Central Journal of Neurological Disorders & Stroke

Review Article *Corresponding author Reiji Koide, Department of Neurology, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, MRI Findings in Spinocerebellar Fuchu, Tokyo 183-0042, Japan, Tel: 81+42-323-5110; Fax: 81+42-322-6219; Email: Submitted: 02 January 2014 Ataxias Accepted: 07 February 2014 Younhee Kim1, Madoka Kondo1, Yoko Sunami1, Akihiro Kawata1, Published: 21 February 2014 Takashi Komori2, Kiyomitsu Oyanagi3, Imaharu Nakano1 and Copyright Reiji Koide1* © 2014 Koide et al. OPEN ACCESS 1Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan 2Department of Pathology, Tokyo Metropolitan Neurological Hospital, Japan 3Department of Brain Disease Research, Division of Neuropathology, Shinshu Keywords University School of Medicine, Japan • Magnetic resonance imaging • Spinocerebellar ataxia • Radiologic-pathologic correlation Abstract • “Hot cross bun” sign The recent advances in genetic analysis have facilitated the classification of autosomal dominant or recessive spinocerebellar ataxia. Although differential diagnosis of spinocerebellar ataxia is clinically very complicated, MRI characteristics may be an important tool for diagnosis. In the present review article, we summarize the skills of neuroradiology for a diagnosis of various types of spinocerebellar ataxia.

Abbreviations atrophy of the brainstem (predominantly in pontine base), and “hot cross bun” sign (Figure 1) [1,2]. In MSA-C, high-signal- MSA: Multiple System Atrophy; SCA: Spinocerebellar Ataxia; intensity lesions are observed in the pons and middle cerebellar DRPLA: Dentatorubral Pallidoluysian Atrophy; AOA: Ataxia peduncles on T2-weighted MR images. Cruciform hyperintensity with Oculomotor Apraxia; ALD: Adrenoleukodystrophy; AMN: Adrenomyeloneuropathy; PSP: Progressive Supranuclear Palsy; MRI: Magnetic Resonance Imaging; KB staining: Klüver-Barrera D).in the pons is known as “hot cross bun” sign, which may reflect (KB) staining loss of myelinated transverse pontocerebellar fibers (Figure 3A, Spinocerebellar ataxia type 3 (SCA3) I ntroduction Spinocerebellar Ataxia (SCA) is one of a group of most common hereditary SCA in worldwide, and is caused neurodegenerative disorders characterized by slowly progressive SCA3, also known as Machado-Joseph disease, is the dis-coordination of limbs and gait. The recent advances in genetic ataxia,by the expansion spasticity, of and a CAG dystonia, repeat whereas in the ataxin-3 those of gene older-onset [3]. The or recessive SCA. The differential diagnosis of SCA is considered clinical features of younger-onset SCA3 patients are cerebellar oneanalysis of the have most facilitated important the skillsclassification in neurology. of autosomal The article dominant aims ophthalmoplegia. Although atrophy of the brainstem and cerebellumSCA3 patients is common are cerebellar features on ataxia, MRI, it polyneuropathy, should be noted that and Imaging (MRI) in the differential diagnosis of various types of SCA.to review research findings on the value of Magnetic Resonance tegmentumthe MRI abnormalities (Figure 2A,B) are [4].difficult In the to laterdetect stage, in the brainstem early stage and of D iscussion and Conclusion cerebellarSCA3. We atrophy should carefully will be clear, evaluate and we the can atrophy often inobserve the pontine linear Multiple System Atrophy (MSA) high-intensity-signal lesion in the midline of pons coursing an Multiple System Atrophy (MSA) is a neurodegenerative disorder that affects cerebellar, extrapyramidal, and autonomic theanteroposterior pontine nuclei direction are slightly (Figure shrunken, 3B). This but finding the cell is architecture considered systems. The most frequent symptoms include cerebellar isto be well early preserved. change of “hot Therefore, cross bun” the sign. myelinated In SCA3, neurons transverse of ataxia, parkinsonism, and autonomic failures (such as postural hypotension and urinary dysfunction). This condition progresses slowly over several years, and will become being a bedridden state. pontocerebellar fibers are preserved as compared to MSA-C, and Physicians often classify MSA as either MSA-C (with predominant loss of these fibers can be observed only in the midline of pons cerebellar ataxia) or MSA-P (with predominant parkinsonism). where the fibers coming from the left and right pontine nuclei crossSpinocerebellar (Figure 3B, E). ataxia type 1 (SCA1) cerebellar atrophy accompanied by dilatation of fourth ventricle, Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant We discuss here MSA-C. Characteristic MRI findings in MSA-C are

