tg(ftO._.- "'-'t'" Appendix 2: Case Study Project The purpose of this assignment is to facilitate students' familiarity with and understanding of neurogenic cognitive-communicative disorders. These concepts will be reinforced via a search for and brief presentation of individual clients with acquired neurogenic cognitive-communicative disorders. Students will work independently to find a clinical case that fits the description of a given topic (e.g., motor speech disorders, executive function problems, etc.). Examples of places to find such cases include: journal articles (case studies), medical textbook cases, online stories (e.g., support group websites for a particular disorder), famous cases (e.g., of the Diane Rehm/SD variety), experiences you've had in observing or treating clients (NO names or identifying info please). Each student must present the following regarding their specific case: 1. Source (e.g., AP A citation ofjournal article, name of website, or name of clinical site) a. If your source is a journal article or website, please turn in a copy of the original case b. Note that your case write-up should be in your own words and in laymen's terms. 2. Medical and social history summary 3. Evaluation plan and brief rationale for plan 4. Patient's estimated performance on planned evaluation (general) 5. Prognosis Students will create a short paper (1-2 pages) including the above information that will be turned in to the professor as well as distributed to the rest of the class (electronic distribution prior to class is fine). Each case study will be used during class for practice in goal-writing activities. An example will be shared with the class early in the semester. Due dates will be staggered according to the course schedule, with case studies following current class topics. Students will sign up for their topic/due date at the beginning of the semester. C ase StulY d G ra d'm~ ru b riC: . General Details Content (complete) Medical/social history summary Evaluation plan Evaluation rationale Estimated performance Prognosis Appropriate Matches assigned topic Original wording, laymen's terms Proofreading Spelling, syntax TOTAL POSSIBLE POINTS 50 Points 10 5 5 5 10 5 5 5 NOTE: Goal Writing Assignments The purpose of these assignments is to facilitate students' ability to write appropriate goals for neurogenic cognitive-communicative disorders, across severity levels, WHO levels and multiple medical settings. These concepts will be reinforced via 10 goal-writing exercises over the course of the semester, corresponding to case presentations. Goal-writing assignments are worth 5 points each for a total of 50 possible points, and are graded as follows: +5: Good (realistic, functional, specific, measurable) +4: Adequate (at least 3/4 above criteria met) <4: Inadequate (fewer than 3 criteria met). Re-write goals and turn in by the next class. Rewritten goals can receive a maximum of 4 points. 7 -------------------, COMD688 Fall 2008, Mayer Case Study Grading rubric: General Details Content (complete) Medical/social history summary Evaluation plan Evaluation rationale Estimated performance Prognosis Appropriate Matches assigned topic Proofreading Spelling, syntax TOTAL POSSIBLE POINTS = 50 Points 10 5 5 10 10 5 5 -\~, (so Case 1: Mr. K Setting: out-patient rehab 1. Medical & Social Summary t ~X~~-~'-~ MEDICAL HISTORY: Mr. K is an 81-yom with a diagnosis of spasmodic dysphonia. He reported that this diagnosis was received about 8 years ago, but no form of treatment was sought at that time. Mr. Kstated that his symptoms have worsened over the years and he now is interested in therapy. On September 4, 2008 Mr. Kcame to the NIU Speech-language-Hearing Clinic for a voice evaluation. He wanted an alternative to Botox injections, as he did not feel comfortable with this form of treatment. Mr. Kfirst noticed a change in his voice between 2000 and 2001. His voice had become hoarse and he feared throat cancer. In January of 2001, he saw Dr. X, an otolaryngologist at his commul1ity Ilospital, who made the diagnosis of spasmodic dysphonia of unknown origin. Mr. K reported that he was did not believe this diagnosis and therefore did not seek treatment at that time. However, as his symptoms worsened over the years he felt remorse about not pursuing treatment and sought medical advice again. He returned to the physician as precursor to this evaluation in September. His larynx was assessed endoscopically and therapy was recommended. Mr. K reports that he takes Prilosec for indigestion and Advil for arthritis. He drinks one pot of caffeinated coffee a day and Coke once or twice a day. On occasion, he drinks water and tea. Alcohol and smoking are a part of Mr. K's daily routine. Mr. Khas no known allergies that would be related to vocal fold dysfunction. Mr. K reports that presently he is in relatively good health. He exercises twice a week at the indoor community pool. Past surgeries include knee surgery, gall bladder surgery, and a hip replacement. SOCIAL HISTORY: Mr. K lives in Waukegan with his wife of 59 years. He has 4 grown children who all live close by and are willing and able to care for him. Mr. Kis an active church member, participating in Bible lessons. He sang for many years but reports that his singing voice has changed so drastically that he is no longer able to sing. 2. Evaluation Plan - Oral Mechanism Exam - Hearing Screen to be performed at 25dB for the 500, 1000,2000,4000 Hz - Assessment of slz ratio - Vowel prolongation ability by sustaining the vowellal for as long as possible - Onset of vowels to be evaluated by repeating "ah-ah-ah-ah-ah" - Pitch range and voice flexibility to be subjectively assessed by two glissandos: low to high - Intensity - Resonance - HOlJ..) ~ - Jitter and Shimmer to be assessed by visi-pitch - Quality Case Study Project September 10, 2008 - Rate of speech \ - Subjective evaluation of vocal quality and behaviors during a reading task 3. Estimated Performance Oral Mechanism Exam- OME revealed adequate jaw strength, symmetrical lip movement, and adequate tongue mobility. Normal elevation of the velum was observed during la/ phonation. Overall, rapid speech productions were WNL. Hearing Screen- Hearing was WNl Assessment of s/z ratio- A ratio of 1,3 was evidenced, A ratio of greater than one indicates poor laryngeal control. Vowel prolongation ability- Mr, Kwas able to sustain longer airflow than average despite aphonic breaks Onset of vowels- demonstrated no latency or glottal attacks Pitch and voice flexibility- low pitches were difficult to achieve; however high pitches were obtained more easily --- Voice Flexibility- inflexible indicating a restricted range .