Background: Olivopontocerebellar atrophy (OPCA) is an adult-onset disorder. It may occur in a sporadic or familial form. Case report: We present the case of a 62-year-old man with clinical symptoms of progressive cerebellar ataxia. Conclusions: Radiography presented atrophy of the pons and cerebellum. Clinically the syndrome results in progressive ataxia and bulbar dysfunction. Spectroscopy MR shows decrease in NAA/Cr ratio in cerebellum and pons - typical patterns of atrophy

Bulski, Tomasz

Modzelewski, Marek

Ochrynik, Tomasz

Szatkowski, Maciej

Polish Journal of Radiology

Jagiellonian Univeristy Repository

100C A S E  R E P O R TOlivopontocerebellar artrophy in MRI spectroscopy – case reportTomasz Ochrynik, Tomasz Bulski, Marek Modzelewski, Maciej SzatkowskiDepartment of Radiological and Imaging Diagnostics, Medical Centre for Postgraduate Education/Central Clinic Hospital of the Ministry of Interior and Administration, Warsaw, PolandAuthor’s address: Tomasz Bulski, Department of Radiological and Imaging Diagnostics, Medical Centre for Postgraduate Education/Central Clinic Hospital of the Ministry of Interior and Administration, Warsaw, e-mail: tomekbulski@poczta.onet.pl Summary	 Background:  Olivopontocerebellar	 atrophy	 (OPCA)	 is	 an	 adult-onset	 disorder.	 It	may	 occur	 in	 a	 sporadic	 or	familial	form.		 Case report:  We	present	the	case	of	a	62-year-old	man	with	clinical	symptoms	of	progressive	cerebellar	ataxia.				 Conclusions:  Radiography	 presented	 atrophy	 of	 the	 pons	 and	 cerebellum.	 Clinically	 the	 syndrome	 results	 in	progressive	 ataxia	 and	bulbar	 dysfunction.	 Spectroscopy	MR	 shows	decrease	 in	NAA/Cr	 ratio	 in	cerebellum	and	pons	–	typical	patterns	of	atrophy.	 Key words:  Olivopontocerebellar atrophy (OPCA) • H1MRS PDF file: http://www.polradiol.com/fulltxt.php?ICID=468436Otrzymano: 2006.03.04 Zaakceptowano:2006.06.20Sygnatura:Pol J Radiol, 2007; 72(1): 100-102BackgroundOlivopontocerebellar atrophy (OPCA)	occurs	familially	as	a	genetically	conditioned	Holmes	form	 (autosomal	dominant	or	 recessive	 pattern	 of	 inheritance)	 as	well	 as	 a	 sporadic	form	of	Marie-Foix-Alajouanine	(acquired	form).	According	to	 latest	 views	 the	 olivopontocerebellar atrophy	 is	 classi-fied	to	the	group	of	spinocerebellar	ataxias	–	large	hetero-genic	group	of	progresive	neurodegenerative	diseases	of	the	central	nervous	 system.	The	 sporadic	 forms	are	 classified	to	the	group	of	multisystem	atrophies.	The	peak	occurrence	is	in	middle	aged	people.	The	 pathomorphologic	 image	 shows	 atrophies	within	 the	cortex	 and	nuclei	 of	 the	 cerebellum,	 ganglia	 of	 the	 pons,	olives,	white	matter	and	grey	matter.	Atrophies	of	frontal,	parietal	 lobes,	 sometimes	 hyperintensive	 lesions	 on	 T2	weighted	images	(of	demyelinational	character)	of	the	white	matter	of	brain	stem,	cerebellum,	 less	often	–	spinal	cord,	and	atrophic	lesions	of	basal	nuclei	are	also	observed.	The	histopathologic	 image	of	 lesions	within	 the	CNS	 is	 related	to	 the	 degenerating	 processes	which	 lead	 to	massive	 loss	of	 neurons	 in	 the	 cerebellar	 granular	 layer	 and	 axons	 of	the	Purkini	 cells	with	 following	 gliosis.	