Cite this article: Kim Y, Kondo M, Sunami Y, Kawata A1, Komori T, et al. (2014) MRI Findings in Spinocerebellar Ataxias. J Neurol Disord Stroke 2(3): 1072. Koide et al. (2014) Email: Central

SCA characterized by cerebellar ataxia, spasticity, increased cerebellum. Atrophy of the pons is more prominent in the pontine base than in the pontine tegmentum, and “hot cross bun” sign is SCA1 is caused by the expansion of a CAG repeat in the ataxin-1 observed in the pons on axial T2-weighted MR images (Figure 5) tendon reflexes, cognitive impairment, and ophthalmoplegia. brainstem and cerebellum (Figure 4), which resemble those of gene [5]. Characteristic MRI findings are atrophy of the MSA-C,[10,11]. we MRI should findings consider of SCA2 a possibilityare quite similar of SCA2. to those of MSA-C. the midline of pons coursing an anteroposterior direction is also When a young patient shows on MRI findings resembling to observedSCA3 [6]. (“hotSimilarly cross to bun” SCA3, sign). linear high-intensity-signal lesion in S pinocerebellar ataxia type 6 (SCA6), Spinocerebellar ataxia type 31 (SCA31) S pinocerebellar ataxia type 2 (SCA2) The clinical features of SCA2 include cerebellar ataxia, slow saccadic eye movement, involuntary movement (such as Both spinocerebellar ataxia type 6 (SCA6) and spinocerebellar myoclonus, dystonia), dementia, and polyneuropathy. SCA2 is cardinalataxia type symptom 31 (SCA31) is cerebellar are autosomal ataxia, dominant although mildSCA, anddystonia exhibit in caused by the expansion of a CAG repeat in the ataxin-2 gene late-onset pure cerebellar phenotype. In SCA6 and SCA31, [7-9]. Brain MRI show prominent atrophy of the brainstem and expansion of a CAG repeat in the alpha1A-voltage-dependent the limbs is occasionally observed. SCA 6 is caused by the small

Figure 1 MR images of a patient with MSA-C.

and brainstem associated with dilatation of the fourth ventricle. (A)A 55-year-old In the sagittal Japanese T1-weighted male presented MR image, with atrophy 2-year of history the pons of dysarthria. is more prominent The MR images in the pontineobtained base at 55 than years in theof age pontine show tegmentum.atrophy of the cerebellum (B) Axial T2-weighted MR image shows cruciform hyperintensity (“hot cross bun” sign) in the pons. Middle cerebellar peduncles are also atrophied, with high-signal-intensity lesions.

Figure 2

MR images of a patient with SCA3 MRA 42-year-old images were Japanese obtained female at 42 presented years of age. with slowly progressive gait disturbance since 36 years of age. At 42 years of age, she was unable to walk (A)without Sagittal assistance, T1-weighted and neurological MR image shows examinations mild atrophy showed of the limb pons and (particularly truncal ataxia, in theincreased pontine tendon tegmentum) reflexes, and and cerebellum. extensor plantar responses. The

the arrows). (C)(B) AxialThe pontine T2-weighted tegmentum MR image is defined shows as mild the atrophy dorsal area of the of pons the pons and cerebellum.between the medial lemniscus and the base of the fourth ventricle (between

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Figure 3 (A) Axial T2-weighted MR image in a patient with MSA-C shows atrophy of the pons with cruciform hyperintensity (“hot cross bun” sign). Radiologic-pathologic correlation of MSA-C and SCA3 coursing an anteroposterior direction. (C)(B) Klüver-BarreraAxial T2-weighted (KB) MR staining image sectionin a patient of the with pons SCA3 from shows normal atrophy control. of the pons with linear high-intensity-signal lesion in the midline of pons (D) KB staining section of the pons from a patient with MSA-C (the same case as Panel B) shows atrophy of the pons and marked reduction of the

myelinated fibers including transverse pontocerebellar fibers. (E) KB staining section of the pons from a patient with SCA3 (the same case as Panel C) shows marked atrophy of the pons. Although loss of myelinated fiber is very mild compared with the MSA-C, it is also observed in the midline of pontine base (arrow).

