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  • Print publication year: 2011
  • Online publication date: May 2011

Case 39 - Young woman with lateralized motor symptoms

Summary

This chapter presents a case study of a 69-year-old right-handed man who was presented in June 2006 with a 1-year history of progressive word finding difficulties and mild phono-articulatory problems. It provides the general history, family history, examination, initial diagnosis and follow-up data of the patient. In a simple delayed recall test, he was able to remember five out of ten figures, which is considered slightly impaired. Based on the overall clinical, neuropsychological, language, and neuroimaging data, a diagnosis of Progressive Non-Fluent Aphasia (PNFA) was made. Duloxetine was started for the depressive symptoms with good clinical response. Primary progressive aphasia (PPA) is a clinical syndrome characterized by progressive dissolution of language with relative preservation of other cognitive abilities for at least 1 to 2 years. Recent studies have classified the clinical presentations of PPA into three main subtypes: agrammatic, logopenic, and semantic variant.

References and suggested reading

AliZS, Van Der VoornJP, PowersJM (2007). A comparative morphologic analysis of adult onset leukodystrophy with neuroaxonal spheroids and pigmented glia–a role for oxidative damage. Journal of Neuropathology and Experimental Neurology, 66(7), 660–72. doi:10.1097/nen.0b013e3180986247
BabaY, GhettiB, BakerMC, UittiRJ, HuttonML, YamaguchiK, BirdT, LinW, DeLuciaMW, DicksonDW, WszolekZK (2006). Hereditary diffuse leukoencephalopathy with spheroids: Clinical, pathologic and genetic studies of a new kindred. Acta Neuropathologica, 111(4), 300–11. doi:10.1007/s00401-006-0046-z
ChuiHC, VictoroffJI, MargolinD, JagustW, ShankleR, KatzmanR (1992). Criteria for the diagnosis of ischemic vascular dementia proposed by the state of california alzheimer's disease diagnostic and treatment centers. Neurology, 42(3 Pt 1), 473–80.
CostelloDJ, EichlerAF, EichlerFS (2009). Leukodystrophies: Classification, diagnosis, and treatment. The Neurologist, 15(6), 319–28. doi:10.1097/NRL.0b013e3181b287c8
DoodyRS, PavlikV, MassmanP, RountreeS, DarbyE, ChanW (2010). Predicting progression of alzheimer's disease. Alzheimer's Research & Therapy, 2(1), 2. doi:10.1186/alzrt25
FreemanSH, HymanBT, SimsKB, Hedley-WhyteET, VossoughA, FroschMP, SchmahmannJD (2009). Adult onset leukodystrophy with neuroaxonal spheroids: Clinical, neuroimaging and neuropathologic observations. Brain Pathology (Zurich, Switzerland), 19(1), 39–47. doi:10.1111/j.1750-3639.2008.00163.x
LopezOL, WisniewskiS, HamiltonRL, BeckerJT, KauferDI, DeKoskyST (2000). Predictors of progression in patients with AD and lewy bodies. Neurology, 54(9), 1774–9.
O'SullivanM, JaroszJM, MartinRJ, DeasyN, PowellJF, MarkusHS (2001). MRI hyperintensities of the temporal lobe and external capsule in patients with CADASIL. Neurology, 56(5), 628–34.
PetersenRC, MorrisJC (2005). Mild cognitive impairment as a clinical entity and treatment target. Archives of Neurology, 62(7), 1160–3; discussion 1167. doi:10.1001/archneur.62.7.1160
PortetF, ScarmeasN, CosentinoS, HelznerEP, SternY (2009). Extrapyramidal signs before and after diagnosis of incident alzheimer disease in a prospective population study. Archives of Neurology, 66(9), 1120–6. doi:10.1001/archneurol.2009.196
RidhaB, JosephsKA (2006). Young-onset dementia: A practical approach to diagnosis. The Neurologist, 12(1), 2–13. doi:10.1097/01.nrl.0000186798.86255.69
RudzinskiLA, FletcherRM, DicksonDW, CrookR, HuttonML, AdamsonJ, Graff-RadfordNR (2008). Early onset familial alzheimer disease with spastic paraparesis, dysarthria, and seizures and N135S mutation in PSEN1. Alzheimer Disease and Associated Disorders, 22(3), 299–307. doi:10.1097/WAD.0b013e3181732399
SampsonEL, WarrenJD, RossorMN (2004). Young onset dementia. Postgraduate Medical Journal, 80(941), 125–39.
ScarmeasN, HonigLS, ChoiH, CanteroJ, BrandtJ, BlackerD, AlbertM, AmatniekJC, MarderK, BellK, HauserWA, SternY (2009). Seizures in alzheimer disease: Who, when, and how common?Archives of Neurology, 66(8), 992–7. doi:10.1001/archneurol.2009.130
SchottJM, FoxNC, RossorMN (2002). Genetics of the dementias. Journal of Neurology, Neurosurgery, and Psychiatry, 73 Suppl 2, II27–31.
StaekenborgSS, van der FlierWM, van StraatenEC, LaneR, BarkhofF, ScheltensP (2008). Neurological signs in relation to type of cerebrovascular disease in vascular dementia. Stroke; a Journal of Cerebral Circulation, 39(2), 317–22. doi:10.1161/STROKEAHA.107.493353
Tournier-LasserveE, Iba-ZizenMT, RomeroN, BousserMG (1991). Autosomal dominant syndrome with strokelike episodes and leukoencephalopathy. Stroke; a Journal of Cerebral Circulation, 22(10), 1297–302.
WhitemanML, PostMJ, BergerJR, TateLG, BellMD, LimonteLP (1993). Progressive multifocal leukoencephalopathy in 47 HIV-seropositive patients: Neuroimaging with clinical and pathologic correlation. Radiology, 187(1), 233–40.
WiderC, Van GerpenJA, DeArmondS, ShusterEA, DicksonDW, WszolekZK (2009). Leukoencephalopathy with spheroids (HDLS) and pigmentary leukodystrophy (POLD): A single entity?Neurology, 72(22), 1953–59. doi:10.1212/WNL.0b013e3181a826c0