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Rigidity and Dorsiflexion of the Neck in Progressive Supranuclear Palsy and the Interstitial Nucleus of Cajal

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Rigidity and Dorsiflexion of the Neck in Progressive Supranuclear Palsy and the Interstitial Nucleus of Cajal J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.50.9.1197 on 1 September 1987. Downloaded from Journal of Neurology, Neurosurgery, and Psychiatry 1987;50:1197-1203 Rigidity and dorsiflexion of the neck in progressive supranuclear palsy and the interstitial nucleus of Cajal JUNKO FUKUSHIMA-KUDO, KIKURO FUKUSHIMA, KUNIO TASHIRO* From the Department ofPhysiology, and Division ofNeurology,* Department ofNeurosurgery, Hokkaido University School ofMedicine, Sapporo, Japan SUMMARY Rigidity and dorsiflexion of the neck are typical signs in progressive supranuclear palsy, but the responsible areas in the brain are unknown. To examine whether bilateral lesions of the interstitial nucleus of Cajal (INC) in the midbrain tegmentum contribute to the signs of patients with progressive supranuclear palsy, we have made bilateral INC lesions in cats and tried to cor- relate these studies with clinical and pathological data, including our case of progressive supra- nuclear palsy. Bilateral INC lesioned cats showed dorsiflexion of the neck and impairment of vertical eye movement, similar to progressive supranuclear palsy patients. Analysis of the previous clinical-pathological studies and our case have shown that dorsiflexion of the neck in progressive supranuclear palsy patients was correlated more with INC lesions than lesions of the basal ganglia. guest. Protected by copyright. Since the first description of progressive supranuclear that of progressive supranuclear palsy.5 Therefore, it palsy by Steele et al,' it has been known that rigidity seems unlikely that the dorsiflexion of the neck can be and dorsiflexion of the neck with disturbances of ver- explained by lesions of the basal ganglia alone. In tical eye movements are typical features in progressive progressive supranuclear palsy patients who exhibited supranuclear palsy patients. Though there have been dorsiflexion of the neck, pneumoencephalogram and many clinical reports on impairment of vertical eye computed axial tomography (CT) have shown atro- movements,2 3 the brain areas responsible for this phy of the midbrain tegmentum,6 7 suggesting that characteristic neck posture and rigidity have not been lesions of the midbrain tegmentum may be involved clarified. in rigidity and dorsiflexion of the neck.89 According to the pathological studies of The interstitial nucleus of Cajal (INC), located in progressive supranuclear palsy, neuronal loss and the the tegmentum of the rostral midbrain is a cell group formation of neurofibrillary tangles occur in the lateral to the Edinger-Westphal nucleus adjacent to globus pallidus, subthalamus, substantia nigra, red the periaqueductal grey. It has been found that bilat- nucleus and the midbrain tegmentum. Unilateral sub- eral INC lesions in cats cause dorsiflexion of the thalamic vascular disorders cause hemiballismus, neck.'0 However, the behavioural deficits of such cats with hypotonicity of muscles, while damage to basal were not examined in detail in the previous studies.'0 ganglia including putamen and globus pallidus with In the present experiments, we have made further birth trauma cause athetosis and spasticity.4 In nei- observations on behavioural deficits produced by http://jnnp.bmj.com/ ther case, has neck dorsiflexion been seen. The patho- bilateral INC lesions in cats to examine whether INC logical findings of Parkinson's disease disclose neu- lesions contribute to the characteristic neck posture of ronal loss and depigmentation in the substantia nigra. progressive supranuclear palsy patients, and have Although patients with Parkinson's disease show tried to correlate these studies with clinical and rigidity of the musculature, their head is bowed and pathological data, including our case of progressive the trunk is bent forward, a different posture from supranuclear palsy. The INC areas have also been shown to participate in vertical eye movements." -'1 We report that INC on September 24, 2021 by Address for reprint requests: J Fukushima-Kudo, MD, Department lesioned cats also show impairment of vertical eye of Physiology, Hokkaido University School of Medicine, West 7, movement characteristic to progressive supranuclear North 15, Sapporo 060, Japan. palsy patients. Part of our case report and results of animal experiments were presented earlier Received 23 September 1986. Accepted 6 November 1986 briefly.71415 1197 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.50.9.1197 on 1 September 1987. Downloaded from 1198 Fukushima-Kudo, Fukushima, Tashiro Methods A 2 Experiments were performed on 11 cats. Chronic bilateral lesions of the INC were made stereotaxically"6 (A 4 5-6 0, L 10-1 2, H -0l1--0 2) under halothane nitrous oxide 1.. 