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SAGE-Hindawi Access to Research International Journal of Alzheimer’s Disease Volume 2011, Article ID 536141, 6 pages doi:10.4061/2011/536141 Case Report An Unusual Cause of Dementia: Essential Diagnostic Elements of Corticobasal Degeneration—A Case Report and Review of the Literature F. Mastrolilli, A. Benvenga,L.DiBiase,F.Giambattistelli,L.Trotta,G.Salomone, L. Quintiliani, D. Landi, J. M. Melgari, and F. Vernieri Department of Neurology, “Campus Biomedico” University, Via Alvaro del Portillo, 21-00128, Rome, Italy Correspondence should be addressed to F. Mastrolilli, firstname.lastname@example.org Received 15 January 2011; Revised 21 April 2011; Accepted 18 May 2011 Academic Editor: Florinda Ferreri Copyright © 2011 F. Mastrolilli et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Corticobasal degeneration (CBD) is an uncommon, sporadic, neurodegenerative disorder of mid- to late-adult life. We describe a further example of the pathologic heterogeneity of this condition. A 71-year-old woman initially presented dysarthria, clumsiness, progressive asymmetric bradykinesia, and rigidity in left arm. Rigidity gradually involved ipsilateral leg; postural instability with falls, blepharospasm, and dysphagia subsequently developed. She has been previously diagnosed as unresponsive Parkinson’s Disease. At our clinical examination, she presented left upper-arm-ﬁxed-dystonia, spasticity in left lower limb and pyramidal signs (Babinski and Hoﬀmann). Brain MRI showed asymmetric cortical atrophy in the right frontotemporal cortex. Neuropsychological examination showed an impairment in visuospatial functioning, frontal-executive dysfunction, and hemineglect. This case demonstrates that association of asymmetrical focal cortical and subcortical features remains the clinical hallmark of this condition. There are no absolute markers for the clinical diagnosis that is complicated by the variability of presentation involving also cognitive symptoms that are reviewed in the paper. Despite the diﬃculty of diagnosing CBD, somatosensory evoked potentials, motor evoked potentials, long latency reﬂexes, and correlations between results on electroencephalography (EEG) and electromyography (EMG) provide further support for a CBD diagnosis. These techniques are also used to identify neurophysiological correlates of the neurological signs of the disease. 1. Introduction posterior cortical atrophy syndrome are other presentations of CBD [3, 4]. Corticobasal degeneration (CBD) is an uncommon, spo- CBD is a tauopathy (characterised by abnormal deposi- radic, neurodegenerative disease described for the ﬁrst time tion of the microtubule-associated protein tau), similar to by Rebeiz et al. . It can be associated with an extraordinary frontotemporal dementia and progressive supranuclear palsy variety of motor, sensory, behavioural, and cognitive symp- (PSP) . toms . The typical pathological ﬁndings in CBD include focal It is an asymmetrical parkinsonism aﬀecting a limb, asymmetric cortical atrophy, nigral degeneration, taupositive typically an arm: rigidity is the most common manifestation neuronal, and glial lesions in both gray and white matters . of the parkinsonian syndrome followed by bradykinesia, To achieve more accurate clinical diagnosis, neuropsy- gait disorder (postural instability and falls), and tremor; chological, electrophysiological, and imaging methods could asymmetrical limb dystonia is common as well. Other car- be applied to diﬀerentiate this disease from the other parkin- dinal signs include higher cortical dysfunctions such as sonism syndromes [3, 4]. apraxia (limb more common than orofacial, eyelid-opening). In comparison with other neurological diseases, the Dementia, progressive nonﬂuent aphasia, speech apraxia, symptoms of CBD are particularly diﬃcult to understand progressive-supranuclear-palsy- (PSP-) like syndrome and and the patients have considerable diﬃculties in describing 2 International Journal of Alzheimer’s Disease their experience. A better understanding of the disease may help clinicians to make diagnosis, providing patients with comprehensive information about prognosis and diﬃculties they will encounter during the course of the disease, improv- ing their quality of life as well as their careers. 