Movernenr Disorders Vol. 10, No. 5 , 1995, pp. 685687 0 1995 Movement Disorder Society

Letters to the Editor

Treatment of Superficial Siderosis of the Central Nervous System

To the Editor: We were interested in the report by River et al. regard-

ing the successful treatment of superficial siderosis of the central nervous system by Trientine in a case in which the source of chronic subarachnoid haemorrhage was not found. The patient’s overall condition stabilised during 2 years’ follow-up, and an associated hemifacial spasm, the first reported in conjunction with superficial siderosis, disappeared. As an aside, the fact that the facial nerve was only transiently affected seems to be a curious fea- ture of this nerve in superficial siderosis. It is rarely af- fected, and only four cases have had a facial nerve palsy (1-3) and all have been intermittent except for one of which the patient died. The report of River et al. is the only one of iron chelation possibly having some effect on the course of superficial siderosis. In the literature, six cases have been treated with Trientine (1,4-6), and two cases with desfemoxamine (43) without any effect. We observed one patient who was on Trientine and lost all hearing during treatment over a period of 2 years. Trien- tine may have some place in the treatment of cases in which the source of bleeding cannot be found, but the fact that the cerebrospinal fluid (CSF) femtin remained ele- vated in the case of River et al. suggests that Trientine is only partially effective.

Surgical ablation of the bleeding source holds the most hope of arresting the disease. Several cases have been treated surgically, but as yet with no follow-up. Encour- agingly, the CSF cleared in one case, having been repeat- edly haemorrhagic, after coagulation of an a spider angi- oma in an arachnoid scar after a cervical root avulsion (7). In contrast to the patient reported by River et al., the CSF ferritin returned to normal after excision of a cauda equina ependymoma in another reported case (8). We would like to suggest a possible source of bleeding in the case reported by River et al., which might be worth ex- ploring, but we accept that it may not be treatable. It is well known that superficial siderosis may be secondary to dural pathology and CSF cavity lesions, which lead to altered hydrostatic pressures and venous rupture. A false cavity may have been created when the cerebellar “as- troblastoma” was resected in the patient at 13 years of age, which communicated with the sixth ventricle. The magnetic resonance imaging slices shown were at the up- permost level of the cerebellum and it is impossible to comment on the operation site, but there does seem to a large CSF space be it the vermian cistern or not. The patient then underwent radiotherapy, which may have caused telangiectasia in the wall of the cavity or the ver- mian cistern, leading to a source of chronic subarachnoid haemorrhage. Certainly posterior fossa surgery has been

associated with superficial siderosis (2,9). Although the patient developed symptoms after a long interval, there have been other cases involving a delay of up to 30 years (5). Lumbar puncture may show no red cells, as in the case reported by River et al., and in one instance of su- perficial siderosis after hemispherectomy (10) lumbar puncture was acellular, but tapping the cavity showed 47,000 red cellslmm3. If the patient reported by River et al. were to deteriorate, it may be worthwhile sampling the CSF in the posterior fossa directly and if this were loca- lise the source of bleeding then exploring the area surgi- cally.

Julian Fearnley Peter Rudge Brain Bank

National Hospital f o r Neurology & Neurosurgery London, England

References

1. Braachi M, Savoiardo M, Triulzi F, et al. Superficial sidero- sis of the CNS: MR diagnosis and clinical findings. Am J Neuroradiol 1993; 14:227-236.

2. Janss AJ, Galetta SL, Freese A, et al. Superficial siderosis of the central nervous system: magnetic resonance imaging and pathological correlation. J Neurosurg 1993;79:75&760.

3. Kott E, Bechar M, Bomstein B, Askenasy HM, Sandbank U. Superficial hemosiderosis of the central nervous system. Acta Neurochir 1966; 14:287-298.

4. Koeppen AH, Dentinger MP. Brain hemosiderin and super- ficial siderosis of the central nervous system. J Neuropathol Exp Neurol 1988;47:249-270.

5 . Pames SM, Weaver SA. Superficial siderosis of the central nervous system: a neglected cause of sensorineural hearing loss. Otolaryngology Head Neck Surg 1992;107:69-77.

6. Stevens I, Petersen D, Grodd W, Poremba M, Dichgans J. Superficial siderosis of the central nervous system. A 37- year follow-up of a case and review of the literature. Eur Arch Psychiatry CIin Neurosci 1991;241:5740.