-- Intensity~. Mr. Kindicated that speaking loudly was difficult for him, but he was able to display loud tone was prompted Resonance-WI'll - Jitter and Shimmer- significantly highe~ than normal Quality-WNl t!': Rate of speech- in aone minute conversational sample, a range of 177-160 syllables per minute was noted indicating Mr. K's rate of speech is on the low end of an adult speaking rate5, -N-p\u.D Subjective evaluation of vocal quality and behaviors during a reading task- chest b~e~~h~n~ .was . noted along with a hunched over body positioning- 1- hctU ~ vtn..l L. ~~( 4. Prognosis Mr. Kdemonstrates spasmodic dysphonia characterized by insufficient pitch range with difficulty reaching low tones, However, Mr. Kexhibited adequate quality, resonance, and intensity, He was cooperative and receptive to instruction during speech tasks. Additionally he has a strong family support system at home. His medical prognosis is!fir at this time. Speech-language therapy is recommended to enhance Mr, K's quality of life by argeting compensatory techniques and healthy vocal behaviors. U-~I".bt..t. . 5, pr006~~-+S 1"L)~\L';' ..f-- ~rt..·.Sb, Case Study Project September 10, 2008 Goals: -t _/ C;D ~ ~'\r,-,,:,\ (\ ~n:.>'\.Wi\rxy~,c , (4 dl~((':,;"~~'CX")' ~, Trl(}S\k- \."pt",_ L(u}ltJ\..:) Cognitive Communication Disorders Case Study \,\ Ms.D Ms. D. is a 48 yof who was admitted to Provena Mercy Hospital in Aurora with sudden o~igh.t sided weakness and loss of speesili. It was determined she suffered aldl asal ganglia hemorrhage, left parietal hematoma. Evaluation revealed significant ' -1 ~ated blood pressUre and e em rna on CTsc~n. She was then transferred to St. of\J"\bef',~lvG . .,- . Joseph in Joliet. Care there inclu e 0 weeks otiiehabilitation, followed by 4 weeks~t l''!.-h(>':O ~"t;l\')r-.J the Rehabilitation Institute of Chicag Hypertension continues to be uncontrolled by current medication. MRI completed ce with findings as listed above. MRA (magnetic resonance angiography) showed les th n 50% stenosis of both carotids. i Serial head CT demonstrated s~ble em toma without mass effects or shift. Upon disch~ge From RI~ the~~.llE&~ste ou pa len ,reatment. . . Broca:s 'S,;P Hr:.\J..J i'~~ (IDr,l\ :{~,hJt.U1. 11. ' '71 . :\ .(.~. ,.. . . '~( P \ _ . ( aphaSia wIth aprax~a. . ; . . 1. : " l' fl.-{ Ms. D's ; : v:>r.~,,):) pcf\A 1 "2 u v I :4;yJ/.re...\,)t'-. hu:. . t">rtl'. ~ , ~)j • . ',J'; '-. :::;i:~:~stOry is remru~-kable .:;~• ..,. hio hypertension, headaChe';' ,,J-.. "to,~ H \\ oJ):)6 f'J'( 1' (h()'\.~P ;:' 'Jst C~~\)IY'0',- .' kidney disease, and arthritisQyo tob~ Medical charts indicate she was non compliant to medications and treatment program. \ J'\" \ 'h 1. r:r,t'tr( ()oJ r d'( Lr(:.0.:. ~) ) \7 ~'rt IS (J "hpy,. f;,()J't '\ ')fS'S:~''''. 't", <r,f .:_) .' Social Hx Before the incident, Ms. D. worked in a SNF as a nurses aid!. She currently lives at home in DeKalb with her two adult daughters. She is brought to therapy by Trans- Vac transport system in DeKalb and Sycamore. Ms. D enjoys spending weekends'in Chicago with her family and going to the movies. Clinical Interview/Observation: She reported that she has difficulty finding the correct wo!£! at the appropriate time. In addition, she has trouble describing event~. When asked to re-cap and event, she responded with short phrases and often asked, "what is that word?, With regard to receptive lan ua e, she had trouble following conversations going on among family mem ers. Following directions is also challenging. She would like to work on these skills and writing her na.,!!!e. However, other rehab professionals report that her attendance and motivation are inconsistent. Evaluation Plan , (\,;f\. .1Q"<\) '-, , .~~ .\ \y,,,) l. Q ')¥-.I . t \. \~·~~S\ · L \ ' . r t - ~./ ",\J' ~. ~ Token Test- (15-20 minutes)- to assess Ms. D's stamina and\1anguage abilities demonstrated by following directions using 20 to~nd commands about how to manipulate the tokens 62 Cognitive Linguistic Quick Test (15-30 minutes)-specific subtests chosen to quickly assess\ar~ded for effective languagel ({)(Y\r(\\)~\"CfJj)$:r\ , ~U)c:;,~ Boston~ng Test- (20-30 minutes) to assess Ms. D's ability to name both frequently and non-frequently occurring objects .~.~ lI0O\.1W(\-b ~ \.AJN',b:to R~ ~<:"J!i)S.d('>..ri) (c~i ~'rxu\~ evalUZlb.o\\ (A~·ltDJ G'~J\~ \a.eA.d. 8, A~h6S\~~~t~.. ®~~~~_~ ~ E'sfunated Perforn\,lDce .t.® ~~ f':ktl:u 0\' (I tfl'" 01', bI.~ . 1 I w-. ~~(~h~ Toke? Te!!t- Ms. D. was able to accurately foHow 30 of the 62 commands indicating .(\ lmpmqnent . -, ,..., N} ' / 4' \ . ~( 1A'i f~ r ,- 7 \.-- mit d '"? C'(1 0 ~JOJ:(6 't,Z'I '--' \.- ~ V$~-,~ \~ ,~\.",,:> b CLQT• • • • • • Confrontation Naming - moderately impaired Story Retelling - severely impaired Generative Naming - moderately impaired Design Memory - severely impaired Mazes- severely impaired Design Generation.- moderately impaired \ BNT- ,Impaired ~ \J\...-( '. I-nA / / v ~"-v ~ P~ ~ .-~ . 0 . . ().b ,. WCR ( . 1 o:f 1-(-" l (t <- ~ C1lc<..\Q" il) (---' ./cfh\S IS C\aT -tn-eSllfT( dt",,:JJ\ \:<OL (A..+~B(1e CyJt&e_..:r- . .r q11 Ms. D presents with moderate ex ressive and rece tive aphasia. Her primary deficits appear in naming tasks, story/event re-telling, and compre ension of directions. She also exhibits difficulty with memory and organization of language. Her prognosis is poor due to her inconsistent particip~tiori and severity of inj\lIJ(]I0wever, with family support and increased motivation during therapy activities, her prognosis may increase to fair.] -Io\JlC : +~ 1:) fC1D{ reGd ~ +//{ (5 rs;(,-d ',.