Cortico-basal degeneration

The cortico-basal degeneration is a neurodégénérative Maladie relatively rare but more and more frequently described, concerning mainly the subcortical cerebral areas, then cortical and presenting typically an asymmetrical driving dysfunction as well as cognitive disorders. The clinical diagnosis is difficult because of the variability of the symptoms and the doctor can help himself of the imagery. The treatment still is very limited.

History

The cortico-basal degeneration was described for the permière time in 1967-1968 per Rebeiz, Kolodny and Richardson, who reports a syndrome including/understanding a rigidity and an asymmetrical akinesy, a dystonie of the upper limbs, an apraxia, myoclonies and an insanity.

Clinical presentation

At the beginning of the disease, classically, one finds an asymmetrical driving dysfunction on the level of the upper limbs, a cortical sensory loss and an apraxia without intellectual deterioration. The clinical picture can often resemble that of a progressive Paralysie supranucléaire (PCP or disease of Steele-Richardson-Olszewski), with a resistance to the L-dopa, a tremor of attitude, a pseudo-bulbar syndrome with dysarthrie, dysphagie, turbid oculomoteurs as well as an insanity. Intellectual deterioration settles then, it is related to the attack of the associative cortex, and in particular of the frontal cortex.

Certain authors proposed like criterion diagnosis, the presence of 3 of the following symptoms:

Bradykinésie and rigidity resistant to the L-DOPA
Syndrome of the “foreign member”
cortical sensory Signs
focal Dystonie with the members
Tremor of attention
Myoclonie

The criteria of exclusion (their presence excludes the diagnosis) are: an good answer with the L-DOPA (as in the Parkinson's disease), a tremor of rest, early attacks of the vertical glance (as in the progressive paralysis supranucléaire), of the severe attacks of the autonomous nervous system (as in the multisystemic atrophy), and all anomalies with the examinations of imagery which could explain the symptoms.

It should be noted that the dementia praecox is now recognized as being one of the initial symptoms of the cortico-basal degeneration.

Differential diagnosis

The diagnosis is difficult, especially at the initial stage, and even in the experienced doctors are mistaken in more than 50% of the cases. The cortico-basal degeneration can be difficult to differentiate from the PCP or a multisystemic atrophy. When the cortical signs appear, the diagnosis is facilitated. The principal differential diagnoses are:

Parkinson's disease
progressive Paralysis supranucléaire (PCP or disease of Steele-Richardson-Olszewski)
multisystemic Atrophy
Disease of Pick

Imagery

IRM watch a parietal atrophy and a widening of the ventricles side.

Anatomopathology

There is a degeneration neurofibrillaire in the subcortical cores and the cortex, especially in the frontal areas. One finds a neuronal loss, a gliose and an accumulation of proteins tau in the astrocytes. One notes also the presence of chromatolytic cells (or cells of Pick). The most touched zones are the frontal and parietal areas.

The evolution is slow but fatal at the end of 6 years on average.

Assumption of responsibility

The L-dopa, treatment of reference for the Parkinson's disease, is not effective on this disease and there is not, to date, of specific treatment making it possible to slow down the evolution of the disease.

See too

Internal bonds

External bonds

cortico-basal Degeneration on http://www.orpha.net

References

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