scholarly journals An Unusual Cause of Dementia: Essential Diagnostic Elements of Corticobasal Degeneration—A Case Report and Review of the Literature

2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
F. Mastrolilli ◽  
A. Benvenga ◽  
L. Di Biase ◽  
F. Giambattistelli ◽  
L. Trotta ◽  
...  

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.


BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Dallah Yoo ◽  
Sung-Hye Park ◽  
Sungwook Yu ◽  
Tae-Beom Ahn

Abstract Background Neurodegenerative disorders are characterized by insidious progression with poorly-delineated long latent period. Antecedent clinical insult could rarely unmask latent neurodegenerative disorders. Here, we report an autopsy-proven case of corticobasal degeneration which was preceded by a lacunar infarction. Case presentation A 58-year-old man presented with acute ataxia associated with a lacunar infarction in the right paramedian pons. His ataxia persisted with additional progressive gait difficulty and left arm clumsiness. Six months later, a follow-up neurological examination showed asymmetrical bradykinesia, apraxia, dystonic posturing, postural instability, and mild ataxia of the left limbs. Cognitive examination revealed frontal executive dysfunction and visuospatial difficulties. Dopamine transporter imaging scan demonstrated bilateral reduced uptakes in mid-to-posterior putamen, more prominent on the right side. Levodopa-unresponsive parkinsonism, asymmetric limb dystonia, and ideomotor apraxia became more conspicuous, while limb ataxia gradually vanished. The patient became unable to walk without assistance after 1 year, and died 4 years after the symptom onset. Autopsy findings showed frontoparietal cortical atrophy, ballooned neurons, and phosphorylated tau-positive astrocytic plaques and neuropil threads with gliosis and neuronal loss, confirming the corticobasal degeneration. Conclusions The case illustrates that precedent clinical events such as stroke might tip a patient with subclinical CBS into overt clinical manifestations.



Neurology ◽  
2016 ◽  
Vol 88 (2) ◽  
pp. 160-163 ◽  
Author(s):  
Ilaria Zivi ◽  
Eugenio Bertelli ◽  
Giacinta Bilotti ◽  
Ignazio Alessandro Clemente ◽  
Leopold Saltuari ◽  
...  

Objective:To describe a rare sign of unilateral brain injury as a form of unwanted blink-associated contralateral eccentric saccades.Methods:A 62-year-old patient who underwent an ischemic stroke affecting the entire right middle cerebral artery territory came to our attention 1 year after stroke, manifesting with transient contralateral conjugate gaze deviations associated with spontaneous blinking. We complemented the regular neurologic evaluation with brain MRI, study of evoked potentials, electroneurography of the facial nerve, and infrared video-oculoscopy.Results:The patient had left-sided hemiparesis, hypoesthesia, hemianopia, and hemispatial neglect. He also showed the occurrence of a rapid leftward conjugate deviation of the eyes, followed by a corrective movement to the primary ocular position. MRI showed a wide malacic area spanning the right frontal, temporal, and parietal cortical and subcortical regions, with signs of wallerian degeneration of the descending right corticospinal tract. Motor and somatosensory evoked potentials were centrally altered on the right side. Electroneurography of the facial nerves was normal. Infrared video-oculoscopy indicated persistence of the same blink-related saccades even in darkness.Conclusions:It is known that unilateral cerebral lesions may manifest with a contralateral conjugate gaze deviation evoked by closure of the lids. This sign, known as spasticity of conjugate gaze, may be due to the suppression of the fixation reflex. In our case, the persistence of this sign in the darkness allowed us to exclude this diagnosis. We hypothesized that the blink-related neural pathways may improperly activate the oculomotor circuitry at both the cortical and subcortical levels.



Author(s):  
Cengiz Tataroglu ◽  
Ahmet Genc ◽  
Egemen Idiman ◽  
Raif Cakmur ◽  
Fethi Idiman

AbstractBackground:Long latency reflexes (LLR) include afferent sensory, efferent motor and central transcortical pathways. It is supposed that the cortical relay time (CRT) reflects the conduction of central transcortical loop of LLR. Recently, evidence related to the cortical involvement in multiple sclerosis (MS) has been reported in some studies. Our aim was to investigate the CRT alterations in patients with MS.Methods:Upper extremity motor evoked potentials (MEP), somatosensory evoked potentials (SEP) and LLR were tested in 28 patients with MS and control subjects (n=22). The patients with MS were classified according to the clinical form (relapsing-remitting [R-R] and progressive groups). The MS patients with secondary progressive and primary progressive forms were considered as the “progressive” group. CRT for LLR was calculated by subtracting the peak latency of somatosensory evoked potentials (SEP) and that of motor evoked potentials (MEP) by transcranial magnetic stimulation from the onset latency of the second component of LLR (LLR2) (CRT = LLR2 – [MEP latency + N20 latency])Results:Cortical relay time was calculated as 7.4 ± 0.9 ms in control subjects. Cortical relay time was prolonged in patients with MS (11.2 ± 2.9 ms) (p<0.0001). The latencies of LLR, MEP and SEP were also prolonged in patients with MS. Cortical relay time was not correlated with disease severity and clinical form in contrast to other tests.Conclusions:Our findings suggested that CRT can be a valuable electrophysiological tool in patients with MS. Involvement of extracortical neural circuits between sensory and motor cortices or cortical involvement due to MS may cause these findings.