-/ r,i- rAb This case study was adapted from a case at Unlimited Performance. Mrs. Bishop assisted in acquiring information about this patient's medical and social history. I worked with this client briefly over the summer and used my experience and her diagnosis to estimate performance on the evaluation. ,I . ~'--l \... I. -. ! -. \ 'J r(v·\i(~\.\t0 .. _ 4, ---1 e\J\ "re iXrt'0l1J'rj\ -I., G Case Project: Mr. M -r ; Medical History: Mr. Mis {j8-YOm,;J,vith a history ofCHF and HTN. He was admitted to the hospital due to intense vertigo, nausea, vomiting, and speech and language difficulties in September of200p. After admission, a MRI scan was administered revealing aright } cerebellar-pontine ischemic infarcti 1 in the vascular territory of th~ superior cerebell~r i "al1.ery· A). After a SPECT scan was administered five weeks sip stroke. data revealed I a generalized ~ognitive decline and transcortical sensory aphasi J¥r. M also had xec.u.t.ive dYSfunctions.',. disfll.pted diVI e en lon, IStur ed ViSU.!.~l-SP. atia.l organiz.".ati.on.' visl~al ,~r~ia, sur~~ dys~:~~a, and behavioral abn0l111alities.! " (, f/I'"" r-T "'.\ ,\ t' tr" j.fJ 0-l0 "\-, . } .' \'. ' L) '( '~ Social Hist~~ .., ,,/ ~-" dQ151nf:: CLO{$) (' ('(lO.t \ 't;;. , 0 \it (O(}", Mr. M is a retired restaurant manager who currently lives at home by himself. He has his ck ((j maste(s_g~,g~in~ement. He is very active in his community and participates at '... th~enjor Citizens C1'lbyi.e. bingo and volunteering at the local YMCA). Mr. M's f't 0 CC', augtrreraffirs. on I. ive nearby to assist w.'ith his care at home even though he lives alone. bu.u iOn., R Mr. M is willing clnd motivated for therapy and wants to be able to communicate his ;"""-"--'. wants and needs t~ his family members. (;. C(\()(YVloJ i b) ~,. f}JA,,8. sg -: 0 S Ir\,}(lJ'f icd-f(, Evaluation Plan: \.J i-f"l "'t)-'l( <: pfCcr', \CJ ~ -Western Aphasia Battery- identifies presence, severity, and aphasia classification U ,'j, '") -Cognitive Linguistic Quick Test- assesses major cognitive domains and provides s.everity rating ~ Co,1\,J hl\.\"C ~J b ~r. ~ .f:: O~ -~.""~,-- Estimated Per~ormance: ~)f1€-t.LVl \~ (i'-~,t J"'rJ-.0,;b f()( tt,f't h.,e) t ~ ()($ Western AphasIa Battery scores: . ! ' I .' 'J (\. ! G ~ ~ -r'b ...- ' . \.j- '_:"'-.\.1, "--" ..,-~ I; \. ,,~, ---" ol() b -- i " "' :? ,/,~'------Contel!t: S~yetl! ,-*" _ .\ . \" •.r.,~1) \" \ ,'~' ~-~\ • ". .n * Jet! ('~\tlr-.r -to.'0r::,s/1:::"'/--""¥--' \ '+!\)t(\.L~.' OI\.~.)Ab, . l_e -+. f'l uel1cy. .,. Sever :; \N.J....0- (l.J: -; -:,-~\,-\j ~ 1\ ~· .. '::::::~r \",' _ ...~ 't".( I l ' ( uditory Comprehension: Moderate,A) severe ' ',~_ R i . < .~~Uli'lia~~\' 01 ~ d' . WN e~ .mg. 'elf. ' hl.S • • ~. X• Vv' 11 1\" t \ "' ~ 0' l +urihltr -·--:r? :'!'! (d'I _, v re.~U~FtG, chJJ It>lr_cf .,--;c'rro!JJ. "-- TSA «\u,Cuk p1.., I _'1 h1...dtk~dw,k\l~ ~-6ro k Cognitive Linguistic Quick Test scores: Ju\s..o-f'-bn~l(A.·.i I . ~ ,. _ . \ ntmg: Moderate to severe (Le. neologIsms) • • • • ,J " l-),..;\... . Q , ( , ' ~.~~bLL Attention: Dismpted divided attention '. Memory: Globally decrea..sed meu.Jory. levels J."~' Executive Fu IOjl: Severe , r ~It.;{t> '\::->~ ~~a e: . L ),",01.0 c..r.HJ\ 'iCSll ~ '.. ISlfOspa Ia ~ 1 ,: Moderate to severe Clock Drawing: Moderate tf \ '!) GJ:, c... Prognosis: Mr. M presents with moderate to severe cognitive-linguistic and visuospatial deficits impacting his daily life activities and communication. Given his relatively )::.oung ,2ge, I£9tivation, premorbid intelligence, and family support system, Mr, M's prognosis to return honiewith moderate assistance froIn his family members or caregiver is. fair to good with intensive multidisciplinary liP rehab. Mr. M will require OIP rehab and supervision as well. { / , ' r ICt.se.o xxx CCRTEX ELSEVIER MASSON (2008) [-[() joorrre'l·homep8.\Je;.:www.eIII6vier.j:Qm/locate/cortex Research report Cognitive, linguistic and affective disturbances following a right superior cerebellar artery infarction: A case study Q Q Peter MarienQ,b,c,*, Hanne Baillieux , Hyo lung De Smet , Sebastiaan Engelborghsb,c,J,9, Ineke Wilssens b, Philippe PaquierQ,d,e and Peter P. De Deyn b,C,9 aDepartment of Linguistics, Vrije Universiteit Brussel, Brussels, Belgium bDepartment of Neurology, ZNA Middelheim Hospital, Antwerp, Belgium "Laboratory of Neurochemistry and Behavior, Institute Born-Bunge, University of Antwerp, Antwerp, Belgium dDepartment of Neurology and Neuropsychology, University Hospital, Erasme ULB, Brussels, Belgium eUnit of Neurosciences, Faculty of Medicine, University of Antwerp, Antwerp, Belgium fDepartment of Nursing Sciences, Faculty of Medicine, University of Antwerp, Antwerp, Belgium gDepartment of Health Care Sciences, University College Antwerp (Hogeschool Antwerpen), Antwerp, Belgium ARTICLE INFO ABSTRACT --~.--- Article history: Received 26 June 2007 Reviewed 21 September 2007 Revised 19 October 2007 Accepted 6 December 2007 Action editor Gereon Fink Published online _ Keywords: Cerebellum Cerebellar cognitive affective syndrome Visual dyslexia SCA SPECT - - -..- - -..