7. Savoiardo M, Grisoli M, Triulzi F, Scotti G, Bradac GB, Agostinis C. MR of superficial siderosis. Am J Neuroradiol 1993;14:1447-1448.

8. Willeit J, Aichner F, Felber S, Berek K, Deisenhammer F, Kiechl SG, Gerstenbrand F. Superficial siderosis of the cen- tral nervous system: report of three cases and review of the literature. J Neurol Sci 1992;111.

9. Kumar A, Agganval S, Willinsky R, TerBrugge KG. Poste- rior fossa surgery: an unusual cause of superficial siderosis. Neurosurgery 1993;32:455-457.

10. Oppenheimer DR, Grifith B. Persistent intracranial bleeding as a complication of hemispherectomy. J Neurol Neurosurg Psychiatry 1 966 ;29: 229-240.

685

686 LETTERS TO THE EDITOR

Apraxia of Lid Opening

To the Editor: I read with interest the recent article by Krack and

Marion (1) in which they contend that apraxia of eyelid opening (ALO) is a focal eyelid dystonia and that botuli- num toxin injections provide the most effective treat- ment. Although I agree that the term apraxia of eyelid opening is “unsatisfactory because the underlying prob- lem is not apraxia,” I feel that Krack and Marion evaded the issue by not providing their own definition and diag- nostic criteria for this disorder. The original definition coined by Goldstein and Cogan (2), “nonparalytic motor abnormality characterized by the patient’s difficulty in initiating the act of lid elevation,” is unsatisfactory be- cause it fails to exclude blepharospasm, probably the most common cause of nonparalytic eye closure. The term focal eyelid dystonia (FED), proposed by the au- thors, is also inappropriate because blepharospasm is considered to be a form of eyelid dystonia and, therefore, FED does not differentiate eye closure due to blepharo- spasm from an inability to open the eyes due to other mechanisms such as ALO. Although it is possible that some patients with inability to open their eyes have iso- lated contractions of the pretarsal orbicularis oculi and the term pretarsal blepharospasm (3) would apply to this group of patients, I suspect that this is not the pathophys- iologic mechanism in most cases of ALO. I prefer the term eyelid freezing (4) because (a) the phenomenon is clinically similar to freezing, or motor blocks, experi- enced by many patients with parkinsonian disorders (5); (b) similar to freezing, it frequently occurs in the setting of atypical parkinsonism and parkinsonism plus syn- dromes (4); and (c) it occasionally improves with levodo- pa (6). Eyelid freezing may or may not be associated with inhibition of the levator palpebrae (7). Krack and Marion do not offer any compelling reason why the term “eyelid freezing” should not be used for this disorder of eyelid motor control.

I also would like to address the proposed technique for injection of botulinum toxin. Krack and Marion state that they “obtained a good functional benefit with botulinum toxin injections into the upper lid directed toward the palpebral part rather than the orbital part of the orbicu- laris oculi.” In the description of their methods, however, they note that the injections were “aimed medially and laterally at the junction of the preseptal and pretarsal parts of the palpebral orbicularis oculi.” They offer no reasons for this modification of their usual treatment pro- tocol, which involved an injection directed at the junction of the orbital and palpebral orbicularis oculi. Further- more, because they did not compare the different meth- ods of injection, there is no basis for their conclusion that “the best results were obtained with injections directed toward the junction of the preseptal and pretarsal parts of the palpebral orbicularis oculi.”

I conducted a “single blind,” controlled study compar- ing the effects of injecting botulinum toxin A (BOTOX) in the medial and lateral segments of the preseptal versus pretarsal portion of the orbicularis oculi in 14 patients

with blepharospasrn. The pretarsal portion of the right eyelid and the preseptal portion of the left eyelid were injected with the same dose of botulinum toxin found to be effective in relieving blepharospasm during previous treatments. The mean dose per eyelid was 10.7 mouse units (range 5-15). There was no difference in response between the two sides: latency, 6.4 days; peak effect, 3.9; mean duration of maximum benefit, 13.5 weeks; mean duration of total benefit, 16.2 weeks. These results were similar to those previously reported by us (8) and by oth- ers (9,lO). However, eight patients reported ptosis in the left eye (preseptal) and none reported ptosis in the right eye (pretarsal) during the follow-up period (3-5 months). The ptosis lasted a mean of 3 1.9 days (range 14-64). The findings from this pilot study suggest that, despite similar response, injections into pretarsal eyelids are associated with lower frequency of ptosis than are injections into the preseptal eyelids. Furthermore, from my personal expe- rience with >300 patients treated for blepharospasm, I have found that pretarsal injections of botulinum toxin are effective in patients with eyelid freezing associated with and “triggered” by blepharospasm, but this treat- ment is usually ineffective in patients in whom eyelid freezing occurs in isolation, without blepharospasm.