2. Case Report A 71-year-old woman, primary school graduate, formerly farmhand, was referred to our Department of Neurology with left rigid-akinetic syndrome and cognitive dysfunction. She had no previous medical or family neurological history; she just reported in the previous months frequent falls and postural instability. Figure 1: Neuropsychological examination show emineglect She initially presented with slowly progressive dysarthria (Raven’s colored matrices and barrage test) and constructional and speech abnormalities two years before. Cognitive symp- apraxia (Rey-Osterrieth complex ﬁgure). toms included impairment of spoken-language production and attention/concentration deﬁcits. Several months later clumsiness and rigidity in her left upper limb appeared. Her main complaint was diﬃculty in using her left arm and hand, which gradually progressed. The limb became severely rigid and adopted a dystonic posture associated with pain and functional disability. Her clinical features slowly deteriorated and, 1 year later, involved also the left lower limb with gait disorder associated to postural instability and falls. Subsequently, the patient underwent brain MRI and 18F-ﬂuorodeoxyglucose-positron emission tomography (PET). Brain MRI showed moderate atrophy, more pronounced on the right side. She has been previously diagnosed as Parkinson’s Disease but with no response to levodopa or do- paminergic medications. Asymmetrical hypometabolism involving the right fron- tal cortex was also conﬁrmed by PET. Her clinical symptoms gradually progressed and, four months later, blepharospasm and mild dysphagia appeared. When she was admitted to our Neurological Cen- tre, the neurological examination revealed: blepharospasm; hypomimia and asmmetric bradykinesia; left upper-arm- Figure 2: Axial, T2-weighted and ﬂuid-attenuated inversion recov- ﬁxed-dystonia; spasticity in left lower limb and pyramidal ery (FLAIR), MRI images of the brain, demonstrating asymmetric cortical atrophy in the right hemisphere of the patient. signs (Babinski, Hoﬀmann, and grasp reﬂex); moderate disturbance of gait with short steps, tendency to drag her left leg, bradykinesia, and propulsion requiring assistance. We performed a complete neuropsychological exami- The diagnosis was based on the gradual onset of a nation (for more details see Table 1). Mini Mental State parkinsonian disorder associated with cortical dysfunctions Examination score was 23/30. The other tests revealed and other supportive features such as cognitive dysfunction, impairment in visuospatial abilities, severe visuospatial asymmetric atrophy on MRI imaging, and asymmetric neglect (Figure 1), constructional and ideomotor apraxia, hypoperfusiononPET. poor word ﬂuency with mild visual confrontation anomia, and nonverbal oral apraxia. Learning and memory were minimally aﬀected. 3. Discussion and Review of the Literature Brain MRI showed asymmetric cortical atrophy in the right frontotemporal cortex (Figure 2). 3.1. Clinical Presentation. Clinically CBD begins in the sixth, No abnormalities were found on EEG examination, even seventh, or eighth decade , with slight predilection for if, a deeper analysis showed a focal slow wave activity in the women [8, 9]. Typically the primary symptoms develop in right parietotemporal area. a profoundly asymmetric way, aﬀecting either one arm or, Related to clinical features, based upon diagnostic criteria less frequently, a leg, which appears to be rigid, dystonic, proposed by Boeve et al. , she has been diagnosed as akinetic, or apraxic. Clinical features include a series of “corticobasal degeneration (CBD)”. motor, cognitive and neuropsychiatric symptoms, that can International Journal of Alzheimer’s Disease 3 Table 1: Complete neuropsychological examination performed by the patient. Obtained score Cut-oﬀ Result Rey Auditory Learning Test Immediate recall 40 28,53 Normal Delayed recall 8 4,69 Normal Recognition recall 14/15, 4/30; accuracy % 95 92 Normal Digits forward 5 7±2Belownormal Digit backward 2 5±2Belownormal Corsi span Forward 3 7±2Belownormal Corsi span Backward 2 5±2Belownormal Rey-Osterrieth Complex ﬁgure 2,5; correct 9,5 9,46 Normal delayed recall Barrage Test Dx (26/30); Sx (5/30) 59 Below normal Time 95 >105 Deux Barrage 5/13, 22/67; accuracy % 57 95 Below normal Prolonged time of Time 210 >133 execution Rey-Osterrieth Complex ﬁgure Test 4,5; correct 7 28,87 