- - - - - - ... -----~---~---- The cerebellar cognitive affective syndrome (CCAS) is a neurobehavioral syndrome that may develop after congenital and acquired cerebellar lesions. The syndrome consists of deficits in executive functioning, spatial cognition, visual-spatial memory and language and also involves personality and behavioral changes. We describe a 58-year-old righthanded man who in addition to affective disturbances presented with a unique combination of cognitive and linguistic deficits fOHoWIng an Ischellilc llifarctlon in the vascular territory of the nght supenor cerebellar artery (SeA). Neurocognitive and neurolinguistir ;;ammatlons were pertonned In the acute phase (10 days post-onset) and lesion phase .; (four weeks post-onset) of the stroke. A Ic-99m·ECD SPECT studt was performed five wee ke. Acu da revealed a eneralized cognitive decline and tical sensory aphasia. In the lesion phase. the neuro e aVIO was dominated by executive ys unctIons, disrupted divided attention, disturbed visual-spatial organization and bel'lllvi.. ra l ~alities. Neurolinguistic investigations disclosed.l.isual dyslexia and surface dysgraphia~g of words and visual lexical decision tasks of words and nonwords were severely defective and predominantly characterized by visual errors. In addition, writing irregular and ambiguous words resulted in regularization errors (phonologically plau~ors based on phoneme-grapheme correspondence rules). In the absence of any structural damage in the supratentorial brain regions, a quantified SPECT study showed a relative hypoperfusion in the right cerebellar hemisphere an9J!re left medial frontal lobe. CCAS is ror the first time reported in association with visual dyslexia and surface dysgnlEf.ua. We hypothesize that the cognitive and lmguistic deficits might result from functional disruption of the cerebellar-encephalic pathwa s, connecting the cerebellum to the frontal supratentorial areas w lch su serve attentional and planning processes. This phenomenon of crossed cerebellar-cerebral diaschisis is supported by SPECT findings revealing a hypopelrUslon m the anatomoclinically suspected brain , Corresponding author. ZNA - A.Z. Middelheim, Department of Neurology, Lindendreef 1, B-2020 Antwerp, Belgium. E-mail address:peter.marien5@telenet.be (P. Marien). 0010-9452/$ see front matter © 2008 Elsevier Masson Sr!' All rights reserved. doi:10.1016/j .cortex.2007.12.010 2 CORTEX XXX (2008) [0 regions. The constellation of cognitive, linguistic and behavioral symptoms adds new evidence to the multifaceted area of cerebellar neurocognition and demonstrates that the cerebellum might playa crucial role in cognitive, linguistic, and affective processing. @ 2008 Elsevier Masson SrI. All rights reserved. 1. Introduction We describe a patient who presented with a unique combination of cognitive and linguistic deficits following an ischemic infarction in the vascular territory of the right superior cerebellar artery (SCA). From a semiological point of view CCAS is for the first time reported in association with visual dyslexia and surface dysgraphia. From an anatomoclinical point of view our findings demonstrate that disruption of cognitive and linguistic skills may also follow SCA lesions. The current interest in the role of the cerebellum in cognitive, linguistic and affective functions is relatively new. During the past two decades, clinical neuroscience has substantially extended the traditional view on the cerebellum as a mere coordinator of motor functions. In the late 19805, Positron Emission Tomography (PET) studies provided preliminary evidence for cerebellar involvement in non-motor language functions (Petersen et aI., 1988, 1989; Leiner et aI., 1989). In2. Case report deed, in addition to activation of Broca's area, verbal-semantic association tasks - which are generally considered to depend on a close cooperation between verbal and executive abilities2.1. History disclosed functional involvement of the inferior-lateral part of the right cerebellum, which is functionally connected to the RDL, a 58-year-old right-handed man, with an educational left prefrontal language areas (Petersen et aI., 1988, 1989). Delevel of 10 years, was admitted to the hospital because of intense vertigo, nausea, vomiting and speech and language difspite variations on the original task design, several studies ficulties. The clinical neurological examination on admission with PET and functional Magnetic Resonance Imaging (fMRI) revealed a discrete right hemiparesis (MRC 4+/5). Examination have consistently reproduced crosswise cerebrocerebellar activation during semantic and phonological word generation of coordination by finger-to-nose and heel-to-knee tests distasks in both left- and right-handers (Raichle et aI., 1994; closed right-sided hemiataxia consisting of dysmetria and Grabowski et aI., 1996; Chee et aI., 1998; Hubrich-Ungureanu hypermetria of the right arm and leg that was too pronounced to be explained by muscular weakness. Ataxia was only paret aI., 2002; Jansen et a!., 2005). In addition, clinical research tially affected by visual clues. Gait ataxia was noticed as has convincingly shown that focal cerebellar damage can induce a variety of non-motor linguistic deficits among which well. Hypometric saccades were observed when eye movedisrupted articulatory and graphomotor planning (Marien ments were tested. Tendon reflexes were slightly brisker at the right than on the left side of the body. Plantar response et ai., 2007), agrammatism, semantic deficits, distorted language dynamics, aphasia, and reading and writing problems was flexor bilaterally. A discrete hypoesthesia for pinprick wa nd at the right side of the body. Speech was slightly (for a review see Marien et ai., 2001; Paquier and Marien, dysarthri uditory-verbal comprehension and naming 2005). On the neurocognitive level cerebellar damage has been associated with a broad spectrum of symptoms such as wer e ective.n MRI scan of the brain showed a right cereimpaired attentional processes (Gottwald et aI., 2003), execubellar-p e infarction in the vascular territory of the SCA. tive dysfunctions (Schmahmann and Sherman, 1998), learnNo evidence of supratentorial damage was found (Fig. lA-H). ing disability (Drepper et aI., 1999) and disrupted temporal A Tc-99 m-ECD SPECT perfusion scan was performed five and spatial processing (Salman, 2002). In addition to the growweeks post-stroke. Trans-axial images with a pixel size of ing body of evidence for cerebellar involvement in language 3.56 mm were anatomically standardized using SPM and comand cognition, 19th century reports already anecdotically pared to a standard normal and SD image obtained from 15 mentioned behavioral alterations following cerebellar lesions normal ECD perfusion studies. Using a 31 ROJ template the (Combettes, 1831; Otto, 1873). Due to a lack of standardized inZ-scores (SD) were then calculated for each region. A