© Borgis - Postępy Nauk Medycznych 10/2013, s. 691-693
Atypowy zespół parkinsonowski – pierwotne postępujące zatrzymanie chodu? Opis przypadku
Atypical parkinsonian syndrome – primary progressive freezing gait? Case report
Department of Neurology and Epileptology, Centre of Postgraduate Medical Education, Professor Witold Orłowski Independent Public Clinical Hospital, Warszawa
Head of Department: prof. Urszula Fiszer, MD, PhD
Rozpoznawanie atypowych zespołów parkinsonowskich jest problematyczne i może wymagać wielu lat. Podobnie jak w przypadku choroby Parkinsona, opiera się na wywiadzie, obrazie klinicznym, prospektywnej obserwacji chorego i wykluczeniu wtórnych zespołów parkinsonowskich. Praca przedstawia opis 59-letniego pacjenta z powoli postępującym od 7 lat opornym na leczenie dopaminergiczne atypowym zespołem parkinsonowskim, w którym przeważa spowolnienie ruchowe oraz wczesne zatrzymanie chodu, polegające na nagłym, przemijającym bloku ruchowym, związanym z niemożnością dalszego wykonywania kroków. Pacjent spełnia kryteria rozpoznania pierwotnego postępującego zatrzymania chodu (PPFG), które należy różnicować z innymi zespołami parkinsonowskimi, zwłaszcza z postępującym porażeniem ponadjądrowym. Kwestia, czy PPFG stanowi odrębną jednostkę nozologiczną, czy też jest początkowym etapem rozwoju innych chorób neurodegeneracyjnych pozostaje otwarta.
The diagnosis of atypical parkinsonian syndromes is problematic and may require many years. Similar to Parkinson's disease, it is based on the history of the disease, clinical evaluation, prospective patient’s observation and exclusion of secondary parkinsonims. This paper presents a report of a 59-years-old patient with slowly progressing levodopa unresponsive atypical parkinsonism of 7 years duration, with predominant hypokinesia and early freezing gait, that consist of sudden, episodic motor block due to inability to take steps. The patient fulfills the criteria of primary progressive freezing gait (PPFG), which should be differentiated from other parkinsonian syndromes, mainly progressive supranuclear palsy. The question whether PPFG is a distinct nosological entity or is an initial manifestation of other neurodegenerative disorders remains open.
Diagnostics of atypical parkinsonian syndromes, also called parkinsonian syndromes “plus”, is, in spite of modern diagnostic methods progress, a clinical challenge. It concerns rare, chronic neurodegenerative diseases of an unknown etiology, unknown causative treatment and often symptomatic treatment of little effectiveness. The terms atypical parkinsonian syndrome and parkinsonian syndrome “plus” are related to the possibility of parkinsonian symptoms occurrence other than typical for idiopathic Parkinson’s disease (PD) and additional, nonparkinsonian, symptoms occurrence. The diagnosis of these diseases, similar to PD, is based on the history of the disease, clinical evaluation, prospective patient’s observation and exclusion of secondary parkinsonims. The most difficult moment in differential diagnostics between PD and atypical parki- nsonian syndrome, as well as between particular atypical syndromes, is the most often the beginning of the disease. Clinical observation and readiness to verify the diagnosis in the course of the disease is important. In the typical course of PD some symptoms (e.g. escalated posture instability, falls, dementia, visual hallucinations not related with treatment and others) occur in the advanced stage but their early occurrence (in the first 3 years from the onset of the disease) is treated as atypical and should induce diagnostic alertness (1, 2).
One of the symptoms commonly observed in the advanced PD is a phenomenon of freezing of gait, also called motor block. It is a levodopa unresponsive parki nsonian feature, which may be the manifestation of the off time and is defined as a sudden, transient inability to continue walking, inability to “move the feet from the ground”. It occurs mainly with change in the walking direction, walking through narrow spaces, avoiding obstacles and so on. It is often accompanied by festination, postural instability, falls and bradykinesia/akinesia (difficulty in starting and impoverishment of movement/inability to start movement – witch concernes different types of movement, not only walking). The symptom yields by itself after a while but it may be made easier by sensorial associations or accessory objects (3).
Freezing gait may also occur in atypical parkinsonian syndromes, e.g. progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal degeneration (CBD) and in many secondary brain diseases: in vascular parkinsonism, normotensive hydrocephalus, in frontal lobe tumor and others (3, 4).
The following report is to present the case of the patient with slowly progressive parkinsonian syndrome, in which hypokinesia and early (in the second year of the disease) freezing gait are predominant.
A 59-year-old male with higher education running his own company stays under care of Outpatient’s Clinic of Department of Neurology and Epileptology of Centre of Postgraduate Medical Education in Warsaw due to gradually progressing gait disorder and slowness of movements. The disease started 7 years ago, in the 52 year of age, with a slight slowing in walking, speech and writing and impoverishment of mimics. At the beginning the patient was suspected of depression and treated with venlafaxine by the psychiatrist but occurrence of festination in the first year of the disease caused the diagnostics to widen. In the physical examination parkinsonian syndrome was then diagnosed with predominance of walking disturbance with periodic festination (especially in initiation of walking, while turn back and in limited space) and movement slowing with micrographia.
