Case report

"Olivopontocerebellar atrophy with dementia syndrome"

Authors: Fitsioris X., Gravas I., Dermitzakis E., Nasiopoulou G. Theofanidis D., Georgiadis G.

Neurological Department, "PAPAGEORGIOU" Hospital, Thessaloniki, Greece


Background: Olivoponocerebellar atrophy (OPCA) is characterized by cerebellar cortex degeneration and atrophic lesions of the stem. Clinically the main shows include limb ataxia, bulbar ataxia, symp­tomatology from the degeneration of pyramidic system, parkinsonism and symptoms from the degeneration of cerebellum, pontile nuclei and cells of Purkinje. The disease appears in sporadic or hereditary form and may be accompanied with dementia, epilepsy, ophthalmoplegia, extrapyramidal signs and incontinence of urine.

Case report: A 65-year-old patient presented with the following symptomatology: memory loss since 2 years, walking instability with frequent falls, speech disturbance and difficulty of daily activities because of the motorial disturbances.
Clinical examination: 1) dystonic movements of the lips and the jaw, 2) contraction of the mesophryon musculi (frozen facial expression), 3) trunk ataxia and ataxia of the upper and lower extremities, 4) presence of the reflexes of conception and lactation, 5) reflexes of increasing tendinous of the extremities with clonus of the lower extremities, 6) paralysis of the up and down movements of gaze, 7) rest tremor of the upper extremities, bradycinesia and extrapyramidal hypertonia and 8) dysarthria.

Laboratory research: Hematological, biochemical and immunological control was normal, while the MRI brain arised important atrophy of vermis- cerebellar hemispheres and atrophy of pons and bulbar.

Neuropsychological examination: Severe impairment of higher brain functions that concern memory, learning ability of new information and the organization of those. Defective attention, perturbation of the visuo spatial functions and reduced speech flow.

Differential diagnosis: 1. Progressive supranuclear paralysis, 2.MSA (Multi- system- atrophy), 3. OPCA, 4. Other extra pyramidal syndrome

Discussion: The neurological examination added with the MRI findings (cerebellum and stem atrophy), the negative hereditary background and the neuropsychological control, drove to the diagnosis of the sporadic form OPCA with a dementia syndrome.
The patient was given L-dopa and anticholesterasic drugs and showed improvement as far as the motorial symptoms but not at the knowledgeable shortage.
Many degenerative diseases of the CNS have a clinical presentation that supports the differential diagnosis. On the other hand many times co- existed or added symptoms may present diagnostic problems. The imaging control and, as much as it can be possible, the genetic, can help to study them.

 

REFERENCES:

  1. Harpeet S. Dyggal, Cognitive affective psychosis syndrome in a patient with sporadic olivopontoce­rebellar atrophy, The Journal of Neuropsychiatry and clinical Neurosciences 17:260-262, May 2005
  2. Berciano J., Monton Fl., Maeso Mc, Ferrer I., [Man aged 49 years suffering from progressive clinical picture with palatal tremor, segmental myoclonous, ataxia, parkinsonism, amyotrophy, pyramidal signs, supranuclear ophthalmoplegia and cognitive decline], case report in Neurologia, 17(5): 237-43, May 2002.
  3. Adam and Victor?s, Principles of Neurology, seventh edition