regional vestigations and pathological verification these observations Z-score of >2.0 is considered significant. In comparison to nordid not receive much attention (Dow and Moruzzi, 1958). To mal database findings. the quantified baseline ECD SPECT define a typical constellation of cognitive, linguistic and affecstudy revealed a relative hypoperfusion in the right cerebellar tive symptoms following cerebellar damage Schmahmann hemisphere (-3.15 SD below average) and decreased cerebral and Sherman (1998) introduced the concept of Cerebellar Cogblood flow in the left medial frontal area (-2.21 SD below nitive Affective Syndrome (CCAS). The core features of this average) (Fig. 2). syndrome consist of executive dysfunctions, disrupted spatial cognition, impaired visual-spatial memory, language distur2.2. Neurolinguistic assessments bances, and personality and behavioral disorders. Since the Formal neurolinguistic investigations were performed 10 days first description of the syndrome, CCAS has been reported in a number of etiologically different patients, both children and four weeks after the stroke. The neurolinguistic examinaand adults, with acquired (e.g., Levisohn et aI., 2000; Baillieux tion consisted of the Dutch version (Graetz et aI., 1992) of the et aI., 2006) and congenital cerebellar damage (e.g., Duggal, -Aachener Aphasie Test (AAT) (Huber et ai., 1983), a semantic _ verbal fluency test (animals, transportation, vegetables and 2005; Marien et a!., 2008). CORTEX XXX (2008) -10 3 Fig. 1 - Brain MRI axial FLAIR slices (A-A) showing a right cerebellar-pontine infarction in the vascular territory of the superior cerebellar artery (SCA) (A-D). No structural damage was observed at the supratentorial level (E-H). clothing), and the Boston Naming Test (BNT) (Kaplan et aI., .I 1983; Marien et a1., 1998). Ten days post-stroke, transcortical sensory aphasia was objectified (Table 1). As shown by defective AAT subtest results, the patient had auditory verbal and visual comprehension deficits (total comprehension, 88/120; mean, 108.49; SO, 10.85). The score on the Token Test was nine errors (mean, 2.28; SO, 2.75). Repetition was normal. A deficient result on he BNT (47/60, mean, 52.8; SO, 3.7) indicated disrupted visual onfrontation nam~. The majority of nam1ng errors consisted of 'don't know responses' (10/13) indicating underlying verbal mnestic problems. Speech was ataxic in nature and marked by distorted consonant production, irregular articulatory breakdowns, hypophonia and fast rate. Spontaneous speech was additionally characterized by verbal perseverations and a slightly increased verbal output. However, results on verbal fluency tasks (Table 2) were severely impaired: semantic and phonological fluency scores were 44 (mean, 60.5; SO, 7.14) and 16 (mean, 41; SO, 5.23), respectively. Reading aloud was within the normal range. Writing contained t ~ (\orl' phonological paragraphias. Intensive semantic therapy and articulation therapy were started on a daily basis. As reflected by repeat AAT results, obtained four weeks post-onset neurologieal symptoms, transcortical sensory aphasia receded but written language disturbances persisted. As demonstrated by normal BNT results visual confrontation naming normalized but semantic verbal fluency did not improve (Table 2). Ataxic dysarthria markedly ameliorated. Written language skills were investigated in more detail four weeks post-onset neurological symptoms by means of the Outch version (Bastiaanse et aI., 1995) of the Psycholinguistic Assessments of Language Processing in Aphasia (PALPA) (Kay et aI., 1992). Results on reading aloud and visual lexical decision tasks are shown in Table 3. Overall reading aloud of words was severely defective but reading accuracy was not significantly affected by length, frequency, grammatical class, or regularity of spelling. Word imageability, however, affected reading performance. The patient obtained a defective score of 36/40 (mean, 39.98; SO, 0.16) on reading low imageability words. As demonstrated by maximum scores Fig. 2 - Quantified ECD SPECT scan five weeks post-stroke showing a hypoperfusion in the right cerebellar hemisphere and the left medial frontal area. 4 CORTEX XXX (2008) 1-10 Repetition Confrontation naming Total Score 1441150 147/1.50 144.1 8.07 Nouns 30/30 27/30 25/30 221313 1.001.20 30/30 3DiS13 27.92 27.69 Cqlorterms Compound not;.n~ Se.ntenees lanD 21/30 41/60 26/30 21/30 4Z!60 $BlUO on letter case matching tasks ofreal words and nonwords, letter processing was normal. In addition, visual lexical decision tasks of words and nonwords revealed a severely disrupted performance. The patient identified 53/140 (37.9%) nonwords as real words. The patient's scores on the writing tests of the PALPA are shown in Table 3. 84/144 (58.3%) real words and 17/24 (70.8%) nonwords were correctly written. Word length clearly affected writing. High imageable-high frequent words (9/10) were more adequately written than low imageable-low frequent items (3/ 10), whereas function words (5/5) were better written than nouns (1/5). Regularity had a significant influence on writing performances: regular words were written more often correctly than irregular words (14120 and 5120, respectively). In addition, a frequency effect was also observed in each category: 5/6 regular-high frequent compared to 9/14 regular-infrequent items, and 2/3 irregular-frequent compared to 3/17 irregular-infrequent items. 2.3. Neurocognitiue assessments To evaluate general cognitive abilities and memory, the Wechsler Adult Intelligence Scale-II! (WeChsler, 1997) and Wechsler Memory Scale-R (WMS-R) (Wechsler. 1987) were administered. Executive functioning and problem-solving were investigated by means of the Wisconsin Card Sorting Test (WCST) (Heaton et al.. 1993), the Stroop Colour-Word Test (Hammes. 1971). and the Trail Making Test (Reitan, 1958). The D2 test of attention (Brickenkamp and Zillmer, 1998) and the copy of the Rey-Osterrieth Figure (Osterrieth. 