Joseph Jankovic Parkinson’s Disease Center and Movement

Disorders Clinic Baylor College of Medicine

Houston, Texas, U.S.A.

References

I .

2.

3 .

4.

5.

6.

7.

8.

9.

10.

Krack P, Marion MH. “Apraxia of lid opening.” A focal eyelid dystonia: Clinical study of 32 patients. Mov Disord 1994;9:610-615. Goldstein JE, Cogan DG. Apraxia of lid opening. Arch Oph- f halmol 1965 ;73: 155-1 59. Elston JS. A new variant of blepharospasm. J Neurol Neu- rosurg Psychiatry 1992;55:36!3-371. Jankovic J , Friedman DI, Pirozzolo FJ, McCrary JA. Pro- gressive supranuclear palsy: motor, neurobehavioral, and neuro-ophthalmic findings. Adv Neurol 1990;53:293-304. Giladi N, Burke RE, Kostic V, et al. Hemiparkinsonism- hemiatrophy syndrome. Neurology 1990;40: 1731-1734. Dewey RB, Maraganore DM. Isolated eyelid-opening apraxia: report of a new levodopa-responsive syndrome. Neurology 1994;44: 1752-1754. Lepore FE, Duvoisin RC. “Apraxia” of eyelid opening: an involuntary levator inhibition. Neurology 1985;35:423-427. Jankovic J , Schwartz K. Longitudinal follow-up of botuli- num toxin injections for treatment of blepharospasm and cervical dystonia. Neurology 1993;43:834-836. Berardelli A, Formica A, Mercuri B, et al. Botulinum toxin treatment in patients with focal dystonia and hemifacial spasm. A multicenter study of the Italian Movement Disor- der Group. Ital J Neurol Sci 1993;14:361-367. Elston JS. Botulinum toxin for blepharospasm. In: Jankovic J, Hallett M, eds. Therapy with botulinum toxin. New York: Marcel Dekker, 1994: 191-198.

Movement Disorders, Voi. 10, No. 5, 1995

LETTERS TO THE EDITOR 687

To the Editor: We thank Dr. Jankovic for his comments on our recent

article. Turning to his second point concerning the site of bot-

ulinurn toxin injections in blepharospasm (BSP), we agree that pretarsal injection is as effective as preseptal injec- tion and is associated with a much lower incidence of postinjection ptosis. We also have modified our injection technique thus because our article was originally submit- ted 2 years ago. He notes also that pretarsal injections are “effective” in patients with “eyelid freezing associated with and triggered by blepharospasm.” This is exactly our experience. We are puzzled, therefore, when faced with this pharmacological response, why Dr. Jankovic considers this fragment of blepharospasm as an “eyelid freezing.”

This brings us his first point. He prefers the term eyelid freezing to focal eyelid dystonia (FED) for the reasons stated in his letter. However, there is definite electro- physiological evidence (1) of typical dystonic patterns of overactivity in the pretarsal orbicularis oculi in FED.

FED is most commonly associated with BSP and re- sponds to botulinum toxin. Hence, we believe that the problem is a focal eyelid dystonia. The term freezing is usually applied to a characteristic difficulty with walking in parkinsonian patients; the pathophysiology and effec- tive treatment of freezing are poorly understood (2). We believe it unjustifiable to transpose the term freezing from the lower limbs to the eyes, and we do not believe that it is an appropriate term for focal eyelid dystonia.

M. H. Marion Clinique Labrouste

Paris, France

P. Krack Department of Neurology

University of Giessen Giessen, Germany

References 1. Elston JS. A new variant of blepharospasm. J Neurol Neu-

rosurg Psychiatry 1992;55:369-37 1. 2. Narabayashi H, Nakamura R. Clinical neurophysiology of

freezing in parkinsonism: In: Delwaide PJ, Agnoli A, eds. Clinical neurophysiology in parkinsonism. Amsterdam, The Netherlands: Elsevier, 1985:49-57.

Movement Disorders, Vol. 10, No. 5 , 1995