Below normal Ideomotor Praxia 8,5 9 Below normal Buccofacial apraxia 10 9 Normal Raven’s colored Matrices 6; correct 10,5 18,96 Below normal Verbal Fluency Phonetc cues 10; correct 18,6 17,35 Normal Semantic cues 12; correct 14,3 10,3 Normal Naming (B.A.D.A.) 24 28 Below normal be explained by impairment of the cortical and subcortical executive impairment, calculation and visuospatial skills structures. impairment, whereas semantic and episodic memory may be Motor symptoms include progressive asymmetric rigid- spared . akinetic parkinsonism usually involving the upper limbs, Neuropsychiatric symptoms may include depression, without resting tremor , focal stimulus-sensitive or apathy, anxiety, irritability, disinhibition, delusions, and action myoclonus [4, 11], blepharospasm, speech abnormal- obsessive compulsive disorder . ities, gait disorder with postural instability and falls, and asymmetric limb dystonia, generally of the upper limbs, 3.2. Electrophysiology. Abnormalities on magnetoenceph- sometimes evolving towards the development of a dystonic alography , an exaggerated electromyographic-electro- clenched ﬁst [3, 12]. myographic (EMG-EMG) coherence , and an alteration Eye movements are usually preserved, although a delay in cortical excitability evaluated by means of transcranial in the initiation of saccades may occur, in absence of pursuit magnetic stimulation (TMS)  were noted in patients and optokinetic nystagmus impairment [13, 14]. with CBD . On the other hand, conventional elec- Involvement of higher cortical functions results in often troencephalography (EEG) may be normal when the ﬁrst symmetric ideomotor apraxia, ﬁrstly aﬀecting the limb, then, clinical symptoms appear, and often remains unchanged as as the disease progresses, eyelid-opening, tongue, lips. The the disease progresses. Nevertheless, an unilateral slowing alien-limb phenomenon, that is seen in 50% of the cases may be evident in some patients, which may occasionally , can be deﬁned as “a circumstance in which one of generalise to the whole cortex as the disease evolves [24, the patient’s hands behaves in a way which the patient ﬁnds 25]. In a study involving six patients, Vion-Dury and foreign, alien or at least uncooperative”; it commonly co- coworkers, using a quantitative standard EEG (EEGq) with occurs with cortical sensory loss [11, 16]. spectral analysis, found indeed the occurrence of several EEG Cognitive decline is a common feature of the disorder abnormalities (generally enhanced by hyperventilation or , occasionally the presenting feature of the disease . intermittent photic stimulation), such as an increase of slow The prominent characteristics are impairment of spoken- rhythms (delta or theta frequency range) and occasionally language production (typically nonﬂuent aphasia), frontal the occurrence of sharp waves . These abnormalities were 4 International Journal of Alzheimer’s Disease lateralised in ﬁve patients (more often after hyperventilation) asymmetrical hypoperfusion on SPECT and asymmetrical and were bilateral in one, conﬁrming the asymmetrical fea- hypometabolism on PET involving the parietal-frontal cor- tures of CBD . Moreover, Huang et al. showed that the tex and basal ganglia . EEG recordings with jerk-locked back average do not present Other neurodegenerative disorders sometimes overlap any jerk-locked cortical potentials . the CBD, making its clinical diagnosis diﬃcult. All criteria In CBD, the cortical sensory evoked potentials (SEPs) stress the combination of an akinetic-rigid syndrome with are not enlarged as in cortical reﬂex myoclonus, and back- apraxia, alien limb syndrome, and cortical sensory deﬁcits. averaged cortical potentials do not precede each myoclonic A universally recognized feature is the asymmetry of clinical jerk [26–28]. Clinical and imaging evidence suggests that the presentation, further corroborated by a contralateral asym- localized parietal cortical damage is a pivotal factor for the metrical atrophy on the structural and hypometabolism on absence of a giant SEP in these patients . An asymmetric the functional neuroimaging. alternation of inhibitory and excitatory balance at the level of cortical neurons leading to a particularly enhanced cortical 3.4. Neuropathology in Corticobasal Degeneration. Neuro- excitability may moreover play an important role in the