CASE REPORT

Parietal Cheiro-oral SyndromeYuzuru Yasuda, Toshiyuki Watanabe and Akira Ogura

Abstract

Cheiro-oral syndrome due to a parietal lesion has beenreported in conjuction with a brain tumor, infarction andmigraine. Only six reports of cheiro-oral syndromedue toa parietal infarction have been reported to date. Wetreateda 45-year-old womanwith cheiro-oral syndrome due to aparietal infarction. Her sensory disturbance was charac-terized by paresthesia in the lower face and hand on theleft side, and severe involvement of stereognosis andgraphesthesia in the left hand. The pathogenesis of pari-etal cheiro-oral syndrome is discussed.(Internal Medicine 39: 1105-1107, 2000)

Key words: paresthesia, threshold, postcentral gyrus, inferiorparietal lobule, mouth, hand

Introduction

Cheiro-oral syndrome is a well-known sensory disturbancewhich affects the unilateral hand and mouth regions. This syn-drome was first described by Sittig in 1914 (1) and was attri-buted to a tumor in the parietal lobe. Although thereafter therewere few reports of cheiro-oral syndrome due to parietal le-sion (2-4), many cases of cheiro-oral syndrome due to vascu-lar lesions in the thalamus, brainstem and thalamocortical pro-jections have been reported and the pathogenesis of cheiro-oral syndrome in conjunction with these lesions has been dis-cussed. However the pathogenesis of cheiro-oral syndrome inconjunction with a parietal lesion remains unresolved. In thepast 15 years, reports of cheiro-oral syndrome due to a parietalinfarction have also appeared (5-10). Weencountered a pa-tient with cheiro-oral syndromedue to a parietal infarction asdetected by magnetic resonance imaging (MRI) and discussthe pathogenesis of parietal cheiro-oral syndrome.

Case ReportA 45-year-old hypertensive womansuddenly developedparesthesia in the left fingers. Hemiparesis, nausea and head-ache were not noted, and CTwas normal. The paresthesia dis-

appeared within 2 weeks. After that she was healthy, but 6months later, she developed dysarthria, paresthesia in the fin-gers and slight weakness of the upper extremity on the leftside. Headache, nausea and vomiting were absent, and CTwasnormal. These symptomsgradually subsided in a month. How-ever, she developed paresthesia in the lower face and hand, andslight weakness of the upper extremity on the left side onemonth later. Headache, diplopia and nausea were absent. Asthese symptomsdid not improve, she was transferred to ourdep artment.Her blood pressure was 162/100 mmHg.She was alert. Herintelligence was normal without disorientation. Eye movementwas normal, with no nystagmus. No cranial nerve palsy wasnoted. She showed slight left hemiparesis. Deep tendon reflexeswere slightly exaggerated on the left side. Babinski's andChaddock'sreflexes werenot elicited. Paresthesia wasobservedin the lower face and hand on the left side. Objective sensoryloss was not present in the face or mouth. Touch, pain, tem-perature and vibration sensation were normal, but position sen-sation, stereognosis and garaphesthesia (recognition of lettersdrawn by the examiner on the palm of the hand) were nearlyabolished in the left hand.Routine blood was normal except for hyperlipidemia. Lu-pus anticoagulant and anticardiolipin antibodies were negative.Urine and cerebrospinal fluid examination were normal. Elec-trocardiogram and roentgenogram of the chest were normal.M-modeand two-dimensional echocardiographies were nor-mal. Anelectroencephalogram and auditory evoked responsesshowed no abnormality. Somatosensory evoked potentials(SEPs) in response to electrical stimulation of the mediannerveof the wrist were recorded. Stimulation duration was 0.2 msecand was of sufficient intensity to produce consistent muscletwitches. Recording electrodes were placed over right and leftErb's point, the seventh cervical vertebra, C3, C4, and Fz. Theoverall band pass ranged from 5Hz to 2 kHz. Analysis timewas 60 msec, and the mean of 1 ,000 responses was determined.N9, Nil and N13 potentials from both sides showed no abnor-mality. The latencies of N20 potential from both sides werenormal, but the amplitude of N20from the left side showed adecrease to 26.8% of the right side. MRIshowed a right pre-and postcentral infarction with underlying white matter dam-age. The infarction was extended posteriorly to underlying whitematter of the anterior part of the inferior parietal lobule (Fig.

