Painful Ataxic Hemiparesis Julien Bogousslavsky, MD; Franco Regli, MD; Joseph Ghika, MD; Jean-Jacques Feldmeyer, MD \s=b\ Right hemiparesis with right-sided pain and ataxia developed in a 68-year$ old man. Sensation, neuropsychological function, and somatosensory evoked potentials were normal. Computed tomography showed an isolated fresh infarct in the left part of the thalamus. The pain and ataxic disturbances were related to involvement of the thalamus itself, but the hemiparesis with hyperactive tendon reflexes and Babinski's sign was probably due to associated dysfunction in the adjacent internal capsule from compression or edema. In the available clinicopathological reports of cases with hemiparesis and thalamic infarction, contiguous involvement of the internal capsule or no associated lesion has been reported. Because of the occurrence of pain, which is not present in pontine, mesencephalic, or capsular ataxic hemiparesis, we suggest that the syndrome seen in our patient be called "painful ataxic hemipar- esis." (Arch Neurol 1984;41:892-893) of hemiparesis and /Recurrence bellar ataxia the side of cere- on same the body has been called ataxic hemi¬ paresis,' and is usually caused by a small deep infarct in the contralateral upper pons or internal capsule.1'9 Accepted for publication Aug 13, 1983. From the University Department of Neurology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland. Reprint requests to Department of Neurology, Centre Hospitalier Universitaire Vaudois, 1011 Lausanne, Switzerland (Dr Bogousslavsky). Ataxic hemiparesis is a rather uncom¬ mon syndrome, and its frequency has been evaluated from less than 1% to 9% among deep infarcts and lacu¬ nae.1012 We report a case of ataxic hemiparesis with associated pain in the homolateral arm, but without altered sensation, in which computed tomography (CT) showed evidence of an isolated recent infarct in the con¬ tralateral thalamus. REPORT OF A CASE A 68-year-old former policeman without known vascular risk factors suddenly expe¬ rienced violent burning pains in the right arm and shoulder that were associated with weakness and clumsiness of the right arm and leg. No diplopia or other visual symptom, speech, or mental disturbances were present. After seven days, when he was admitted to the hospital, the pain, as well as the hemiparesis, had improved, but the patient still complained of right-sided ataxia. Neurological Examination Optic fundi, visual acuity, and visual fields were normal. The pupils were in the medium range and fully reacted to light and accommodation. Ocular movements were normal, without nystagmus. Optokinetic nystagmus was normal and symmet¬ rical. Facial sensation was preserved, with symmetrical corneal reflex. A slight inferi¬ or facial paresis was noted. Mandibular movements were intact, as was the jaw reflex. There was no dysarthria or dysphagia, and the palate moved symmetrically. Gag reflex was normal. The tongue pro¬ truded in the midline. Bilateral conduction hypoacusis was present. There was rightsided weakness of the limbs, with crural Fig 1.—Initial computed tomographic scan seven days after onset of symptoms. Note area of low density in lateral part of left side of thalamus. and proximal predominance (3/5). Tendon reflexes were hyperactive on that side, with Babinski's sign. Palmomental and grasp reflexes were absent. The superficial abdominal reflexes were present and sym¬ metrical. There was right-sided dysmetria, hypermetria, and dysdiadochokinesia, with intention tremor and abnormal rebound phenomena. No asterixis was present. The disturbances were more severe in the leg than in the arm. Touch, pain, temperature, position, and vibration sensation were fully preserved, with nor¬ mal two-point discrimination, graphesthesia, topesthesia, and stereognosis. The gait was only slightly disturbed by the rightsided disturbances, without gross ataxia. Detailed neuropsychological examination did not disclose any speech, memory, gnosia, or praxia disturbance. No motor, sen¬ sory, visual, or auditory neglect was present. Results of general examination were normal. The right arm and shoulder did not show any local abnormality that would explain the pain. No carotid bruit was heard. Blood pressure was 140/90 mm Hg. Results of standard blood and urine tests normal. The ESR was 2 mm in the first hour. The ECG was normal. On Dopp¬ ler ultrasonography, the carotid and oph¬ thalmic arteries were normally detected, but the left vertebral artery could not be detected (retromastoid approach). The CSF was normal. Somatosensory evoked potentials (stimulation of the median nerve) did not show any asymmetry or abnormal responses. Computed tomogra¬ phy (Fig 1) showed a hypodense area in the lateral part of the left side of the thalamus. The cerebral hemispheres, the brain stem, and the cerebellum did not show any other area of decreased attenuation. were Evolution After ten days, the pain and weakness had nearly disappeared, but ataxia and Babinski's sign still persisted. Computed tomography (Fig 2) showed a better demarcation of the area of decreased attenuation in the left part of the thala¬ mus. Fig 2. Second computed tomographic scan done seven days after that in Fig 1. Hypodense area af same site is better demarcated. — Downloaded From: