The ‘Hot Cross Bun’ Sign Is Not Always Multiple System Atrophy: Etiologies of 11 Cases

OBJECTIVE: To clarify the specificity of the ‘hot cross bun’ sign (HCBS) for multiple system atrophy (MSA) in adult cerebellar ataxia or parkinsonism. METHODS: The radiologic information systems at an academic center and affiliated veterans' hospital were queried using the keywords ‘hot cross bun,’ ‘pontocerebellar,’ ‘cruciate,’ ‘cruciform,’ ‘MSA,’ ‘multiple system atrophy,’ and ‘multisystem atrophy.’ Scans were reviewed by a neurologist and neuroradiologist to identify the HCBS. Subjects with the HCBS were reviewed by 2 neurologists to identify the most likely etiology of the patient's neurologic symptoms. RESULTS: Eleven cases were identified. Etiologies included MSA (4 probable, 2 possible), hereditary cerebellar ataxia (3/11), probable dementia with Lewy bodies (1/11), and uncertain despite autopsy (1/11). CONCLUSION: MSA was the most common etiology. However, 5 of the 11 patients did not have MSA. The most common alternate etiology was an undefined hereditary cerebellar ataxia (3/11).

Clinics in diagnostic imaging (191). Multiple system atrophy-cerebellar type (MSA-C)

A 49-year-old Chinese man was evaluated for progressive uncoordinated movements, dysphagia and urinary symptoms. Magnetic resonance imaging demonstrated a cruciform pattern of T2-weighted hyperintensity within the pons and selective atrophy of the cerebellar hemispheres and pons. The clinical history and radiological findings were consistent with a diagnosis of multiple system atrophy-cerebellar type. This article discussed the background, proposed mechanisms, diagnosis, radiological characteristics, prognosis and management of multiple system atrophy-cerebellar type.

Development of the cross sign of olivepontocerebellar atrophy and its diagnostic value / 中国基层医药

Objective To explore the development of the cross sign of olivepontocerebellar atrophy(OPCA) and its diagnostic value.Methods The clinical and imaging data of 8 patients with OPCA were analyzed retrospec-tively.MRI imaging used SE sequence,plain T1 WI,T2 WI,axial,coronal,sagittal imaging.Results All patients were hide onset.The major clinical presentation was cerebellar atsxia and autonomic nerve dysfunction.Positive radiographic findings were consistent with clinical presentation,mainly for the brain stem atrophy,8 patients of the group had this performance,followed by cerebellar atrophy,6 patients had symmetrical atrophy and 2 patients had asymmetric atro-phy.6 patients after onset of 1.5 -3 years showed a typical cross.Conclusion The cross sign gradually appears at a certain stage of development,and it has high specificity.So the combination of clinical and MRI is valuable for the diagnosis and differential diagnosis of OPCA.

Presentación de un caso clínico de atrofia multisistémica y actualización de criterios diagnósticos / Multisystem atrophy and diagnostic criteria updating: A clinical case presentation

La atrofia multisistémica constituye un trastorno neurodegenerativo esporádico de etiología no precisada que se caracteriza por parkinsonismo, trastornos cerebelosos, disfunción autonómica y piramidalismo; los hallazgos patológicos comprenden pérdida celular y gliosis en las neuronas estriatonígricas, olivopontocerebelosas y autonómicas; y la presencia de inclusiones intracitoplasmáticas oligodendrogliales y neuronales, ubiquitina, tau y alfasinucleína positivas. Afecta tanto a hombres como a mujeres, con inicio en la sexta década de la vida como promedio y una prevalencia de 4/100 000. Se presentaron los últimos criterios diagnósticos de atrofia multisistémica y el caso clínico de un paciente de 65 años con un cuadro progresivo, de 4 años de evolución, ataxia cerebelosa progresiva, síndrome rígido acinético, disfunción autonómica, signos piramidales y mala respuesta a la levodopa, con imágenes de resonancia magnética que muestran atrofia de vermis, hemisferios cerebelosos, tallo cerebral (puente) e hipointensidad de ambas regiones putaminales en t2. Se concluyó el caso con el diagnóstico de atrofia multisistémica tipo C