1944) 26/30 114/120 25.69 1.09.3 2.9 1.99 2.61 3.71 8,.42 28/30 1{)~120 26.49 26.79 53.28 28.3 26.91 $$;21 108.49 3.3 3.41 6.08 2.29 3.9 4.9 10.85 2. errors 2.28 2.75 28/30 28130 56/60 26/30 20/~O 4(>/60 28.04 were used to evaluate visual-motor concentration and visuoconstructive praxis (Table 2). In the acute phase of the stroke (14 days post-onset neurological symptoms), disruption of general cognition was reflected by a total Wechsler IQ level of 66. The verbal IQ was 68 and the performal IQ was 71. The Wechsler Memory Scale-R revealed globally decreased memory levels. A discrepancy of 18 index points was found between visual (index = 66) and verbal memory (index 84). As reflected by an index of 59 (- 2. 7 SD) recent memory was severely distorted. Ataxic symptoms affected visuo-motor concentration [D2 impaired for both working rate (Percentile 1) (Pct.) and accuracy, (Pct. 1)]. Generalized concentration disturbances were objectified by means of non-motor tasks (e.g., WMS-R concentration index = 60). Investigation of frontal lobe functions revealed executive disturbances involving divided attention (Stroop: Card I1IIIIll: Pct. 101111). mental flexibility (WCST: Pct. 1; Trail Making A: Pct. 1). and visual-spatial planning (Trail Making B: Pct. 1). A distorted copy of the Rey-Osterrieth figure indicated constructional apraxia (20/36; mean, 35; SD, 3). Behavior was characterized by disinhibited actions, overfamiliarity. confabulations and perseverations. In addition to speech and language therapy a cognitive rehabilitation program was started. Five weeks after the stroke, an overall improvement of cognitive functioning was found (Table 2). Visual (index = 102) and verbal memory (index = 101) had normalized and the discrepancy between both levels resolved. As reflected by deficient scores on the Stroop (Pct. 5/1/ 1) and Trail MakingTest (Pct. 10/10). dysexecutive dysfunctions persisted. A defective performance on concentration (WMS-R: 5 CORTEX XXX (2008) I-fO Table 2 - Acute and lesion phase: cognitive results Lesion phase Norms SD 1.3 15 .15 15 15 .102 101 100 ·sa· Language Boston Naming<Test (BNT) Verbal tl~ency Semantic generation 1'btal'Ournbero('pet~e;verati()ns Total number Qf intnisibns Phon 44 47 3 4 o o 1~ 1 lOG 100 .100 100 15 15 15 15 52.8 3.7 60.5 7.14 41 5.23 g P\:t.l Pct;lO!lIl Pct.5/l11 Pct.50/50/50 PeUO/10 Pct.50/SQ/SO 15 index = 74) and visuo-constructive praxis (Rey-Osterrieth Figure: 25/36) was found as welL Behavior and affective regulation remained aberrant. The patient acted impulsively and displayed disinhibited behavior. He strongly confabulated and had a severely distorted sense of reality. He forcefully denied any cognitive, linguistic or affective problems. Consequently, he refused additional testing and therapy and left the hospital against the advice of family members, doctors and caregivers. This attitude fitted in an overall behavioral profile of indifference and withdrawal. According to his relatives, the patient had become unconcerned and emotionally labile. He withdrew from his friends and family and became socially isolated. He displayed irresponsible, impulsive and stereotypical behavior. After one year, he still avoids any social contact and keeps reo fusing medical follow-up and therapy. 3. Discussion After an ischemic infarction in the vascular territory of the right SeA, our patient developed cognitive and behavioral characteristics consistent with CCAS. In addition, persistent visual dyslexia and surface dysgraphia were found after reo mission of mild transcortical sensory aphasia in the acute hase ofthe'Stroke. Reading an writing disturbances due to cerebellar damage are only scarcely documented in the literature (Moretti et aI., 2002a, 2002b; Marien et aI., 2007). Analysis of RDL's readingerrors disclosed marked visual similarity between the stimulus words and responses indicating acquired visual dyslexia (Marshall and Newcombe, 1973). To our knowledge, this syndrome has only been recorded in nine patients and has never been described in association with cerebellar damage (Casey and Ettlinger, 1960; Marshall and Newcombe, 1973; Lambon Ralph and Ellis, 1997; Cuetos and Ellis, 1999; Sinn and Blanken, 1999; Biran et aI., 2003; Crutch and Warrington, 2007). Acquired visual dyslexia is characterized by visual errors in which at least half the number of the letters of the target word are present (Lambon Ralph and Ellis, 1997). RDL's reading errors on the PALPA subtests were all classified as visual errors. In 13/18 instances, errors occurred in medial word position (72.2%) and in only 2118 (11.1%) in initial and in 3/18 i 6 CORTEX XXX (2008) I Table 3 - PALPA subtests lesion phase: results for reading aloud, visual lexical decision and writing to dictation Mean',. Test $0 24 Word len~•.(llyUab!E!!I) TotaL Stor~ '23124, 23.98 0.16 23-,24 19,98 20.00 0.16 19-20 ~. 0.16 Table 3 (continued) Mean SO Range 4/6 2/6 17/24 5.9 5.98 23.75 0.3 0.16 0.58 5-6 5-6 21-24- lmageability and frequency 9/10 I+IF+ l+/F~ 6/10 1-/1'+ 4/10 1-/1'3/10 Total score 22140 9.9 9.8 9.95 9.65 39.S 0.3 0.51 0.22 0.88 1.42 9-10 115 4.98 0.4 4-5 4/5 SIS 4.98 4.93 19,88 0;16 0,4 4-5' , 4-5 1&-'20 Test SCraphemes 6 Graphemes Total score Reading aloud (palpl\~5;37. ~9. 43) Word lengt'!:l (~~b.mE\l) Total $core '23124 Imageabiltty, ahd,Jreq~e~):Y I+IF+ '20/20 20/20 19/20 17/20 10 19-20 ,39{40 Grammatical class Nouns AdjeetWes '. Verbs func.tionwords To~tlscore ~orE! 3/5 la/20 G~mmatit;;!l class and, im~!ibility ~puns function words Total score 5/10 8/\1.0 1.~110 5 0:16 0 9.75 9.93 19.68 0.58 0,26' 0.75 9.88 13.88 5.68 19.73 0.40 0..61 1;2'3 0.91 0.87 9-10 9-10 9-10 33-40 8-10 9-10 17-20 S.~l)ing--soundregTllarity Regular 1- IRG Totalsc:ore Total regular Npnword~eadirig 3 4 3/6 4/6 5/13 5/6 17/24 Le~r,pr~~sing(P~a 20) Total sl:ore' 60160 ' 6.00 5.98 5B5 0.16 0.22 23,S3 0.3 0.54 59.53 G.B7 5.9 Visu~\l.~~si¢l(p~1;;23.'24"2S} 11;9& 14l~ 'Z9.98 59.as 0;16 oAg '0.16 5-6 s-:6 5-6 21-24 14-15 .12-is Morpitology R.eal words Inflectipns Qel.'j.vatiol\S No,nwords Inflectitms D1!rivations Total score 28130 HilS 14/15 6/30 5/1S ft15 14/60 29.85 14.88 $3.83 13,1!'I 0.42 0.4 0.16 337 2.29 1$7 Writing to dictation (palpa 31,;aS. 39! 