Moreover,	 lesions	are	also	found	within	the	white	matter	–	demyelination	of	spinocerebellar	 tracts.	Occupancy	 of	 olivopontocerebellar	system	 is	 secondary	 to	 the	 occupancy	of	 the	Purkini	 cells	and	 ought	 to	 be	 associated	 with	 retrograde	 transsynap-tic	 degeneration.	 In	 view	of	 such	hypothesis	 the	 1HMRS		examination,	 especially	 the	evaluation	of	NAA	and	excito-toxic	neurotransmitters	(glutamates-Gln,Glu	“hidden“	in	the	Glx	band)	can	provide	important	information.	Typical	clinical	symptoms	of	the	Olivopontocerebellar	syn-drome	include:	ataxia,	tremor,	dysarthria,	gait	dysfunction,	Parkinson	 symptoms.	The	CT	and	MR	 imaging	 techniques	show	mainly	atrophy	of	the	structures	in	posterior	cranial	fossa,	i.e.	cerebellum	and	brain	stem	(fig.	1a,	1b).	Moreover,	cortical	 atrophy	 of	 frontal	 and	parietal	 lobes	 is	 also	 pos-sible,	 as	well	 as	 the	 less	 frequent	 demyelization	 in	 brain	stem,	 cerebellum	and	 spinal	 cord	which	 are	 presented	 as	hyperintensive	lesions	on	T2	images	and	FLAIR	sequence	of	MR	examination.	Case reportA	62-year-old	patient	with	ataxia	progresing	over	the	last	4–5	years	and	gait	dysfunctions	was	referred	to	the	Department	of	Imaging	Diagnostics	for	definite	diagnosis.	The	MR	of	cer-ebellum	 and	 previous	 tomographic	 examinations	 revealed	101Pol J Radiol, 2007; 72(1): 100-102 Olivopontocerebellar artrophy in MRI...massive	symmetric	cortical-subcortical	atrophy	of	cerebellar	hemispheres	 and	vermis	with	 enlarged	 sulci,	 adjacent	 sub-arachnoid	 cisterns	 and	 the	 4th	 ventricle	widened.	Discrete	signs	of	pons	atrophy	were	also	visible	(fig.	1a,	1b).The	 MR	 examination	 was	 extended	 with	 spectroscopic	single	voxel	H1MRS.	The	H1MRS	spectrum	was	 registered	from	an	8	cm3	single	voxel	using	PRESS	sequence,	with	fol-lowing	parameters:	TR=1.5	s,	TE=35	ms.	The	examinations	were	performed	with	the	use	of	head	coil	and	the	spectrum	recordings	were	preceded	with	field	homogeneity	 improve-ment	within	the	whole	head.	Spectra	were	approximated	in	frequency	domain	with	the	producer	–	supplied	software.	Measuring	 fields	were	 placed	 in	 the	 pons	 and	 symmet-rically	 in	 the	 cerebellar	 hemispheres.	 The	 results	were	Figure 1.	 	Axial	T-1W	and	sagittal	T-1W	images	show	severe	atrophy	of	the	pons	and	cerebellum.	The	supratentorial	structures	show	a	mild	degree	of	atrophy.Figure 2.	 	Single-voxel	proton	MR	spectroscopy	of	cerebellar	hemispheres	and	pons.	Pontine	and	cerebellar	NAA/Cr	and	Cho/Cr	ratios	are	significantly	decreased.	Expression	of	Cho,	mI	and	Lip.	Lesions	poorly	marked	within	the	stem.	A B102Pol J Radiol, 2007; 72(1): 100-102Case Reportreferred	 to	 the	values	of	mean	 indicators	 (metabolite/Cr)	obtained	in	two	institutes	which	use	identical	spectrome-ters	(control	group	comprised	12	patients	with	no	clinical	symptoms	of	cerebellar	injury).	It	was	arbitrarily	decided	that	due	 to	 the	 lack	of	proper	study	group	values	higher	or	lower	than	the	mean	15%	will	be	threshold	for	the	nor-mal	values.	