pathologically CBD presents as asymmetrical focal atrophy generation of myoclonus [27, 28]. The loss of the inhibitory of the cerebral cortex focused on the peri-Rolandic posterior input from the somatosensory cortex to the relatively frontal and parietal cortex, especially the motor and sensory intact motor cortex, which results from the prominent areas . There is a relative sparing of temporal and occip- asymmetric parietal atrophy, may give rise to the asymmetric ital cortex, except in some forms presenting with dementia hyperexcitable motor cortex without giant SEP [30, 31], or primary progressive aphasia, which are characterised by a even-though the existence of an alternative hyper-excitable more symmetric and more severe involvement of the frontal thalamo-cortical pathway cannot be excluded . In eﬀect, and temporal lobes . motor cortex disinhibition has been clearly demonstrated Basal ganglia are also involved with substantial atrophy in in CBD by means of TMS applied in several paradigms the lateral two-thirds of the substantia nigra, and, to a lesser in diﬀerent neurophysiological studies [32–34]. By applying extent, of putamen, pallidum, thalamus, and hypothalamus single pulse-TMS, Lu and coworkers discovered a relatively . higher motor evoked potential (MEP) amplitude and a Histologically CBD is characterised by large pale bal- signiﬁcantly shorter cortical silent period in the aﬀected looned neurons (neuronal achromasia), with tau-positive hand of CBD patients . They therefore supposed that the cytoplasmatic inclusions and astrocytic plaques (annular relatively enlarged MEP may be explained postulating that clusters of tau-positive deposits within the distal processes an increased number of motorneurons are being recruited of astrocytes), typically distributed in atrophic cortices [5, 6, by the descending volleys from the motor cortex , 42]. while the shorter silent periods may reﬂect mainly defective Molecularly CBD is a taupathy, characterised by accu- inhibitory processes . The result from paired pulse-TMS mulation of abnormal ﬁlamentous inclusions of hyperphos- studies also supported the last hypothesis [22, 23, 35]; for phorylated tau-protein in neurons and glia, similarly to example, Frasson et al.  showed that, in patients with progressive supranuclear palsy (PSP), and some forms of CBD, paired magnetic stimuli delivered at short (inhibitory) frontotemporal dementia with parkinsonism (FTD) [5, 44]. interstimulus intervals (ISIs) invariably elicited enlarged This molecular overlap, especially with PSP is very argued, MEPs; moreover, asymmetric corticocortical disinhibition and whether they are the extremities of the spectrum of [22, 36], as well as asymmetric TMS maps organization a single disorder or two diﬀerent disorders with a similar [32, 37], has been observed in patients with CBD. genetic predisposition is not clear [3, 18]. In conclusion, several mechanisms could explain this abnormal motor cortical excitability, namely, loss of inhibitory neurons in the cortex or thalamus, eﬀect of 3.5. Diagnostic Criteria and Growing Importance of Cog- morphological changes in cortical neurons mainly in the nitive Symptoms. The symptoms can be gathered in four somatosensory cortices, disruption of some neuronal cir- categories: natural history and presentation, motor, sensory cuits, or the existence of alternative cortical-subcortical motor, and cognitive symptoms. The ﬁrst three categories pathways [33, 38]. Further electrophysiological studies are include characteristics which have been taken into account necessary to better circumstantiate these hypothesis. in almost all the previous diagnostic criteria. Up to 1994, dementia was an exclusion criterion of CBD; from 2003, 3.3. Imaging in Corticobasal Degeneration. Morphologic on the base of new criteria, cognitive impairments support imaging of the brain, although normal in the early phases of diagnosis, so the inclusion of the cognitive criteria reﬂects the disease, may demonstrate asymmetrical cortical atrophy, the growing recognition of the importance of cognitive in particular of the frontal and parietal lobe, more evident assessment in the diagnosis of CBD [3, 45]. contralaterally to the side most severely clinically aﬀected The core features of disease are insidious onset and [39, 40]. 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