A rare case of frontal degeneration
presenting with Gait Apraxia
Fulvio Da Re1, Giorgio Gelosa1, Debora Traficante1, Ildebrando Appollonio1, Valeria Isella1.
1Neurology
Section, S. Gerardo Hospital , Department of Surgery and Translational Medicine, University of Milano-Bicocca, Monza, Italy.
GAIT APRAXIA (G.A.)
• G.A. is defined by the inability to activate and maintain ambulation non attributable to primary motor, sensory, ataxic or psychiatric deficits.
• Most cases of G.A. described in the literature have involved frontal tumours or infarction in the territory of the anterior cerebral artery.
• Very few cases were degenerative: 2 patients with concomitant upper limb dyspraxia and medial frontal hypometabolism at 15O2 PET (Rossor et
al. 1999) and 5 with predominant frontal atrophy (Pick’s disease in 2, aspecific frontal degeneration in 1 and neurosyphilis in 2) (Della Sala et al.
2002).
CASE S.B.
Here we report on the case of S.B., a 75 yrs old right-handed woman.
•When she was first referred to the Neurologist, she had been complaining about progressive difficulties with walking (when going up a stair, in
particular) for about one year. The neurological examination showed only gait difficulties with no deficits in muscle strength or movement
coordination, nor extrapyramidal signs.
•Brain MRI showed moderate enlargement of frontal sulci.
•She was then evaluated again one year later, and she presented with memory loss and bilateral upper limb apraxia.
•Neuropsychological tests evidenced a strong closing-in behaviour, as part of a severe dependency syndrome, in addition to moderate memory,
attention and executive deficits.
•At her third assessment, three years after onset, she showed:
mild bilateral akinetic-rigid parkinsonism, with severe tendency to fall backward
severe ideomotor apraxia of upper and lower limbs
severe environmental dependency syndrome, with left hemisomatoagnosia and left hemiinattention
left hand levitation.
•[18F]FDG-PET analysed with SPM showed the following hypometabolic areas at a significance level of p < 0.01 (see Fig.1 and 2):
right frontal medial and orbital areas, and deep white matter (severe impairment)
right post-central and supramarginal gyri and deep white matter (severe impairment)
right inferior and middle temporal gyri (moderate impairment)
left post- and para-central regions (mild impairment).
CONCLUSIONS
•Patient S.B. represents one of the very few reports dealing with
frontal (and parietal) degeneration presenting as progressive
gait disorder
•An important difference in the anatomic substrate of the G.A.
compared with the cases described in the literature emerged
(i.e. the Supplementary Motor Area, usually involved, is spared
in our case), while the important involvement of the right parietal
lobe can explain the successive severe environmental
dependency syndrome (Lagarde et al. 2013)
•The full blown clinical presentation of this case suggests a
diagnosis of Cortico-Basal Degeneration (CBD), but with an
atypical onset characterised by G.A.. One of the two cases
described by Rossor et al. (1999) also had a neuropathological
diagnosis of CBD.
References:
-Della Sala et al. Gait apraxia after bilateral supplementary motor area lesion. J Neurol Neurosurg Psychiatry 2002; 72: 77-85.
-Lagarde et al. The clinical and anatomical heterogeneity of environmental dependency phenomena. J Neurol 2013; 260: 2262-2270.
-Rossor et al. Progressive frontal gait disturbance with atypical Alzheimer’s disease and corticobasal degeneration. J Neurol NeurosurgPsychiatry. 1999 Sep;67(3):345-52
180