Riechert T, Hassler R, Mundinger F, Bronisch F, Schmidt K
Confin Neurol. 1975;37(1-3):24-40. doi: 10.1159/000102709.
Two postmortem case of multiple sclerosis treated by sterotactic operations for the intention shaking of limbs, trunk, and head, and for the action myoclonus are analyzed to determine the location of the substrate of myoclonic and ballistic movements, the location of the coagulations for relief of these movements, and whether fresh demyelinating foci are elicited by intracerebral interventions. In the first case of a clinically typical multiple sclerosis, the foci responsible for the severe action myoclonus and intention ataxia of the trunk are demyelinations in the right and left red nucleus resulting in nerve cell damage and loss and an almost complete destruction of myelinated fibers. The restricted foci in the white matter of the cerebellum which do not involve the cerebellar nuclei are not extensive enough or old enough to be the cause of the action myoclonus but may, perhaps, sustain the pathogenesis. - In the second case of cerebral palsy and combined multiple sclerosis (detected post mortem), the combination of the severe damage of putamen and caudate nucleus by status marmoratus and the extensive nerve cell and fiber damage due to demyelinating foci in the substantia nigra are probably the substrate of the jactitation and intention myoclonus of the left limbs. The stereotactic coagulation of the dentatothalamic and pallidothalamic fibers in the base of V. o.p. and V.o.a. at the point where they pass through the zona incerta (location confirmed post mortem) resulted in a nearly complete relief of hyperkinetic movements. In the first case, fresh demyelinating foci are present in both hemispheres with stereotactic interventions; these foci are located, amongother places, around the coagulation and the electrode track. In the second case, post mortem serial brain sections demonstrate that stereotactic operations even in subacute multiple sclerosis can be carried out without eliciting any exacerbation of demyelination foci. Therefore, the danger exists that stereotactic intervention in cases of multiple sclerosis may precipitate fresh demyelinating foci. As our clinical experience [Riechert and Richter, 1972a, b] indicates, however, this occurred in markedly less than 10% of the cases.
分析了两例因肢体、躯干和头部意向性震颤以及动作性肌阵挛而接受立体定向手术治疗的多发性硬化症尸检病例，以确定肌阵挛和弹道运动的底物位置、缓解这些运动的凝固位置，以及脑内干预是否会引发新的脱髓鞘病灶。在第一例临床典型的多发性硬化症病例中，导致严重动作性肌阵挛和躯干意向性共济失调的病灶是左右红核的脱髓鞘，导致神经细胞损伤和丢失以及有髓纤维几乎完全破坏。小脑白质中不涉及小脑核的局限性病灶不够广泛或不够陈旧，不足以成为动作性肌阵挛的原因，但可能维持发病机制。- 在第二例脑瘫合并多发性硬化症（尸检时发现）病例中，大理石样状态对壳核和尾状核的严重损害以及黑质脱髓鞘病灶导致的广泛神经细胞和纤维损伤可能是左肢抖动和意向性肌阵挛的底物。在丘脑腹外侧核和丘脑腹前核底部，齿状丘脑纤维和苍白球丘脑纤维穿过未定带的部位（尸检时确认位置）进行立体定向凝固，几乎完全缓解了运动亢进。在第一例中，立体定向干预后两侧半球均出现新的脱髓鞘病灶；这些病灶除其他部位外，位于凝固部位和电极轨迹周围。在第二例中，尸检连续脑切片显示，即使在亚急性多发性硬化症中，立体定向手术也不会引发脱髓鞘病灶的任何恶化。因此，存在多发性硬化症病例中立体定向干预可能引发新的脱髓鞘病灶的风险。然而，正如我们的临床经验[里歇特和里希特，1972a，b]所示，这种情况发生的病例明显少于10%。