Figure 4 MR images of a patient with SCA1

obtainedThe patient at 44is ayears 44-year-old of age. Japanese female. Her first symptom was gait instability, which began when she was 35 years old. At 44 years of age, (A)neurological Sagittal T1-weighted examinations MR showed image shows obvious atrophy limb andof the truncal cerebellum ataxia, and increased brainstem. tendon reflexes, and moderate dementia. The MR images were (B) Axial T2-weighted MR image shows atrophy of the cerebellum, brainstem, and middle cerebellar peduncles. In the pons, linear high-intensity- signal lesion in the midline of pons is slightly observed (arrow).

D entatorubral pallidoluysian atrophy (DRPLA) insertion containing pentanucleotide repeats within an intron of calcium channel gene [12], and SCA31 is caused by 2.5- to 3.8-kb DRPLA is an autosomal dominant spinocerebellar ataxia caused by the expansion of a CAG repeat in the atrophin-1 gene cerebellar atrophy without brainstem and cerebral involvement the BEAN gene [13]. MRI findings of SCA6 or SCA31 show isolated the patients into a juvenile-onset group and an adult-onset group. [16,17]. On interpretation of the MRI of DRPLA, we should divide (Figure 6 and 7) [14,15]. We are not able to distinguish between SCA6 and SCA31 clinically or radiologically. J Neurol Disord Stroke 2(3): 1072 (2014) 3/5 Koide et al. (2014) Email: Central

Figure 5 MR images of a patient with SCA2

The patient, a 28-year-old Japanese male, presented with gait instability and tremor of the head. His initial symptoms of head tremor developed (A)in his Sagittal 20s. At T1-weighted 28 years of MRage, imageneurological shows examinationsatrophy of the demonstrated cerebellum and tremor brainstem of the associatedhead, slow withsaccadic dilatation eye movement, of the fourth limb ventricle. and truncal Similar ataxia, to MSA-C,dysarthria, atrophy and neckof the dystonia. pons is more The MR prominent images werein the obtained pontine baseat 28 thanyears in of the age. pontine tegmentum. showed in the middle cerebellar peduncles. (B) Axial T2-weighted MR image show atrophy of the cerebellum and brainstem, and “hot cross bun” sign. High-intensity-signal lesions are slightly

Figure 6

MR images of a patient with SCA6. (A)A 60-year-old Sagittal T1-weighted Japanese female MR image presented shows with atrophy slowly of progressivethe cerebellar gait hemispheres disturbance and since vermis. 45 years of age. At 60 year of age, neurological examinations showed limb and truncal ataxia, and she was unable to walk without assistance. The MR images were obtained at 60 years of age. (B) (C) Axial T2-weighted MR images show atrophy of the cerebellum without brainstem and cerebral involvement.

Figure 7

MR images of a patient with SCA31. (A)A 63-year-old Sagittal T1-weighted Japanese male MR presentedimage shows with atrophy a 5-year of history the cerebellar of unsteady vermis. gait. At 63 years of age, neurological examinations showed dysarthria, limb and truncal ataxia. The MR images were obtained at 63 years of age. (B) Axial T2-weighted MR image shows atrophy of the cerebellum without brainstem and cerebral involvement.

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Figure 8 MR images of a patient with DRPLA (adult-onset group).

(A)The Sagittal patient T1-weightedis a 63-year-old MR Japanese image shows male. atrophy He presented of the cerebellumwith unsteady and gait brainstem. at 60 years of age. At 63, he presented limb and truncal ataxia. The MR images were obtained at 63. (B) (C) (D) Axial T2-weighted MR images show high-signal-intensity lesion in the cerebral white matter and pons, in addition to atrophy of the cerebellum.

Figure 9 MR images of a patient with DRPLA (juvenile-onset group). gradually, and the epilepsy remained resistant to drug therapy. At 40 years of age, he was bedridden, and was unable to communicate. The MR imagesThe patient were is obtained a 40-year-old at 40 yearsJapanese of age. male. His symptoms began at 25 years of age with generalized seizure. His intellectual function deteriorated (A) Sagittal T1-weighted MR image shows atrophy of the cerebellum, brainstem, cerebral hemisphere, and corpus callosum. (B) (C) (D) Axial T2-weighted MR images show prominent atrophy of the cerebral hemispheres, in addition to atrophy of the cerebellum. Involvement of the cerebral white matter is not clear.