From the Department of Neurology, Otsu Red Cross Hospital, OtsuReceived for publication January 26, 2000; Accepted for publication June 4, 2000Reprint requests should be addressed to Dr. Yuzuru Yasuda, the Department of Neurology, Otsu Red Cross Hospital, 1-1-35, Nagara, Otsu 520-85 1 1

Internal Medicine Vol. 39, No. 12 (December 2000) 1105

Yasuda et al

Figure 1. MRI showed a right pre- and postcentral infarction (arrows) with underlying white matter damage, and theinfarction was extended posteriorly to the underlying white matter of the anterior part of the inferior parietal lobule.A : FLAIR images (6,856/110; inversion time, 1,700), axial view, B: T2-weighted image (2,500/80), saggital view.

1). No lesion was found in the thalamus, brainstem or else-where. Paresthesia in the lower face and hand on the left sideremained for three years.

Discussion

To date, only six reports of cheiro-oral syndrome due todefinite parietal infarction have been reported (5-10). Table 1indicates the cases of cheiro-oral syndrome due to a parietalinfarction. We identified three causative lesions in parietalcheiro-oral syndrome.

The first causative lesions, as revealed in the cases of Tagawaet al (5) and Isobe et al (10), is restricted to the postcentralgyrus (PCG in Table 1). The sensory dysfunction is character-ized by the preservation of stereognosis and graphesthesia. In-volvement of the rostral postcentral gyrus may cause decreasedposition and discriminative touch sense, and that of the morecaudal part of the postcentral gyrus maycause decreased painand temperature sense.The second causative lesion is in the postcentral gyrus andthe anterior part of the inferior parietal lobule (PCG+APIPLinTable 1), as described by Bogousslavsky et al (6), Mrabet et al(7) and Matsuoka et al (8). The sensory dysfunction is charac-terized by severe involvement of stereognosis and graphesthesia.In this group, pain, temperature, or position sense can be in-volved according to the extent of involvement of the postcen-tral gyrus. As it has been reported that the anterior part of the

inferior parietal lobule plays a crucial role in tactile object rec-ognition (1 1), the involvement of this area causes astereogno-sis. The causative lesion in the case reported by Matsuokaet al(8) was confined to the cerebral cortex in postcentral gyrusand the anterior part of the inferior parietal lobule. However,the causative lesion in the cases of Bogousslavsky et al (6) andMrabet et al (7) was not confined to the cerebral cortex in thepostcentral gyrus and was extended to the underlying whitematter of the postcentral gyrus and the anterior part of the infe-rior parietal lobule, as in the present case.The third causative lesion is restricted to the base of the

central sulcus (BASEin Table 1), as in the case reported byShiga et al (9), and the sensory disturbance is characterized bythe absence of objective sensory loss. The primary sensory areain the cerebral cortex is thought to be in Brodmann's area 3, 1,and 2. Brodmann's area 3b is positioned in the anterior wall ofthe postcentral gyrus, but Brodmann's area 3a is in the bottomof the central sulcus and partially in the posterior wall of theprecentral gyrus. The origin of N20 potential in somatosen-sory evoked potentials (SEPs) to median nerve stimulation isthought to be in Brodmann's area 3b (12, 13). The case of Shigaet al (9) showed normal N20 potential which indicates thatBrodmann's area 3b was not involved.In a patient with parietal cheiro-oral syndrome, the reasonparesthesia appears only in the hand and mouth regions hasbeen examined. Chin, nose and face areas exist between thehand and mouth areas in the postcentral parietal cortex of

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Parietal Cheiro-oral Syndrome

Table 1. Clinical Findings of Cheiro-oral Syndrome Following a Parietal InfarctionAuthor Age/Sex Causative Topo- Pares- Superficial Vibration Position Stereo- Graphes- Paresis SEPdate, lesion graphy thesia sensation sense sense gnosis thesiareference

Tagawa 51/M PCG O-A + D D SD normal normal yes ND1984(5)Bogous- 63/M PCG+APIPL O-A + SD D SD SD SD yes NDslavsky 71/M PCG+APIPL O-A + normal normal SD SD SD yes ND1991(6)Mrabet 51/M PCG+APIPL O-F + normal normal normal SD SD no ND1993(7)Matsuoka 64/M PCG+APIPL O-H + SD normal normal SD SD yes ND1995(8)Shiga 67/M BASE O-F + normal normal normal normal normal no normal1996(9)Isobe 54/M PCG O-A + normal normal normal normal normal yes ND1997(10)Present 45/F PCG+APIPL O-H + normal normal SD SD SD yes Lowamp.

case of N20

PCG: postcentral gyrus, APIPL: anterior part of the inferior parietal lobule, BASE: base of the central sulucus, O: perioral, F: finger, H:hand, A: arm, D: disturbed, SD: severely disturbed, ND: not described, SEP: somatosensory evoked potential, amp.: amplitude

