Dongen, H.R. (Hugo) van

Doorn, P.A. (Pieter) van

Koning, I. (Inge) de

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Inge de KoningPieter A. van DoornHugo R. van DongenSlowly progressive isolated dysarthria: longitudinal course, speech features, and neuropsychological deficitsReceived: 29 April 1997Received in revised form: 22 August 1997Accepted: 25 August 1997Sirs: Dysarthria can be the first signof a neurological disease. In the ini-tial phase it can be difficult to differ-entiate dysarthria from other (ac-quired) speech disturbances, espe-cially if the speech problem remainsmild and isolated for a long period.We describe the course of a slowlyprogressive dysarthria in relation tomotor deficits mainly limited to thespeech musculature in a patient whoproved to have primary lateral scle-rosis (PLS).In 1992, a 72-year-old healthywoman visited our department withthe complaint of a persistent speechproblem, which – in her view – hadstarted immediately after the death ofher husband in 1988. However, lateron relatives unanimously reported agradual onset of speech problems 1 year before this event. As speechtherapy was not effective, she wasreferred to a neurologist elsewhere in1990, but as neurological examina-tion and CT showed no abnormalitythe speech disturbance was consid-ered to be psychogenic.Full neurological re-examinationrevealed no abnormality, except fordysarthric signs (see Table 1 for thecourse of dysarthria [3] and motordeficits of the speech musculature).CT and laboratory investigation, in-cluding complete blood count, TSH,copper, ceruloplasmin, very longchain fatty acid ratios and arylsul-phatase levels were normal, and anti-bodies against acethylcholinreceptorswere absent. Neuropsychological ex-amination demonstrated an IQ withinthe normal range (25–50th per-centile) [8]; there were no signs ofmemory disorders or aphasia. In1994, neurological examination onlyrevealed a bilateral positive palmo-mental reflex. Cranial MRI was nor-mal.In 1995, pathological brainstemreflexes and a Babinski sign on theleft side were present. ExtendedEMG examination, including thetongue, revealed no denervation po-tentials or any other abnormalities.Brain MRI showed one lacunar in-farct in the right cerebral peduncle.SPECT revealed bilaterally reducedactivity preferentially in the basalmotor cortex with normal activity atthe level of the brainstem nuclei. Thediagnosis of PLS was made as thepatient fulfilled the diagnostic crite-ria proposed by Pringle et al. [7].In September 1995, neuropsycho-logical examination still showed anormal intelligence (50–75th per-centile) [8]. She was well orientedwithout manifest memory disorder,aphasia, or apraxia. However, shehad problems with concept shifting,generating new ideas, planning andorganizing. Behaviour at testing wasslightly impulsive [1]. Based onthese results, it was concluded thatthere were mild frontal lobe defi-cits.In January 1996 the patient couldonly communicate with paper andpencil; she no longer attempted tospeak. She had major problems withswallowing and chewing owing tosevere weakness of the tongue. De-spite the anarthria and concomitantproblems the patient still lived inde-pendently and communicated withher relatives by telefax.Slowly progressive, isolatedspeech disorders have been knownsince Mesulam et al. [6] reported onprimary progressive aphasia. How-ever, a primary progressive pseudob-ulbar dysarthria has not been explic-itly reported before. Nevertheless, thisphenomenon is known [4, 5]. Proba-bly the course of slowly progressivedysarthria has never been analysed,because in most of the reported pa-tients the onset of the dysarthria wasrelatively mild and the patients con-sulted a neurologist only when con-comitant motor deficits were distinc-tively present. The pathological ab-normalities in patients with PLS arelimited to the precentral motor cortexand the corticospinal tracts [7]. Inthe patient we describe frontal lobedysfunction is suggested by the localhypoperfusion on the SPECT scanand is supported by the neuropsycho-logical findings in 1995, similar tothe findings by Caselli et al. in pa-tients with PLS [2].In conclusion, an unclassifiablespeech disturbance with an insidiousonset and a slow course without neu-rological concomitant signs suggestsa psychogenic cause, but long-termfollow-up may reveal a neurologicaldisorder. In our patient, dysarthriawas the first and only sign of PLSfor 5 years.LETTERS TO THE EDITORSJ Neurol (1997) 244 :664–667© Springer-Verlag 1997665Table 1 Perceptual analysis ofthe patient’s dysarthric speechin relation to the motor deficitsof the speech musculaturea > Increasing severity ofspeech dimension compared toprevious years [3]; – no abnor-malities, + mild involvement,++ severe involvement, +++ impossible1992 1993 1994 1995 1996Perceptual analysis of the dysarthric speechaArticulation Imprecise > Imprecise > Imprecise > Imprecise Unintelligibleconsonant consonant consonants consonants speechclusters clustersVowelsdistorted,phonemes pro-longedVoice quality Monotonous Monotonous > Monotonousvolume volume and volume andpitch pitch > harsh voice > harsh voicestrained, harsh voicestrangled voiceRespiration Audible > Audible > Audibleforced in- > forced in-expiration expirationProsody Variable rate Slow speech >> Slow speech Reducedof speech rate rate stressShort phrases, >> Intervals February: intervals pro- prolonged no speech longed possibleThe course of motor