The multisystem atrophy is a sporadic neurodegenerative disorder of unknown origin characterized by parkinsonism, cerebellar disorders, autonomic dysfunction and pyramidal disease, provoked by a cellular loss and gliosis in the nigrostriatal, olivopontocerebellar and autonomic neurons and the presence of oligodendroglia and neuronal intracytoplasmic positive inclusions, ubiquitin, tau and alpha-sinuclein affecting men and women starting as average during the sixth decade of life and a prevalence of 4/100 000. The last diagnostic criteria of multisystem atrophy were showed as well as the clinical case of a patient aged 65 with a progressive picture of 4 years of evolution, progressive cerebellar ataxia, a rigid akinetic syndrome autonomic dysfunction, pyramidal signs and a poor response to levodopa with magnetic resonance images showing vermis atrophy, cerebellar hemispheres, cerebral stem (bridge) and hipointensity of both putamen regions in T2. We conclude that case was diagnosed with type C multisystem atrophy

Clinical and Electro-Oculographic Characteristics of Ocular Flutter

BACKGROUND: Ocular flutter is a rare horizontal eye movement disorder characterized by rapid saccadic oscillations. Excessive discharge of burst neurons, and/or loss of tonic excitation of pause cells cause ocular flutter in several neurologic diseases. Ocular flutter can be easily differentiated from other saccadic oscillations with the aid of electro-oculography (EOG) findings showing an absence of intersaccadic intervals. METHODS: We analyzed EOG findings of ocular flutter in four patients. RESULTS: Ocular flutter, which was shown as rapid, repetitive, horizontal, symmetrical, and sinusoidal movements without intersaccadic intervals on EOG, was confirmed in four patients. The etiology of each patient was olivopontocerebellar atrophy (1 case), meningoencephalitis (2 cases), and lithium intoxication (1 case). CONCLUSIONS: Ocular flutter can be present in numerous neurologic diseases. Characteristic EOG findings are useful in the diagnosis of ocular flutter.

Analysis of the gene mutation and clinical characteristic of hereditary spinocerebellar ataxia type 7 / 临床神经病学杂志

Objective To study the gene mutation and clinical characteristic of hereditary spinocerebellar ataxia type 7 (SCA7).Methods The SCA7 (CAG) trinucleotide repeat mutations were detected by polymerase chain reaction(PCR) and polyacrylamide gel electrophoresis technique in 24 patients with autosomal dominant SCA from 15 families, 20 sporadic SCA patients and 41 normal persons from the same family and 30 healthy persons from different family,the abnormal allele fragments were sequenced by ABI 373 DNA sequencing machine.Results 24 patients with SCA had CAG repeat numbers of SCA 7 allele from 9~18.Normal alleles of SCA 7 had CAG repeat number from 9 to 19. One sporadic SCA patient had one abnormal SCA 7 allele with the CAG repeat expanded to 63 repeats, being confirmed by DNA sequencing.Conclusion CAG expansions were pathogenic cause of SCA 7. The technique of gene mutation detection could provide an effective way for the prediction of asymptomatic and genetic counseling,which was a basis for gene typing.

Changes of vertebral-basal artery and regional cerebral blood flow in patients with olivopontocerebellar atrophy / 临床神经病学杂志

Objective To investigate the changes of vertebral-basal artery and regional cerebral blood flow(rCBF) in patients with olivopontocerebellar atrophy(OPCA).Methods 10 patients with OPCA were examined by digital subtraction angiography(DSA) and single-photon-emission-computed tomography(SPECT),and compared with the control group(patients with vertigo or transient ischemic attack).Results In OPCA group,there were 5 cases with small vertebral artery(50%),4 cases with single vertebral artery(40%),9 cases with small and little vessels(90%),10 cases with poor stain in later arterial phase(100%) could be seen by DSA.The lower rCBF in cerebellum and brain stem were seen in 8 cases(80%)by SPECT.In control group,there were 3 cases(15%),4 cases(20%),4 cases(20%),3 cases(15%) and 7 cases(35%) respectively.There were significant differences between the two groups(all P

Olivopontocerebellar Atrophy

Between 1985and 1987, 31 patients with sporadic olivopontocerebellar atrophy (SOPCA) and 3 patients with familial olivopontocerebellar atrophy (FOPCA) were examined in the Neurologic Clinic of Yongdong Severance Hospital. The incidence of the disease among our neurology clinic patients was 0.9% and 3.4% of those patients were admitted. Seventeen of them were men and seventeen women, and their ages of onset ranged from 16 to 75 years (mean, 48.2 years). In comparison with SOPCA, the disease began earlier in FOPCA (mean age, 51.0 VS 19.3 years), but there were no other differences in clinical feature of the disease. Four patients had parkinsonism, one dementia, and one ophthalmoplegia. None presented spinal involvement or abnormal movements. Eight had a coexisting disease; 3, ch(03)nic alcoholism; 2, hypertension; 2, diabetes mellitus; and 1, malignant neoplasm.