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Relapsing alternating ptosis in two siblings
  1. J P SIEB,
  2. A HARTMANN
  1. Department of Neurology, University Hospital, Bonn, D-53105, Germany
  1. Dr JP Sieb jp-sieb{at}t-online.de

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In this Journal, Peatfield described the recurrence of cluster headaches presenting with a virtually painless Horner's syndrome in a 56 year old man.1 This publication caused controversy on the dissociation between autonomic dysfunction and pain during cluster headache.2 We add to this discussion our report on relapsing alternating ptosis in two siblings:

A 46 year old woman had intermittent episodes of alternating ptosis for more than 8 years. Her 47 year old sister was affected similarly. As shown in figure 1, attacks occurred more often than once a month with a mean duration of the episodes of 8 days (range 3–14 days). Intrinsic oculomotor muscles and the bulbar muscles were spared (fig 2). She never complained about double vision. Elevating and maintaining the ptotic eyelid in a fixed position during sustained upward gaze did not result in a drop of the opposite eyelid.3 Signs of autonomic dysfunction and miosis were absent. The ptosis was never accompanied by miosis. There was no history of migraine or cluster headache. However, during the episodes they experienced some mild aching at the frontal region of the affected side. On the serotonin antagonist pizotifen, the younger sister felt improved due to slightly prolonged symptom free intervals. However, 60 mg prednisone every day for 6 weeks did not change the occurrence of ptosis.

Figure 1

Occurrence of ptosis over a period of 21 months (protocol of the 46 year old patient). One eye after the other was affected intermittently (filled bars=presence of ptosis.

Figure 2

Alternating ptosis: representative findings between November 1998 (11/98) and December 1999 (12/99). Note the absence of miosis. The ptosis resolved completely each time.

On repeated neurological examination, there was no abnormality apart from the fluctuating ptosis. Magnetic resonance imaging of the brain, the orbital region, and the cervicothoracic spinal cord segments were normal. Laboratory studies showed no abnormality. Westergren sedimentation rate and serum creatine kinase activity were normal. Repeated tests for antiacetylcholine receptor antibodies were negative. Low rate repetitive nerve stimulation did not result in pathological decremental responses. Thyroid hormones and antibodies were in the normal range and absent, respectively. In the symptom free interval, pupillary responses to various pharmacological agents did not indicate a sympathetic dysfunction.

Relapsing alternating ptosis in two sisters is unique. In some aspects, our observation resembles Bielschowsky's relapsing alternating ophthalmoplegia.4 Distinctive clinical features of this rare syndrome are the intermittent evolution of external ophthalmoplegias, the alternate involvement of one eye after the other, the constant sparing of the intrinsic oculomotor muscles, and the absence of pain.

We think that our finding of relapsing alternating ptosis is related to intermittent sympathetic dysfunction. Interestingly, in a subgroup of patients with cluster headache a “partial” Horner's syndrome may develop during each attack and disappear as the attack subsides. The term “partial” Horner's syndrome indicates that in patients with cluster headache one or two components of the typical Horner's syndrome are present—that is, miosis or ptosis, whereas a third characteristic, anhidrosis, is lacking or even replaced by hyperhidrosis.5 Some of the patients with cluster headache even show a permanent Horner-like syndrome on the symptomatic side.6 Several studies on the pupil responsiveness in patients with cluster headache indicate that dysfunction of the sympathetic nervous system, whether peripheral or central, is involved in the pathophysiology of the cluster headache.6 Additionally, alternating Horner's syndrome has been reported in patients with lesions of the lower cervical und upper thoracic spinal cord segments.7 In those cases, Horner's syndrome may alternate sides at intervals ranging from 2 hours to 2 weeks. Horner's syndrome alternating on a daily basis can occur rarely in multisystem atrophy with dysautonomia.7 Unfortunately, we cannot offer any proof for a sympathetic dysfunction in our patients.

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