deficits of the speech musculatureN. II, IV, VI – – – – –N. VOpen jaw – – – – +++Close jaw – – – – +Shake jaw – – – +++ +++Chewing – – – + +++Sensory – – – – –N. VIIWrinkle – – – – +++foreheadClosure of eyes – – – + ++Spreading lips – + + ++ +++Tingling lips – – – ++ +++Blowing – – – ++ +++Facial mimicry – – – + ++N. VIII – – – – –N. IX, XElevation – – – – –palatumSwallowing – – – + ++N. XI – – – – –N. XIITongue:Atrophy – – – – –Fasciculations – – – – –Protrusion – - – + ++Upward – – – +++ +++Downward – – – +++ +++Lateral – + + ++ +++References1.Bechara A, Damasio H, Tranel D,Damasio AR (1997) Deciding advanta-geously before knowing the advanta-geous strategy. Science 275: 1293–12952.Caselli RJ, Smith BE, Osborne D (1995)Primary lateral sclerosis: a neuropsycho-logical study. Neurology 45: 2005–20093.Darley FL, Aronson AE, Brown JK(1969) Differential diagnostic patternsof dysarthria. J Speech Hear Res 12:246–2694.Fisher CM (1977) Pure spastic paralysisof corticospinal origin. Can J Neurol Sci4: 251–2585.Gastaut JL, Michel B, Figarella-BrangerD, Somma-Mauvais H (1988) Chronicprogressive spinobulbar spasticity. A rare form of primary lateral sclerosis.Arch Neurol 45: 509–5136.Mesulam MM (1987) Primary progres-sive aphasia – differentiation from Alz-heimer’s disease. Ann Neurol 22: 533–5347.Pringle CE, Hudson AJ, Munoz DG,Kiernan JA, Brown WF, Ebers GC(1992) Primary lateral sclerosis. Clinicalfeatures, neuropathology and diagnosticcriteria. Brain 115: 495–5208.Raven JC (1965) Guide to using thecoloured progressive matrices. Lewis,LondonI. de Koning · P. A. van Doorn ·H. R. van Dongen (Y)Department of Neurology, University Hospital Rotterdam-Dijkzigt,dr. Molewaterplein 40, 3015 GD Rotterdam, The NetherlandsKevin CroftsRosie BrennanPeter KearneyGerard O’ConnorVigabatrin-induced optic neuropathyReceived: 31 January 1997Received in revised form: 17 July 1997Accepted: 1 August 1997Vigabatrin [7] may have an impor-tant role in the treatment of seizuresin tuberous sclerosis, as well as be-ing a helpful anticonvulsant in otherepileptic conditions. The reportedside-effects of vigabatrin are centralnervous system-related and mainlyof a behavioural nature. Visual com-plaints such as diplopia and constric-tion of visual fields have been noted.We report a child with partial sei-zures and secondary generalisationwho developed optic neuritis whiletaking vigabatrin.An 8-year-old girl presented withepisodes of pain and twitching in theright lower limb, which at times pro-gressed to a generalised tonic clonicseizure. The initial EEG was normal,but video telemetry confirmed theclinical diagnosis of partial epilepsywith secondary generalisation. CTand MRI findings were normal. Thepatient was initially treated with car-bamazepine without effect. She sub-sequently improved on valproate 400mg b.i.d. The focal episodes recurredwith twitching of the right face andneck associated with loss of speech.Vigabatrin (Sabril, Marion MerrellDow Ltd) 500 mg nocte was addedto valproate 400 mg b.i.d. Two daysafter starting the vigabatrin she wasunable to speak. She subsequentlysaid that she knew what she wantedto say but could not get the wordsout. She was also unable to read. Theparents discontinued the vigabatrinas they felt she was having some sortof reaction to the drug. Two dayslater she was well again and was re-ferred for a paediatric neurologyopinion. She had further episodes oftrace-like states and associated dys-phasia. The Landau-Klaffner syn-drome was considered, but the finalassessment was partial complexseizures. She was recommenced onvigabatrin 125 mg b.i.d. and valproate400 mg b.i.d. She remained stablewithout seizures for 6 months, whenshe developed a tremor of her righthand. The vigabatrin was increasedto 375 mg/day. She remained wellfor a further 3 months when she de-veloped pain over her left eye for 1day. She had no systemic symptoms,no rash, and had not received anyimmunisations recently. On routinereview 6 months later she was re-ferred for an ophthalmological opin-ion because of disc pallor. Her fullblood court was normal and intrader-mal allergen testing was negative.Opthalmology examination re-vealed a visual acuity of “handmovements” in the right eye and6/18 in the left eye. There was aright afferent pupillary defect andred desaturation on colour visiontesting. Fundoscopy confirmed bilat-eral optic disc pallor. Electrodiagnos-tic tests showed an abnormal patternvisually evoked response (VER) bi-laterally. The VER on the right wasalmost absent with no recordablepeak. On the left there was a delayedlatency to 163.5 ms. Electrooculog-raphy was normal with normal Ar-den ratios. Electroretinography wasnormal with normal oscillatory po-tentials. Vigabatrin was stopped.Treatment included dexamethosoneIV and Pabrinex IV. Six weeks laterher visual acuity had improved to6/24 on the right and 6/12 on theleft. Five months following her ini-tial presentation her visual acuity hadimproved to 6/9 bilaterally. Her VERhad improved markedly with a la-tency of 153 ms on the right and 147ms on the left. MRI showed no evi-dence of demyelination.666

Slowly progressive isolated dysarthria: Longitudinal course, speech features, and neuropsychological deficits [1]

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Slowly progressive isolated dysarthria: Longitudinal course, speech features, and neuropsychological deficits [1]

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