40, 42, 43) Word length ' 3 Grapneln~ 6/6 '6 Sr6 5.88 0.33 4 Graphemes Total irregular Total score ," %78, 8-10 7,.~O a-:.14 3-6 15-20 9..28 1;34 3/17 213 5/20 19/40 8.88 15.10 2.85 18.10 37.38 1.71 2.64 0.36 2.93 3.53 5-10 5-10 5-17 2-3 7-20 26-40 17/24 23,33 0,96 21~24 3/10 211() SD, standard deviation; lW. regular. IRG. irregular; 1+, high imageP+, frequentil-, low 1'..... loVl :.29.-30 57-60 70.:&0 156-160 'total ~~e 1'1'+ Nonwcirds Totsl score I~ Nlilnword~ Irregular 11+ 8/10 ~10 9/14 5/6 14120 28-30 13-,15 ,14-15 1S-30 4-15 9-15 5-6 5-6 (16.7%) in final word position. Since initial or final letter positions did not influence RDL's reading performance, a diag· nosis of neglect dyslexia was excluded (Ellis et aI., 1987; War· rington, 1991). A diagnosis of letter·by-letter reading, surface and attentional dyslexia could also be excluded since there was not any effect of word length, regularity, and flanking or migration of letters. However, in addition to a visual component RDL's reading accuracy was influenced by imageability, which is generally considered a characteristic feature of deep dyslexia and indicative of impairments in or around the semantic system (Plaut and Shalliee, 1994). However, although RDL produced a few semantic paraphasias (3/6) on the BNT, he did not make any semantic or visuo-semantic paralexias, which are the cardinal feature of deep dyslexia. RDL's reading ability and visual lexical decision skills were markedly better for words than nonwords. According to Lambon Ralph and Ellis (1997), poor performance on nonword reading may be due to an almost exclusive reliance on lexical processes. Biran et al. (2003) suggested that their patients CORTEX XXX (2008) relied on the available lexical options during word reading, whereas in nonword reading they could only rely on the deficient visual analyzer, resulting in better reading of words over nonwords. ROL's real word reading was marked by verbal morphological paralexias, a type of error that also characterized reading performances of the patients described by Lambon Ralph and Ellis (1997), Cuetos and Ellis (1999), Sinn and Blanken (1999), and Crutch and Warrington (2007). Nonword reading was characterized by application of putative grapheme-phoneme conversion rules (17/24); 617 nonwords were lexicalized and in one nonword a monophthong was diphthongized. A lexicalization effect was also present in visual lexical decision of words versus nonwords since 53/140 (37.9%) nonwords were incorrectly identified as real words. In writing to dictation, RDL produced many neologisms (84.5%) which frequently matched the phonetic characteristics of the target word (pseudohomophones). Paragraphias representing real words occurred only in 9/58 instances (15.5%). Most of ROL's writing errors stemmed from orthographic mistakes and represented phonologically plausible errors (60.3%), characteristic of surface dysgraphia. Patients with surface dysgraphia only apply phoneme-grapheme correspondence rules which typically results in disrupted spelling of irregular and ambiguous words. In surface dysgraphia, spelling depends on the direct translation of the sounds of words into those spelling patterns that are appropriate in a given language (McCarthy and Warrington, 1990). Beauvois and Derouesne (1981) suggested that impairment of a lexical spelling system, without disruption of the phonological writing process, produces surface dysgraphia. Patients with surface dysgraphia make regularization errors, which represent plausible renditions of sound-to-letter correspondences appropriate to the language. Although RDL was able to write some high-frequent irregular or ambiguous words, he also produced regularization errors. Correct spelling of irregular words may occur, suggesting some minimal preservation and contribution from word-specific spelling (Hatfield and Patterson, 1983). Summing-up, on the linguistic level ROL presented with visual dyslexia and surface dysgraphia induced by a right SCA infarction. Fabbro et al. (2000) hypothesized that cerebellar structures, such as the right cerebellar hemisphere and some portions of the vermis, may control written language processes by integrating their activity with the "frontal lobe system". Our findings seem to corroborate this view. Ouring reading and writing, action and perception are strongly coupled, and visual perception is firmly bound to attention and to activation ofbrain regions necessary for action preparation (Moretti et aI., 2002b). Consequently, acquired visual dyslexia after cerebellar lesions might be related to altered oculomotor functioning. However, we hypothesize that ROL's reading and writing deficits might result from damage to the cerebellarencephalic projections, connecting the cerebellum to the prefrontal supratentorial areas which subserve attentional and planning processes (Moretti et aI., 2002a). Visual dyslexia may follow widely distributed dominant hemisphere lesions such as damage to the left fronto-parietal region (Cuetos and Ellis, 1999), and bilateral frontal atrophy (Crutch and Warrington, 2007). However, the condition has not been described in association with cerebellar damage. Clinical <0 7 observations of patients with prefrontal lobe dysfunctions also suggest that complex aspects of writing such as planning and maintained attention may be disturbed (Ardila and Sur!off, 2006). In our patient, the view of a functional disruption of the prefrontal brain regions is supported by quantified SPECT findings which revealed crossed cerebellar-cerebral diaschisis, reflecting the functional impact of the cerebellar lesion on a distant contralateral supratentorial region due to a lack of excitatory impulses (Baron et aI., 1981; Marien, et aI., 2001). Indeed, a quantified SPECT study performed five weeks after the stroke showed a hypoperfusion in the right cerebellar hemisphere and significantly decreased cerebral blood flow in the left medial frontal area. This pattern of perfusion deficits was clinically also associated