Macacafascicularis with multi-unit microelectrode recordings(14). Because Sutherling et al reported a similarity of corticalsensory organization of the fingers between humans and otherprimates (15), almost the samesomatotopy, namelythe exist-ence of the chin, nose and face area between the hand and mouthareas, seems to exist in humans. To date, the classical cheiro-oral syndrome has been explained by the closeness of the sen-sory nuclei or fibers from the hand and mouth at any level inthe parietal lobe, thalamus, brainstem and thalamocortical pro-jections. However, the precise mechanism remains unclear.The present case showed normal latency and decreasedamplitude of N20 potential, elicited by stimulation on the dis-turbed side, which indicates the partial involvement ofBrodmann's area 3b (12, 13). Recently, Yasuda et al reportedthat paresthesia recovery occurs in the following sequence:thorax, foot, mouth and then hand, in patients with cerebralinfarction or hemorrhage whoshowedparesthesia in the tho-rax, foot, mouth or hand region (16). This suggests that thedetection threshold, from highest to lowest, occurs in the fol-lowing sequence: thorax, foot, mouth, and hand. Likewise, thedetection threshold of the chin, nose and face may be higherthan the mouth or hand; therefore, paresthesia appears only inthe mouth and hand region in parietal cheiro-oral syndrome.

Refe rences

1) Sittig O. Klinische Beitrage zur Lehre von der Lokalisation der sensiblenRindenzentren. Prag Med Wochenschr 45: 548-550, 1914 (in German).

2) Schuster P. Beitrage zur Lehre von den sensiblen der Grosshirnrinde.Neurol Zentralbl 36: 33 1-336, 1917 (in German).

3) Bruyn GW, Weenink HR. Migraine accompagnee. A critical evaluation.Headache 6: 1-22, 1966.

4) Noda S, Umezaki H, Nagata S, Kuromatsu C. Cortical cheiro-oral syn-

drome due to convexity meningioma. Shinkeinaika 18: 506-509, 1983(in Japanese).

5) Tagawa K, Ikeda Y, Hirata Y, Takahashi A, Satoh Y. Cheiro-oral syn-drome due to infarction of the parietal lobe. Shinkeinaika 20: 70-73, 1984(in Japanese).

6) Bogousslavsky J, Dizerens K, Regli F, Despland PA. Opercular cheiro-oral syndrome. Arch Neurol 48: 658-661, 1991.

7) Mrabet A, Gouider R, Bouteraa M, Haddad A. Cheiro-oral syndrome andparietal stroke. Cerebrovasc Dis 3: 183-184, 1993.

8) Matsuoka H, Tamaru T, Chuma T, Mano Y, Takayanagi T. Opercular cheiro-oral syndrome due to cerebral infarction. A case report. Shinkeinaika 43:48-52, 1995 (in Japanese).

9) Shiga K, Makino M, Ueda Y, Nakajima K, Hirata T. Cheiro-oral syn-drome following a cortical brain infarction in the precentral gyrus and atthe base of the central sulcus. A case report. Rinsho Shinkeigaku 36: 1 104-1 106, 1996 (in Japanese).

10) Isobe C, Konno S, Yamazaki K, Takahashi H, Tohgi H. Cheiro-oral syn-drome due to cortical in farction. A case report. Shinkeinaika 47: 413-416, 1997 (in Japanese).

1 1) Caselli RJ. Rediscovering tactile agnosia. Mayo Clin Proc 66: 129-142,1991.

12) Dinner DS, Luders H, Lesser RP, Morris HH. Cortical generators of so-matosensory evoked potentials to median nerve stimulation. Neurology

37: 1141-1145, 1987.

13) Wood CC, Cohen D, Cuffin BN, Yarita M, Allison T. Electrical sourses inthe human somatosensory cortex: Identification by combined magneticand potential recordings. Sciences 227: 1051-1053, 1985.

14) Nelson RJ, Sur M, Felleman DJ, Kaas JH. Representation of the bodysurface in postcentral parietal coetex of Macacafascicularis. J Comp

Neurol 192: 61 1-643, 1980.

15) Sutherling WW,Levesque MF, Baumgartner C. Cortical sensoryrepreaentation of the human hand: Size of finger regions and

nonoverlapping digit somatotopy. Neurology 42: 1 020-1028, 1992.16) Yasuda Y, Watanabe T, Tanaka H, Akiguchi I, Kimura J, Kameyama M.Unusual sensory disturbance in the thoracic region after stroke: Relation-ship to cheiro-oral and cheiro-oral-pedal syndrome. J Neurol Sciences

153: 68-75, 1997.

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