Cortical-basal Ganglionic Degeneration Research Articles

ID 211 – Can rTMS be Useful in the symptomatic treatment of cortical basal ganglionic degeneration?

Objective Cortical basal ganglionic degeneration (CBGD) is a rare neurodegenerative disease affecting the cortex and basal ganglia asymmetrically. The symptoms of the disease are generally resistant to therapy. In this report, we present the effects of repetitive transcranial magnetic stimulation (rTMS) on the decapacitating apraxia in two patients with CBGD. Methods Patient 1 is a 55 year-old man and Patient 2 is an 80 year-old woman. Apraxia was remarkably more prominent on the right side in both of them. 1 Hz and teta-burst stimulation was applied on the less affected motor cortex (M1) while 10 Hz stimulation was applied on the more affected M1. Responses to rTMS sessions were evaluated with nine-hole peg test (9-HPT), Purdue-Pegboard test and apraxia tests. Results Periods of recovery from apraxia were observed following certain rTMS sessions on the background of the natural progression of the disease. Conclusions Variable motor cortical excitability changes are seen in CBGD. Temporary recovery of the symptoms may be provided by rTMS modulation of the equilibrium of cortical excitabilities between the two hemispheres.

Apraxias in neurodegenerative dementias.

Background:Apraxia is a state of inability to carry out a learned motor act in the absence of motor, sensory or cerebellar defect on command processed through the Praxis circuit. Breakdown in default networking is one of the early dysfunction in cortical dementias and result in perplexity, awkwardness, omission, substitution errors, toying behavior and unrecognizable gestures in response to command with voluntary reflex dissociation where, when unobserved patient will carry out reflex movements normally. Awareness into the organicity of these phenomenas will help in early diagnosis, which will help in initiating appropriate treatment and slowing down the progression of the disease.Aims and Objectives:The aim was to look for the various kinds of apraxias in patients with dementia using appropriate simple tests.Patients and Methods:Three hundred patients satisfying Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition criteria for dementia were evaluated in detail with mandatory investigations for dementia followed by testing for ideational, ideomotor, limb-kinetic, buccopharyngeal, dressing apraxia, constructional apraxia and gait apraxias in addition to recording of rare apraxias when present.Results:Alzheimer's disease showed maximum association with apraxias in all the phases of the disease ideational, ideomotor, dressing and constructional apraxias early and buccopharyngeal and gait apraxia late. Frontotemporal lobe dementia showed buccopharyngeal and gait apraxias late into the disease. Cortical basal ganglionic degeneration showed limb apraxias and diffuse Lewy body disease showed more agnosias and less apraxias common apraxias seen was Ideational and Ideomotor.Conclusion:Recognition of the apraxias help in establishing organicity, categorization, caregiver education, early strategies for treatment, avoiding anti-psychotics and introducing disease modifying pharmacotherapeutic agents and also prognosticating.

MRI Eye-of-the-Tiger Sign in Organophosphate Poisoning

We describe the eye-of-the-tiger sign on magnetic resonance imaging (MRI) of the brain in a 40-year-old man presenting with extra pyramidal symptoms like chorea, flexion neck dystonia, tongue tremors, dysarthria and postural instability as the sequelae of organophosphorus poisoning six months previously. This typical radiological sign has been described in extrapyramidal parkinsonian disorders including cortical-basal ganglionic degeneration, early onset levodopa-responsive parkinsonism and Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy) but hitherto has not been reported in insecticide poisoning. T2-weighted scans showed low signal intensity surrounding a central region of high signal intensity in the anteromedial globus pallidus (gliosis), producing an eye-of-the-tiger appearance with the central hyperintense signal intensity better appreciated in T2W and fluid attenuated inversion recovery (FLAIR) sequences.

Clinical manifestations of Parkinson's disease and parkinsonism.

The most common disorder in a patient presenting to a movement disorder clinic will be parkinsonism. The challenge is to provide the patient with the most accurate diagnosis and prognosis possible. The assumption at the time of initial presentation of the clinical diagnosis of Parkinson's disease is often wrong (20-25%). Waiting to see the pattern of progression, and response to medication provides invaluable additional information. This manuscript summarizes the clinical manifestations of Parkinson's disease and the main akinetic-rigid syndromes (progressive supranuclear palsy, multiple system atrophy, cortical-basal ganglionic degeneration, and dementia with Lewy bodies) that make up the differential diagnosis.