1," anesthesia by passing 0 5 mA DC current for 1-2 min at 2-4 different points through Isonel coated steel electrodes with exposed tips of about 0 2 mm. To locate the INC, trains of 21, 02 ims, 30,pA cathodal pulses (at a rate of 333/s) were applied. Stimulus-evoked 5 neck muscles contraction was observed under light anaes- thesia, because of the direct monosynaptic excitatory con- nections from the INC to the ipsilateral neck muscles (sternocleidomastoideus, splenius, biventer cervicis- complexus).17 18 All surgery was done under aseptic condi- l-m tions and a head holder was installed on the skull in some of l1mm the animals. All cats were treated with antibiotics con- tinuously for about a week after surgery. To discriminate the B 1 2 3 effect of lesions of the passing fibres that come from the structures rostral to the INC, chronic lesions of the rostral /\ reticular formation were performed by passing 0-5 mA DC currents for 2 min at lateral planes 1 5, 3 0 and 4-5 mm on both sides (A 6-5-7-0, H -0 3--0 4). Vertical electro- oculograms (EOGs) were recorded 2 to 3 days after surgery CZ 0A h by using surface electrodes placed above and below one eye ~b\l/timm guest. Protected by copyright. (DC recording). Horizontal EOGs were recorded similarly, _,- _// e-A, with surface electrodes placed on the lateral aspects of both orbits. The cats were comfortably placed in a bag and the Fig I (A) Location ofchronic lesions that did not cause skull of each cat was fixed by the head holder during EOG dorsiflexion ofthe neck. Schematic cross sections ofthe recording. Behaviour of the animals that had received suc- midbrain gofrom rostral (1) to caudal (5). Lesions of4 of cessful bilateral INC lesions was observed for 3 weeks after the 6 cats that did not show impairment ofneck posture are surgery at which time they were deeply anaesthetised with shown by diferent symbols. (B) Location ofchronic lesions Nembutal (60 mg/kg) and perfused through the heart with that caused dorsiflexion ofthe neck. Schematic cross sections 10% formalin. Other cats were killed similarly 1 week after of1-3 in (B) correspond to sections 3-5 in (A). Lesions of3 surgery. The extent of the lesions in the midbrain was ofthe 5 cats that developed dorsiflexion ofthe neck are shown verified histologically, using Kluiver-Barerra methods. by different symbols. ND: nucleus ofDarkschewitsch; EW: Edinger- Westphal nucleus; R: red nucleus; IfIn: oculomotor Results nerve. Figure IA illustrates the extent of lesions in four of the six cats in which lesions were made outside the INC. Lesions made in the bilateral wide areas rostral INC are not responsible for the disturbance of head to the INC without damaging it did not cause any posture following INC lesions. clear disturbances of the neck posture in three cats Bilateral INC lesioned cats showed upturned head tested (fig 1(A) 1,2). Lesions that damaged the nucleus posture (fig 2(A)l), and walked with neck retracted of Darkschewitsch, periaqueductal grey and the backwards. Locomotion was smooth although they neighbouring structures, excluding the INC did not showed slight stiffness of the forelimbs (fig 2(A)2). In http://jnnp.bmj.com/ cause impairment of the neck posture in three other two of them, dorsiflexion was so strong that they ten- cats (fig I(A)3-5). On the other hand, typical ded to fall backwards (fig 2(B)l). Neck dorsiflexion dorsiflexion of the neck occurred in five cats, two of appeared soon after the animals recovered from the which are presented in fig 2 as examples. Figure 1(B) effects of anaesthesia after surgery and lasted for at illustrates the extent of lesions of three of the five cats least 3 weeks consistently, whether they were sitting that caused neck dorsiflexion. The lesions covered (fig 2(A)l; (B)1), standing or walking (fig 2(A)2). most of the INC. The extent of lesions of the remain- Furthermore, when they drank water from a basin on ing two cats were similar. These results show that the floor, the neck remained extended and was not on September 24, 2021 by neck dorsiflexion resulted from lesions of bilateral flexed ventrally (fig 2(B)2). These observations indi- INC, but that it was not caused by lesions of the cate that head movement is also impaired in INC neighbouring structures especially areas rostral to it, lesioned cats. also indicating that interruption of descending fibres The cats' posture was observed in the dark using an originating in the reticular formation rostral to the infrared camera. The dorsiflexion of the neck J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.50.9.1197 on 1 September 1987. Downloaded from Neck rigidity in extension and the interstitial nucleus of Cajal 1199 when they were allowed to walk around on the table. Similar behaviour was observed in all the cats that had received bilateral INC lesions. Figure 3, (A) and (B) shows examples of eye move- ments in a normal cat and a bilateral INC lesioned cat, during spontaneous saccades. Normal cats were able to maintain both horizontal and vertical eye pos- ition (fig 3(A)).
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