The	H1	MRS	 examination	 revealed	 a	 decrease	 of	 NAA-	concentration,	 mainly	 in	 both	 cerebellar	 hemispheres	where	the	NAA/Cr	proportion	was	about	twice	lower	than	the	 standard,	 and	 a	 significant	 increase	 of	 the	 Cho/Cr	indicator	(fig.	2).	In	addition,	proportion	of	these	metabo-lites	was	also	altered	in	the	pons,	but	to	a	smaller	extent.	However,	 high	 concentration	 of	 Glx,	 Cho	 and	 Lip	 was	observed	(high	lipid	bands	were	also	found	in	the	control	group).	Morphologic	 image	 and	 chemical	 analysis	were	typical	for	atrophy	corresponding	to	the	olivopontocerebel-lar	atrophy	[4,	5,	6].	ConclusionsThe	olivopontocerebellar	atrophy	can	be	recognized	based	on	clinical	symptoms	and	characteristic	morphologic	image.	Extending	the	diagnostics	with	H1	MRS	provides	additional	data	which	 characterize	metabolic	 lesions	 revealed	 in	 the	morphologic	examination	[1,	2]	and	enables	ruling	out	other	neurodegenerative	 diseases	with	 similar	 neuropathologi-cal-clinical	 image,	 such	 as	 the	Friedrich	diseases,	 SLA,	 or	Parkinson.Moreover,	 it	 allows	monitoring	 the	 course	 of	 disease	 and	reaction	 to	 the	 applied	 treatment.	 According	 to	 hitherto	experiences	the	morphologic	image	of	olivopontocerebellar	syndrome	corresponds	to	the	level	of	clinical	advancement	of	the	disease.	Precise	analysis	of	metabolic	profile	(1HMRS)	and	 clinical	 image	 (based	 on	 appropriate	 representative	material)	can	have	significant	therapeutic	implications.	 1.	M.	Mascalchi,	M.	Cosottini,	F.	Lolli,	F.	Salvi,	C.	Tessa,	M.	Macucci,	M.	Tosetti,	R.	Plasmati,	A.	Ferlini,	C.	Tassinari,	N.	Villari.	Proton	MR	spectroscopy	of	the	cerebellum	and	pons	in	patients	with	degenerative	ataxia.	Radiology.	2002	May;	223(2):	371–8.	 2.	H.	Terakawa,	K.	Abe,	Y.	Watanabe,	M.	Nakamura,	N.	Fujita,	N.	Hirabuki,	T.	Yanagihara.	Proton	magnetic	resonance	spectroscoopy	(1H	MRS)	in	patients	with	sporadic	cerebellar	degeneration.	Neuroimaging.	1999	Apr;	9(2):	72–7.	 3.	H.	Nabatame,	H.	Fukuyama,	I.	Akiguchi,	M.	Kameyama,	K.	Nishimura,	Y.	Nakano.	Spinocerebellar	degeneration:	qualitative	and	quantitative	MR	analysis	of	atrophy.	Comput	Assisat	Tomogr.	1998	Mar-Apr;	12(2):	298–303.	 4.	N.	Futamura,	R.	Masumura,	K.	Murata,	A.	Suzumura,	T.	Takayanagi.	An	apparently	sporadic	case	with	spinocerebellar	ataxia	type	1	(SCA1).	Rinsho	Shinkeigaku.	1997	Aug;	37(8):	708–10.	 5.	G.	Tedeschi,	A.	Bertolino,	S.	Massaquoi,	G.	Campbell,	N.	Patronas,	A.	Burnett,	J.	Alger,	M.	Hallett.	Proton	magnetic	resonance	spectroscopic	imaging	in	patients	with	cerebellar	degeneration.	Ann	Neurol.	1996	Jan;	39(1):	71–8.	 6.	M.	Mascalchi,	M.	Tosetti,	R.	Plasmati,	M.	Bianchi,	C.	Tessa,	F.	Salvi,	M.	Valzania,	C.	Bartolozzi,	C.	Tassinari.	Proton	magnetic	resonance	spectroscopy	in	an	Italia	family	with	spinocerebellar	ataxia	type	1.	Ann	Neurol.	1998	Feb;	43(2):	244–52.References:

Olivopontocerebellar artrophy in MRI spectroscopy : case report

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Olivopontocerebellar artrophy in MRI spectroscopy : case report

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