http://archneur.jamanetwork.com/ by a University of Iowa User on 06/18/2015 COMMENT Ataxic hemiparesis, the combina¬ tion of homolateral weakness and "cerebellar" dysfunction, was initially reported as a vascular syndrome in 14 patients with homolateral ataxia and crural paresis.2 In the one case studied at autopsy, infarction of the posterior limb of the contralateral internal cap¬ sule was present, but was associated with other infarcts. Since then, the only available pathological examina¬ tions showed lacunar infarction in the upper basis pontis in three cases' and pontine glioma in one case.13 In these cases, the lesion interrupted the corticospinal fibers and the corticopontine fibers before the decussation of the superior cerebellar peduncles. In these patients with pontine infarc¬ tion, tumor, or hemorrhage,14 associ¬ ated oculomotor disturbances1 or cra¬ nial nerve dysfunction" are usually present. Other reports with CT study3·5·6·11·12 described small deep infarcts involv¬ ing the posterior limb of the contralateral internal capsule sometimes extending toward the corona radiata region. In these cases, the frontopontine and temporoparietopontine bun¬ dles are not involved, because they respectively course in the anterior limb and the retrolenticular or sublenticular portion of the internal cap¬ sule. The cerebellar dysfunction is probably related to destruction of cor¬ ticopontine fibers from the precentrai cortex or connecting fibers between the ventrolateral nucleus of the thala¬ mus and the precentrai region. Unlike the situation in pontine lacuna, no oculomotor or cranial nerve abnor¬ mality is present. Ataxic hemiparesis from a mass in the contralateral ros¬ tral mesencephalon has also been reported.15 Our patient showed typical ataxic hemiparesis, and CT scan showed an isolated hypodense area compatible with fresh contralateral infarction of the thalamus, immediately adjacent to the posterior limb of the internal capsule. No objective sensory distur¬ bances were present, but the patient complained of initial burning pain in the corresponding upper limb. Al¬ though the typical Dejerine-Roussy syndrome (hypoesthesia with hyperpathia) is more usual, a painful syn¬ drome without altered sensation has already been reported in thalamic infarcts.16·17 Usually, thalamic lacuna or gross infarction produce objective sensory disturbances, and sometimes mental dysfunction, aphasia, dysmnesia, or neglect.8·1822 None of these trou¬ bles was present in our case. However, contralateral cerebellar ataxia may also be present, when the middle part of the lateral thalamus is involved, with subsequent interruption of the dentato(rubro)thalamic afferent fi¬ bers.16·17·21 Also, Garcin23 and Garcin and Lapresle16 reported the cases of two patients with unilateral cerebellar ataxia, in whom an isolated infarction of the ventrolateral nucleus of the contralateral thalamus (in the first case) and an infarction involving the dorsomedian nucleus, the anterior part of the centromedian nucleus, and intralaminar nuclei of the contralat¬ eral thalamus (in the second case) were demonstrated at autopsy. The first patient initially showed associ¬ ated pain in the inferior limb and transient disturbances of deep sensa¬ tion. The second patient showed asso¬ ciated numbness and paresis of the lower limb, with Babinski's sign. In this case, in our case, and in other reported cases with corticospinal dys¬ function,17·21·24·26 hemiparesis and Ba¬ binski's sign may be related to func¬ tional involvement of the adjacent internal capsule, from local edema or compression.16·25 In the autopsied cases,16·25 no visible focal lesion along the corticospinal pathways could be established. Associated infarction of the adjacent portion of the internal capsule may be another explanation, although such a possibility has been reported only once.27 Involvement of the cerebral peduncle, as in the hémi¬ plégie syndrome of the posterior cere¬ bral artery,28 seems less probable, because of the absence of severe pare¬ sis, hemianopia, or alexia. In summary, our case suggests that ataxic hemiparesis may be related to contralateral thalamic infarction. An¬ other case with ataxic hemiparesis was reported in association with a thalamic syndrome,13 but no CT scan was provided, and the point was not discussed. In our case, involvement of the ventrolateral nucleus of the thala¬ mus was probably responsible for the ataxia, because CT scan showed infarction of the lateral part of the thalamus. More medial nuclei (dorsomedial nucleus, intralaminar nu¬ clei) were probably not involved. Despite initial pain, the ventroposterior nucleus was probably partially spared because of normal sensation and somatosensory evoked potentials. Because severe pain was initially present in the corresponding upper limb, we suggest that homolateral ataxia and corticospinal dysfunction from thalamic infarction should be called "painful ataxic hemiparesis." Downloaded From: http://archneur.jamanetwork.com/ by a University of Iowa User on 06/18/2015 References 1. Fisher CM: Ataxic hemiparesis. Arch Neurol 1978;35:126-128. 2. Fisher CM, Cole M: Homolateral ataxia and crural paresis: A vascular syndrome. J Neurol Neurosurg Psychiatry 1965;28:48-55. 3. 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