with a constellation of cognitive and behavioral deficits resembling CCAS (Schmahmann and Sherman, 1998). Several studies of patients with cerebellar lesions have shown similar patterns of neuropsychological deficits in executive functioning, visualspatial abilities and behavior (Chafetz et aI., 1996; Neau et aI., 2000; Paulus et aI., 2004; Kalashnikova et aI., 2005). However, Gomez-Beldarrain et al. (1997), for instance, did not find any memory disturbances, visual-spatial disabilities or executive dysfunctioning in 26 patients with cerebellar strokes. In addition, a number of studies have documented variations in type and severity of symptoms, leading to the conclusion that cognitive and behavioral consequences following cerebellar lesions may vary among the population (Aarsen et aI., 2004; Baillieux et ai., 2006). However, the crosswise functional impact of focal cerebellar damage on distant supratentorial regions that subserve cognitive processes has been amply documented and contributes to the view of a functionally lateralized and topographic organization of the "cognitive cerebellum". In the early 19905, BotezMarquard et al. (1994) for the first time described a patient who in the absence of a supratentorial lesion developed typical posterior right-hemisphere dysfunctions after a left cerebellar lesion. Based on the SPECT findings which revealed a right fronto-parietal hypoperfusion, the authors concluded that the visual-spatial deficits were caused by crossed cerebello-cerebral diaschisis. Further evidence of right-hemisphere dysfunctioning following left cerebellar lesions has been provided by Chafetz et al. (1996) and Allin et al. (2001), supporting the notion of contralateral cerebellar involvement in cognitive modulation. On the neurolinguistic level, Marien et al. (1996) described a patient with left hemisphere "cerebellar induced aphasia" following a right cerebellar infarction. Based on a close longitudinal follow-up of five years with SPECT, the authors introduced the concept of a "lateralized linguistic cerebellum" (Marien et aI., 2001), emphasizing the involvement of the right cerebellar hemisphere in a variety of linguistic processes subserved by the contralateral, language dominant, hemisphere. Consistent with this evidence are the studies of Gottwald et al. (2004) and Hokkanen et al. (2006) who showed that patients with right-sided cerebellar lesions were generally more impaired in the verbal domain, while left-sided cerebellar lesions affected patients significantly in visual-spatial tasks. In addition, many functional neuroimaging studies by means of SPECT (Baillieux et aI., 2006; Marien et aI., 2007) and fMRI (Fink et aI., 2000; Hubrich-Ungureanu et aI., 2002; Jansen 8 <0 CORTEX XXX (2008) 1-10 et aI., 2005) have convincingly demonstrated a lateralized functional organization of the cerebellum in the cerebellocerebral network of cognitive modulation. Exner et al. (2004) recently investigated whether vascular lesions in different parts of the cerebellum result in differential cognitive and affective impairments. The authors concluded that in contrast to subjects with SCA lesions a pattern of memory impairment, executive disturbances and emotional withdrawal is found in patients with infarcts in the PICA territory. These findings are in concordance with many reports in which CCAS follows from PICA lesions (Schmahmann and Sherman, 1998; Paulus et aI., 2004; Willert et aI., 2005). However, Neau et al. (2000) found no significant differences between the cognitive consequences of infarcts in PICA or SCA territory. As in our patient. Botez-Marquard et al. (1994), and Schmahmann and Sherman (1998) reported patients with cognitive disturbances, such as executive dysfunctions and visual-spatial deficits following SCA lesions and Marien et al. (1996, 2000) described dynamic aphasia following a right SeA infarction. In our patient, a possible influence of the brainstem lesion on the cognitive and affective symptoms should be considered since the stroke slightly extended into the pons. Garrard et aL (2002) reported longterm neurocognitive symptoms, mostly involving attention and executive functions in six adult patients with isolated vascular brainstem lesions. The authors explained the specific pattern of symptoms as a disruption of frontal cortical functions, possibly resulting from diaschisis. Similar observations have been made by Salgado et al. (2007). Fazekas et al. (1993) also observed reduced blood flow in the contralateral cerebellar and ipsilateral cortical regions (most prominent in the fronto-parietal region) in two patients with isolated vascular lesions in the upper pons. Unfortunately, no information with regard to the cognitive status of the patients is provided (Fazekas et aI., 1993). The symptoms observed in CCAS are, however, consistent with predictions derived from neuroanatomical and neuroimaging studies, revealing extensive neural circuitries that reciprocally connect the prefrontal, temporal, posterior parietal and limbic cortices with the cerebellum (Desmond, 2001; Schmahmann, 2004). According to Schmahmann (2004), these anatomical sub circuits constitute the structural basis for functional subunits, reflecting a topographic organization of motor and cognitive functions of the cerebellum: the anterior cerebellar lobe is mainly involved in motor functions, while the posterior parts of the cerebellum are involved in higher cognitive modulation. However, as variability may exist with regard to the functional organization of the cerebellum, future research is needed to further elucidate intrigumg Issue 0 a cerebellum". Acknowledgements This study was supported by grant G.0209.05 of the Fund for Scientific Research - Flanders (F. W.O. - Vlaanderen), by Onderzoeksraad (OZR-VUB), Nationale Vereniging tot Steun aan Gehandicapte Personen (NVSG-ANAH), Stichting Integratie Gehandicapten (SIG), and Deloitte Belgium. SE is a postdoctoral fellow of the Fund for Scientific Research Flanders (F.W.O. - Vlaanderen). 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