In the second year of the disease there was gait freezing and then postural instability as well as slightly stooped posture. In the brain magnetic resonance imaging there were discrete lesions of vascular origin in the subcortical white matter on both sides – with no progress in the following examinations. Wilson’s disease was excluded. In the next years there were more accompanying diseases: type II diabetes five years ago and arterial hypertension and hypercholesterolemia two years ago.
Since the parkinsonian syndrome diagnosis there were a few attempts of levodopa treatment (in the maximum daily dose 600 mg) also with piribedil and amantadine. Every time the patient stopped the treatment after a few weeks/months with no improvement. A year ago he was taking 2 mg/day of ropinirole and 20 mg/day of paroxetine for a few months but with no satisfactory effect. The patient systematically undergoes motor and orthophonic rehabilitation.
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Płatny dostęp do wszystkich zasobów Czytelni Medycznej
1. Gibb WR, Lees AJ: The relevance of the Lewy body to the pathogenesis of idiopathic Parkinson’s disease. J Neurol Neurosurg Psychiatry 1988; 51: 745-752.
2. Gelb DJ, Oliver E, Gilman S: Diagnostic criteria for Parkinson’s disease. Arch Neurol 1999; 56: 33-38.
3. Fahn S, Elton RL: Unified Parkinson’s Disease Rating Scale. [In:] Fahn S, Marsden CD, Calne DB et al. (eds.): Recent Developments in Parkinson’s Disease. Mac Millan Healthcare Information, Plorhan Park, vol. 2, New York 1987: 293-304.
4. Giladi N, Kao R, Fahn S: Freezing phenomenon in patients with parkinsonian syndromes. Mov Disord 1997; 4: 302-305.
5. Achiron A, Ziv I, Goren M et al.: Primary progressive freezing gait. Mov Disord 1993; 8: 293-297.
6. Atchison PR, Thompson PD, Frackowiak RSJ, Mardsen CD: The syndrome of gait ignition failure: a report of six cases. Mov Disord 1993; 8: 285-292.
7. Fabre N, Bretel C, Sabatini U et al.: Normal frontal perfusion in patients with frozen gait. Mov Disord 1998; 13: 677-683.
8. Factor SA, Jennings DJ, Molho ES, Marek KL: The natural history of the syndrome of primary progressive freezing gait. Arch Neurol 2002; 59: 1778-1783.
9. Jennings DL, Factor SA, Molho ES et al.: Primary progressive freezing gait disorder (PPFG): a neuroimaging study to evaluate dopamine transporter density (abstract). Neurology 2000; 54 (suppl. 3): A191.
10. Feve AP, Fenelon G, Wallays C et al.: Axial motor disturbances after hypoxic lesions of the globus pallidus. Mov Disord 1993; 8: 321-326.
11. Shine JM, Matar E, Ward PB et al.: Exploring the cortical and subcortical functional magnetic resonance imaging changes associated with freezing in Parkinson’s disease. Brain 2013; 134: 1204-1215.
12. Ostrem JL, Christine CW, Glass GA et al.: Pedunculopontine nucleus deep brain stimulation in a patient with primary progressive freezing gait disorder. Stereotact Funct Neurosurg 2010; 88(1): 51-55.
13. Wilcox RA, Cole MH, Wong D et al.: Pedunculopontine nucleus deep brain stimulation produces sustained improvement in primary progressive freezing of gait. J Neurol Neurosurg Psychiatry 2011; 82(11): 1256-1259.
14. Thevathasan W, Cole MH, Graepel CL et al.: A spatiotemporal analysis of gait freezing and the impact of pedunculopontine nucleus stimulation. Brain 2012; 135: 1446-1454.
15. Zuniga C, Lester J, Cersosimo MG et al.: Treatment of primary progressive freezing of gait with high doses of selegiline. Clin Neuropharmacol 2006; 29(10): 20-21.
16. Coria F, Cozar-Santiago Mdel P: Rasagaline improves freezing in patient with primary progressive freezing gait. Mov Disord 2008; 23(3): 449-451.
17. Morgante F, Fasano A: Improvement with duloxetine in primary progressive freezing gait. Neurology 2010; 75(23): 2130-2132.
18. Quinn NP, Luthert P, Honavar M, Mardsen CD: Pure akinesia due to Lewy body Parkinson’s disease: a case with pathology. Mov Disord 1989; 4: 85-89.
19. Factor SA, Higgins DS, Qian J: Primary progressive freezing gait: a syndrome with many causes. Neurology 2006; 66(3): 411-414.
20. Imai H, Narabayashi H, Sakata E: “Pure akinesia” and the later added supranuclear ophtalmoplegia. Adv Neurol 1986; 45: 207-212.
21. Riley DE, Fogt N, Leigh RJ: The syndrome of “pure akinesia” and its relationship to progressive supranuclear palsy. Neurology 1994; 44: 1025-1059.
22. Hee K, Park MD, Jae S et al.: Functional brain imaging in pure akinesia with gait freezing: [18F]FDG PET and [18F]FP-CIT PET analyses. Mov Disord 2009; 24(9): 237-245.
23. Compta Y, Valldeoriola F, Tolosa E et al.: Long lasting pure freezing of gait preceding progressive supranuclear palsy: a clinicopathological study. Mov Disord 2007; 22(13): 1954-1958.