Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination

Wenning, Gregor K.; Litvan, Irene; Jankovic, Joseph; Granata, Roberta; Mangone, Carlos A.; McKee, Ann C.; Poewe, Werner; Jellinger, K. A.; Chaudhuri, K. Ray; D’Olhaberriague, Luis; Pearce, R. K. B.

doi:10.1136/jnnp.64.2.184

Cited by 301 publications

(258 citation statements)

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Section: Resultsmentioning

confidence: 99%

“…Myoclonus in CBD may be superimposed on dystonia 18 and is most commonly described in the upper extremities, but can also be present in the face. 13,18 Descriptions include "focal myoclonus," 18 "stimulussensitive myoclonus," 13,16 and "action myoclonus." 16 Studies of myoclonus in CBS suggest that a very short latency may be characteristic, 29,30 but it is unclear if this is true in CBD.…”

Section: Resultsmentioning

confidence: 99%

“…Presentation was a mean of 3.0 (SD 1.9) years after symptom onset in one series. 18 When data were abstracted, features were considered as present or absent only if specifically described. While this approach carries the risk of overestimating the frequency of each feature, it was thought suitable due to the complexity of CBD and the varying degrees of detail in the retrospective data.…”

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confidence: 99%

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Criteria for the diagnosis of corticobasal degeneration

et al. 2013

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Current criteria for the clinical diagnosis of pathologically confirmed corticobasal degeneration (CBD) no longer reflect the expanding understanding of this disease and its clinicopathologic correlations. An international consortium of behavioral neurology, neuropsychology, and movement disorders specialists developed new criteria based on consensus and a systematic literature review. Clinical diagnoses (early or late) were identified for 267 nonoverlapping pathologically confirmed CBD cases from published reports and brain banks. Combined with consensus, 4 CBD phenotypes emerged: corticobasal syndrome (CBS), frontal behavioral-spatial syndrome (FBS), nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and progressive supranuclear palsy syndrome (PSPS). Clinical features of CBD cases were extracted from descriptions of 209 brain bank and published patients, providing a comprehensive description of CBD and correcting common misconceptions. Clinical CBD phenotypes and features were combined to create 2 sets of criteria: more specific clinical research criteria for probable CBD and broader criteria for possible CBD that are more inclusive but have a higher chance to detect other tau-based pathologies. Probable CBD criteria require insidious onset and gradual progression for at least 1 year, age at onset $50 years, no similar family history or known tau mutations, and a clinical phenotype of probable CBS or either FBS or naPPA with at least 1 CBS feature. The possible CBD category uses similar criteria but has no restrictions on age or family history, allows tau mutations, permits less rigorous phenotype fulfillment, and includes a PSPS phenotype.Future validation and refinement of the proposed criteria are needed. When first described, "corticodentatonigral degeneration with neuronal achromasia" was considered a distinct clinicopathologic entity, 1 eventually termed corticobasal degeneration (CBD). 2Clinicopathologic studies have since revealed that the originally described clinical features of CBD, now called corticobasal syndrome (CBS), are often due to other pathologies. As a pathologic diagnosis, CBD is characterized by widespread deposition of hyperphosphorylated 4-repeat tau in neurons and glia, the latter as astrocytic plaques, in specific topographic areas. 3 Despite various clinical diagnostic criteria (table e-1 on the Neurology ® Web site at www.neurology.org), 4-10 the pathology of CBD is predicted antemortem in only 25% to 56% of cases.11-17 Additionally, while these clinical criteria continue to be widely applied and cited, they reflect CBS alone and not the more recently recognized behavioral presentations of CBD.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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confidence: 99%

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Criteria for the diagnosis of corticobasal degeneration

et al. 2013

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“…Rigidity is common symptom not only for PD. However it can be noticed in 67-99% of patients with this disease [11,12]. Bradykinesia is characterised by gait disturbances and deceleration.…”

Section: Introductionmentioning

confidence: 99%

“…Bradykinesia is characterised by gait disturbances and deceleration. 71-99% of patients with PD refer to bradykinesia [12][13][14].…”

Section: Introductionmentioning

confidence: 99%

Parkinson’s disease: the most common diagnostic mistakes in Lithuania

et al. 2009

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AbstractParkinson’s disease (PD) belongs to group of neurodegenerative diseases. PD diagnosis is clinical, based on these signs: tremor, rigidity, bradykinesia, akinesia or hypokinesia. The aim of the work was to determine the frequency of separate clinical forms of Parkinson’s disease and difficulties at this disease diagnosis. After examining 267 patients, foreseen clinical criterion of Parkinson’s disease correspond 202 (44.0% persons) − 115 women and 87 men and for 65 patients diagnosis of PD was not confirmed, because they did not correspond with accepted criteria of Parkinson’s disease. While analyzing clinical peculiarities of disease we ascertained that rigidity-tremor form of disease prevailed for 152 (75.2%, 86 women and 66 men) patients. The rigidity form was more rare − 28 (13.9%, 13 women and 15 men). Not very frequent was a tremor form of disease -− 22 (10.9%, 16 women and 6 men) patients. According to data of our research, for almost one fourth of patients (65, 24.3%) the diagnosis of Parkinson’s disease was not confirmed after clinical examination. These patients did not correspond with clinical criteria of PD. The data of our research maintain that for almost one fourth (one fourth of what?) (24.3%) the diagnosis was incorrect. Although these patients did not correspond with accepted criteria of PD, they had been treated with antiparkinsonic medications. The PD diagnosis for them was determined only according to separate symptoms: tremor, gait alterations or memory deterioration and behaviour alternations. It must be noted, that symptoms of Wilson’s disease, MSA or brain infarction were estimated as PD. Examining patients at home, we ascertained that not all patients use prescribed L-dopa preparations. A part of patients or their relatives stopped using of this drug independently. We also made note of the fact that urinary incontinence manifested using dopamine agonist ropinirole. This side effect became significant problem for patient himself and for his relatives.

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