Lumping and splitting the parkinson plus syndromes: Dementia with lewy bodies, multiple system atrophy, progressive supranuclear palsy, and cortical-basal ganglionic degeneration

The atypical parkinsonian or Parkinson Plus syndromes are often difficult to differentiate from Parkinson's disease and each other. In this article, the clinicopathological characteristics of dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy, and cortical-basal ganglionic degeneration are discussed. These disorders, although clinically distinct, may have more similarities than previously thought, based on modern immunocytochemical techniques and new genetic findings. These intriguing interconnections at a basic molecular level have provided the scientific rationale for lumping these diseases into two groups, the synucleinopathies and the tauopathies.

Cortical-basal ganglionic degeneration: a clinical, functional and cognitive evaluation (1-year follow-up)

We decided to evaluate a patient who was diagnosed with cortical-basal ganglionic degeneration from a clinical, instrumental and neuropsychological perspective. Our aim was to employ a new instrumental tool, functional magnetic resonance, in order to evaluate his cortical damage. We then followed the pathological course for 1 year and tested the patient again: we discuss the results of our evaluation, having an overview of the literature on the topic. In particular, we focused our attention on his apraxia, trying to suggest a dynamic and anatomical model to guarantee a possible explanation of his behavior.

Dementia as the most common presentation of cortical-basal ganglionic degeneration.

To evaluate the clinical presentations and dominant symptoms of patients with postmortem proven cortical-basal ganglionic degeneration (CBGD) from one neuropathology center. CBGD is a rare but increasingly recognized condition with clinical and pathologic features that continue to evolve. Attempts have been made to develop clinical criteria to enhance the specificity of diagnosis, but it is not clear what proportion of patients harboring CBGD disease present in the "classical" fashion versus other presentations. Previous large-case series that emphasize a parietal/perceptual-motor presentation may be biased because the cases mainly originate from movement disorder centers. Thirteen cases of pathologically confirmed CBGD with sufficient clinical data were identified from a single neuropathology center between 1981 and 1996. Before death, only 4 of the 13 patients had a clinical diagnosis of CBGD, 6 had a clinical diagnosis of Alzheimer's disease (AD), 1 had AD and parkinsonism, and 2 had an atypical dementia of the frontotemporal type. Nine of 13 cases had early dementia. Dementia was the most common presentation of CBGD in this study. Despite the best efforts to define criteria to enhance the specificity of a diagnosis of CBGD, it is becoming clear that the clinical syndrome that accompanies this disease is quite varied. Unfortunately, patients fulfilling classical diagnostic criteria may represent a minority of those with this pathologic diagnosis.

The eye of the tiger sign in cortical-basal ganglionic degeneration.

The eye of the tiger sign in cortical-basal ganglionic degeneration.

2-30-01 Clinicopathologic heterogeneity in clinically- and/or pathologically-diagnosed cortical-basal ganglionic degeneration

2-30-01 Clinicopathologic heterogeneity in clinically- and/or pathologically-diagnosed cortical-basal ganglionic degeneration

Cortical Degeneration in Progressive Supranuclear Palsy. A Comparison with Cortical-Basal Ganglionic Degeneration

We report 3 patients with progressive supranuclear palsy (PSP) who developed limb apraxia, focal dystonia, and arm levitation late in the course of the disease. Neuropathological examination revealed cortical degeneration in addition to the characteristic pathological findings of PSP. Semiquantitative comparative histological and immunohistological studies of the neocortex of these patients as well as 5 cases of classical PSP and 4 cases of cortical-basal ganglionic degeneration (CBGD) revealed a distinctive form of cortical degeneration in PSP. The cortical degeneration was often circumscribed and confined to premotor and motor cortex. It was characterized by neuronal loss and gliosis. Swollen neurons were only rarely observed in neocortex of PSP cases in contrast with CBGD, where they were abundant. Neuronal and glial tau as well as tau immunoreactive threads were seen in both PSP and CBGD, but were more abundant in CBGD. The appearance of tau reactive astrocytes also differed in both disorders; tufted astrocytes were seen exclusively in PSP, while typical annular astrocytic plaques were confined to CBGD. These observations indicate that cortical degeneration occurs in PSP and may be associated with atypical clinical manifestations that lead to diagnostic difficulties.