2017 ◽  
Vol 29 (11) ◽  
pp. 1918-1931 ◽  
Author(s):  
Nicolas A. McNair ◽  
Ashleigh D. Behrens ◽  
Irina M. Harris

Previous behavioral and neuroimaging studies have suggested that the motor properties associated with graspable objects may be automatically accessed when people passively view these objects. We directly tested this by measuring the excitability of the motor pathway when participants viewed pictures of graspable objects that were presented during the attentional blink (AB), when items frequently go undetected. Participants had to identify two briefly presented objects separated by either a short or long SOA. Motor-evoked potentials were measured from the right hand in response to a single TMS pulse delivered over the left primary motor cortex 250 msec after the onset of the second target. Behavioral results showed poorer identification of objects at short SOA compared with long SOA, consistent with an AB, which did not differ between graspable and nongraspable objects. However, motor-evoked potentials measured during the AB were significantly higher for graspable objects than for nongraspable objects, irrespective of whether the object was successfully identified or undetected. This provides direct evidence that the motor system is automatically activated during visual processing of objects that afford a motor action.



2019 ◽  
Vol 14 (6) ◽  
pp. 1025
Author(s):  
Raffaele Nardone ◽  
PatrickB Langthaler ◽  
Andrea Orioli ◽  
Viviana Versace ◽  
GiudittaIlaria Scarano ◽  
...  


CoDAS ◽  
2013 ◽  
Vol 25 (5) ◽  
pp. 407-412 ◽  
Author(s):  
Renata Parente de Almeida ◽  
Carla Gentile Matas

PURPOSE: To characterize the findings obtained in the long latency auditory evoked potentials in malnourished children, as well as to compare them to the results obtained for children at the same age who present typical development. METHODS: Sixty-five children aged between 7 and 12 years old, of both genders, were evaluated. The control group consisted of 34 children with normal development and the study group consisted of 31 children diagnosed with malnutrition. All children underwent conventional pure tone audiometry, acoustic impedance measurement, dichotic digit test and long latency auditory evoked potentials. RESULTS: The study group presented a statistically significant difference for latencies of the components P1, N1 and P300. The latencies of these components were higher than those of the control group. Concerning the types of alterations found in the study group, increased latency for P1 and P300 and the absence of response in N1 were predominant. The control group showed a statistically significant difference in the amplitude of P300 when compared to the right and left ears. CONCLUSION: Malnourished children present with changes in the long latency auditory evoked potentials, suggesting a deficit in central auditory pathways and alterations in the processing of acoustic information.



2003 ◽  
Vol 114 (10) ◽  
pp. 1808-1818 ◽  
Author(s):  
Y Vandermeeren ◽  
E Bastings ◽  
L Fadiga ◽  
E Olivier


2018 ◽  
Author(s):  
Lawrence P. Behmer ◽  
Kelly J. Jantzen ◽  
Matthew Crump

Past, present, and future actions must be regulated online to produce sequences of actions, but the regulation process is not well understood because of measurement limitations. We provide the first direct tests of the parallel action regulation hypothesis during sequencing in humans. We used transcranial magnetic stimulation to probe the level of excitation for flexion of the right index finger during typing. Motor evoked potentials (MEPs) were recorded at the onset of typing 5-letter words and nonwords. A single letter typed by the right index finger varied across letter positions 1 to 5. MEP amplitude was largest for the upcoming action in the second position and decreased monotonically across future serial positions, suggesting a serial inhibition process regulates all future actions in parallel during sequencing. This is the most direct human evidence to date corroborating models of sequence production that assume parallel regulation of actions.



Author(s):  
Janet Brenya ◽  
Katherine Chavarria ◽  
Elizabeth Murray ◽  
Karen Kelly ◽  
Anjel Friest ◽  
...  

Only by understanding the ability to take third-person perspective can we begin to elucidate the neural processes responsible for one&rsquo;s inimitable conscious experience. The current study examined differences in hemispheric laterality during a first-person perspective (1PP) and third-person perspective (3PP) taking task, when using Transcranial Magnetic Stimulation (TMS). Participants were asked to take either the 1PP or 3PP when identifying the number of spheres in a virtual scene. During this task, single-pulse TMS was delivered to the motor cortex of both the left and right hemispheres of 10 healthy volunteers. Measures of TMS-induced motor-evoked potentials (MEPs) of the contralateral abductor pollicis brevis (APB) were employed as an indicator of lateralized cortical activation. The data suggest that the right hemisphere is more important in discriminating between 1PP and 3PP. These data add a novel method for determining perspective taking and add to the literature supporting the role of the right hemisphere in meta representation.



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