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The former exhibits myoclonus epilepsy, and the latter cerebellar brainstem atrophy, and, in advanced cases, cortical atrophy ataxia with chorea. On MR imaging, atrophy of the brainstem (Figure 10) [21]. Ataxia with oculomotor apraxia type 2 (AOA2) of MSA, atrophy of the pons is more prominent in the pontine tegmentumand cerebellum than are in the common pontine findings. base. In In adult-onset contrast to group, those AOA2 is an autosomal recessive neurodegenerative disorder high-signal-intensity lesions are observed in the cerebral white characterized by cerebellar ataxia, saccadic ocular pursuit, matter, brainstem, and thalamus on T2-weighted MR images and is caused by mutation in the senataxin gene [22]. The most signal abnormalities in the cerebral white matter are usually strikingoculomotor characteristics apraxia (less of than AOA2 50%), are andthe peripheralpresence of neuropathy, peripheral absent(Figure (or 8) periventricular [18]. On the other white hand, matter in changes juvenile-onset appear group, in the most advanced stage). Severe cerebral atrophy is a characteristic neuropathy and elevated serum levels of alpha-fetoprotein [23]. Miscellaneous diseases masquerading as in juvenile-onset group may correlate to the severe dementia and MRI finding of AOA2 is cerebellar atrophy (Figure 11). spinocerebellar ataxia epilepsyfinding in [19]. juvenile-onset group (Figure 9) [18]. Cerebral atrophy Superficial siderosis: Ataxia with oculomotor apraxia type 1 (AOA1) nervous system is a rare disorder that results of choronic Superficial siderosis of the central AOA1 is an autosomal recessive neurodegenerative disorder subarachnoid hemorrhage and hemosiderin deposition on the characterized by cerebellar ataxia, oculomotor apraxia, and leptomeninges of the brain and spinal cord [24]. Clinical features peripheral neuropathy, and results from a mutation in the of this condition include sensorineural hearing loss, cerebellar aprataxin gene [20]. The key clue that should alert physicians to ataxia, and pyramidal signs. By T2-star weighted image, consider the possibility of AOA1 is the presence of oculomotor hemosiderin deposition on the leptomeninges can be detected with high sensitivity (Figure 12). apraxia (86%) [21]. MRI of AOA1 show cerebellar atrophy, mild

Figure 10 MR images of a patient with AOA1.

The patient is a 37-year-old Japanese male. He presented with unsteady gait at 2 years of age. At 8, a pediatrician had pointed out abnormal ocular (A)movement Sagittal andT1-weighted cerebellar MR atrophy image on shows the MRI. prominent At 37, heatrophy was unable of the cerebellum.to walk, and neurological examinations showed mild mental retardation, limb and truncal ataxia, absent tendon reflexes, and oculomotor apraxia. The MR images were obtained at 37 years of age. (B) Axial T2-weighted MR image shows atrophy of the cerebellum, without obvious involvement of the brainstem.

Figure 11 MR image of a patient with AOA2.

pursuit,The patient but notis a oculomotor55-year-old apraxia.Japanese The male MR with image consanguineous was obtained atparents. 55 years He of presented age. Sagittal with T1-weighted gait disturbance MR image in his shows teens. marked Cerebellar atrophy ataxia of andthe cerebellum.peripheral neuropathy progressed gradually to a wheelchair-bound state at around 30 years. Ophthalmologic examinations showed saccadic ocular

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Figure 12

MR image of a patient with superficial siderosis. AxialThe patient T2-star is weighted a 63-year-old MR image Japanese shows male low-signal presented intensity with asurrounding 1-year history the centralof unsteady nervous gait system and hearing surface, loss. indicating At 63 years hemosiderin of age, he deposition. presented bilateral sensorineural hearing loss, truncal ataxia, and urinary incontinence. The MR image was obtained at 63 years of age.

Figure 13 MR images of a patient with AMN.

(A)The Sagittal patient T1-weightedis a 65-year-old MR Japanese image shows male. mild His firstatrophy symptom of the wascerebellum. gait difficulty, which began when he was 40 years old. Progression of the spastic gait was very so slow that he was still able to walk with a cane at 65 years of age. The MR images were obtained at 65 years of age [25]. (B) Axial T2-weighted MR image shows no remark

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Cite this article Kim Y, Kondo M, Sunami Y, Kawata A1, Komori T, et al. (2014) MRI Findings in Spinocerebellar Ataxias. J Neurol Disord Stroke 2(3): 1072.

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