos, con cuadro clínico inicial consistente en cefalea crónica, fiebre, convulsiones y alteración del estado mental. Hallazgos clínicos y métodos diagnósticos: Al principio sin signos de hipertensión intracraneana ni meníngeos y examen neurológico normal, con posterior desarrollo de ataxia, disdiadococinesia y dismetría. Se diagnosticó Cerebelitis Criptocococica con ayuda de repetidos estudios de LCR y resonancia magnética nuclear. Tratamiento: Se inició terapia antifúngica con Anfotericina B y Fluconazol, con respuesta tórpida y el paciente fallece. Relevancia clínica: La Cerebelitis Criptocococica es una presentación clínica infrecuente que requiere sospecha clínica y recursos diagnósticos para definir el tratamiento de forma temprana. La inmunosupresión no es requisito para padecer esta infección.

Hipoplasia pontocerebelosa secundaria a deleción en el gen CASK: Caso clínico / Pontocerebellar hypoplasia secondary to CASK gene deletion: Case report

Introducción: La hipoplasia pontocerebelosa (HPC) es la reducción del tamaño del cerebelo y la protuberancia secundaria a una alteración en su desarrollo, pudiendo ser provocado por enfermedades neurodegenerativas de causa genética, de las que se conocen 10 subtipos (PCH 1-10), malformaciones corticales, enfermedades metabólicas y enfermedades genéticas. Objetivo: Presentar el caso de una niña con microcefalia, HPC y Síndrome de West, en que el estudio genético permitió llegar al diagnóstico de una deleción en el cromosoma X. Caso clínico: Lactante de 7 meses al diagnóstico, sin antecedentes familiares ni obstétricos de interés, perímetro cefálico (PC) al nacimiento en -1.5 desviaciones estándar (DE). Evolucionó con escasa progresión ponderal y estancamiento del crecimiento del PC, retraso del desarrollo psicomotor, caracterizado por ausencia de fijación de la mirada e hipotonía con reflejos osteotendinosos conservados, y epilepsia refractaria. En los potenciales evocados auditivos se demostró compromiso de las vías pontomesencefálicas y en las neuroimágenes HPC. El estudio genético Array de Hibridación Genómica Comparada (aCGH) demostró deleción parcial heterocigota en el cromosoma X, afectando al gen CASK. Conclusiones: Ante el amplio diagnóstico diferencial que plantea las HPC, las nuevas técnicas citogenéticas han permitido mejorar la clasificación y en algunos casos establecer su etiología, pudiendo ofrecer en estos casos un adecuado asesoramiento genético a las familias.

Introduction: Pontocerebellar hypoplasia (PCH) is a reduction of the size of the cerebellum and pons secondary to an alteration in its development, and can be caused by neurodegenerative diseases of genetic origin, of which there are known 10 subtypes (PCH 1-10), cortical malformations, metabolic and genetic diseases. Objective: To present the case of a child with microcephaly, PCH and West syndrome, in which the genetic study allowed to make the diagnosis of a deletion on chromosome X. Case report: This is a female infant of 7-month at diagnosis, without family or obstetric history of interest, head circumference at birth -1.5 standard deviations (SD). She had little weight and growth in head circumference progression. In addition, physical examination revealed no fixating gaze, hypotonia with preserved deep tendon reflexes. Progressively developed refractary seizures. Brainsteam Auditory Evoked Potential demonstrated involvement of pontomesencefphalic ways and neuroimaging Pontocerebellar hypoplasia. The genetic study (aCGH) showed heterozygous deletion on the X chromosome, affecting the CASK gene. Conclusions: Given the wide differential diagnosis proposed at the PCH, new cytogenetic techniques have improved the classification of HPC and in some cases establish their etiology, so in these cases can provide appropriate genetic counseling to families.

Synthetic Cannabinoid-Induced Immunosuppression Augments Cerebellar Dysfunction in Tetanus-Toxin Treated Mice

Synthetic cannabinoids are one of most abused new psychoactive substances. The recreational use of abused drug has aroused serious concerns about the consequences of these drugs on infection. However, the effects of synthetic cannabinoid on resistance to tetanus toxin are not fully understood yet. In the present study, we aimed to determine if the administration of synthetic cannabinoids increase the susceptibility to tetanus toxin-induced motor behavioral deficit and functional changes in cerebellar neurons in mice. Furthermore, we measured T lymphocytes marker levels, such as CD8 and CD4 which against tetanus toxin. JWH-210 administration decreased expression levels of T cell activators including cluster of differentiation (CD) 3ε, CD3γ, CD74p31, and CD74p41. In addition, we demonstrated that JWH-210 induced motor impairment and decrement of vesicle-associated membrane proteins 2 levels in the cerebellum of mice treated with tetanus toxin. Furthermore, cerebellar glutamatergic neuronal homeostasis was hampered by JWH-210 administration, as evidenced by increased glutamate concentration levels in the cerebellum. These results suggest that JWH-210 may increase the vulnerability to tetanus toxin via the regulation of immune function.

Progressive Supranuclear Gaze Palsy with Predominant Cerebellar Ataxia: A Case Series with Videos

Progressive supranuclear palsy (PSP) with predominant cerebellar ataxia (PSP-C) is a rare phenotype of PSP. The clinical and radiological features of this disorder remain poorly characterized. Through a retrospective case series, we aim to characterize the clinical and radiological features of PSP-C. Four patients with PSP-C were identified: patients who presented with prominent cerebellar dysfunction that disappeared with the progression of the disease. Supranuclear gaze palsy occurred at a mean of 2.0 ± 2.3 years after the onset of ataxia. Mild cerebellar volume loss and midbrain atrophy were detected on brain imaging, which are supportive of a diagnosis of PSP. Videos are presented illustrating the co-existence of cerebellar signs and supranuclear gaze palsy and the disappearance of cerebellar signs with disease progression. Better recognition and the development of validated diagnostic criteria would aid in the antemortem recognition of this rare condition.