Dystonia in progressive supranuclear palsy.

OBJECTIVES: To document the nature, distribution, and frequency of dystonic symptoms in progressive supranuclear palsy (PSP). METHODS: Charts and videotapes of all clinically diagnosed patients with PSP seen between 1983...

Neuropsychological functioning in cortical-basal ganglionic degeneration

Patients with cortical-basal ganglionic degeneration (CBGD) display prominent rigidity and apraxia, exhibit an asymmetric onset of symptoms, and may show other symptoms including abnormal saccadic eye movements, the "alien limb" sign, limb dystonia, and myoclonus. We compared the neuropsychological test performances of 21 CBGD patients with 21 Alzheimer's disease (AD) patients displaying no extrapyramidal symptoms and with 12 ADA patients who did show such symptoms. Groups were matched for age, educational level, and overall severity of dementia. Since the cognitive deficit was mild in most CBGD patients, most AD patients included in this study were also only mildly demented. The CBGD patients performed significantly better than the AD patients on test of immediate and delayed recall of verbal material; whereas the AD patients (with or without extrapyramidal symptoms) performed better on tests of praxis, finger tapping speed, and motor programming. The CBGD and AD groups all displayed prominent deficits on tests of sustained attention/mental control and verbal fluency, and exhibited mild deficits on confrontation naming. The CBGD patients endorsed significantly more depressive symptoms on the Geriatric Depression Scale.

Alzheimer's disease in a case of cortical basal ganglionic degeneration with severe dementia.

Alzheimer's disease in a case of cortical basal ganglionic degeneration with severe dementia.

Stimulus-sensitive myoclonus in akinetic-rigid syndromes.

The cutaneous reflexes of upper limb muscles were studied in five patients with Parkinson's disease and 10 patients with stimulus-sensitive myoclonus associated with akinetic-rigid syndromes. The middle finger was stimulated with ring electrodes and rectified electromyographs were averaged from seven upper limb muscles and orbicularis oculi. Responses from subjects with Parkinson's disease without stimulus-sensitive myoclonus were similar to those of normal subjects. The responses from patients with stimulus-sensitive myoclonus associated with Parkinson's disease or multiple system atrophy had the normal pattern except that a long latency facilitation, which is present in normal subjects and known as E2, was greatly exaggerated. Patients with stimulus-sensitive myoclonus associated with cortical-basal ganglionic degeneration had a completely different pattern of responses. There was synchronous activation of all recorded upper limb muscles with latencies substantially shorter than those of the long latency facilitation (E2) in normal subjects. Cutaneous reflex testing may therefore be useful in the differentiation of akinetic-rigid syndromes.

Cortical‐basal ganglionic degeneration

We report our experience with 15 patients believed to have cortical-basal ganglionic degeneration. The clinical picture is distinctive, comprising features referable to both cortical and basal ganglionic dysfunction. Characteristic manifestations include cortical sensory loss, focal reflex myoclonus, "alien limb" phenomena, apraxia, rigidity and akinesia, a postural-action tremor, limb dystonia, hyperreflexia, and postural instability. The asymmetry of symptoms and signs is often striking. Brain imaging may demonstrate greater abnormalities contralateral to the more affected side. Postmortem studies in 2 patients revealed the characteristic pathologic features of swollen, poorly staining (achromatic) neurons and degeneration of cerebral cortex and substantia nigra. Biochemical analysis of 1 brain showed a severe, diffuse loss of dopamine in the striatum. This condition is more frequent than previously believed, and the diagnosis can be predicted during life on the basis of clinical findings. However, as with other "degenerative" diseases of the nervous system, a definitive diagnosis of cortical-basal ganglionic degeneration requires confirmation by autopsy.

Gonorrh a; Endometritis; Oophoritis; Ovarian Neuralgia; Lateral Flexion

<b><i>Article abstract</i></b> Spontaneous arm levitation (SAL) is well-recognized in cortical–basal ganglionic degeneration, but not in other neurodegenerative diseases. We report six cases of progressive supranuclear palsy (PSP) with SAL. Pathologic examination in four revealed the classic features of PSP with additional areas of focal cortical degeneration. We conclude that SAL can occur in PSP and its occurrence may be a marker of cortical degeneration.