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Year : 2010  |  Volume : 58  |  Issue : 5  |  Page : 790-791

Chronic progressive external ophthalmoplegia with recurrent quadriparesis : An unusual presentation

1 Department of Neurology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

Date of Acceptance26-Oct-2010
Date of Web Publication28-Oct-2010

Correspondence Address:
K Prasad
Department of Neurology, All India Institute of Medical Sciences, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.72176

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How to cite this article:
Rai N K, Sharma M C, Prasad K, Jetli S, Tripathi M, Padma M V. Chronic progressive external ophthalmoplegia with recurrent quadriparesis : An unusual presentation. Neurol India 2010;58:790-1

How to cite this URL:
Rai N K, Sharma M C, Prasad K, Jetli S, Tripathi M, Padma M V. Chronic progressive external ophthalmoplegia with recurrent quadriparesis : An unusual presentation. Neurol India [serial online] 2010 [cited 2022 Sep 28];58:790-1. Available from: https://www.neurologyindia.com/text.asp?2010/58/5/790/72176


A 32-year-old lady, product of nonconsanguineous marriage, with normal perinatal and developmental history presented with history of nonprogressive, mild proximal limb-girdle weakness and mild exercise intolerance since childhood. She was referred from another hospital with history of three episodes of acute pure motor flaccid quadriparesis, with near-complete recovery in the last four years. First episode occurred four years back, on postpartum day-3, when she developed acute flaccid quadriplegia, which progressed for two days, without unconsciousness; and without cranial nerves, sensory or sphincter involvement. On third day, she developed breathlessness and received intravenous immunoglobulins (IVIg) 2 g/kg over five days. She improved over 6 to 8 weeks and achieved independent activities of daily living ADL in the next three months. Two years later, she developed high-grade fever, followed by similar pure motor quadriplegia; and required mechanical ventilation for the next two months, followed by gradual improvement. Her last episode occurred six months back, similar to previous episodes. There was no history suggestive of diurnal variation in power, muscle cramp, fasciculations, rashes, joint pain, abdominal pain or myoglobinuria or other comorbid illness. Family history was noncontributory. Her routine biochemical parameters, including serum potassium during episode, were normal.

Examination revealed mild bilateral ptosis and extra-ocular muscles weakness in all directions, without diplopia. Neck flexors were weak and also mild limb-girdle weakness without differential involvement. Sensory examination was unremarkable. Deep tendon reflexes were reduced and Babinski's reflexes were normal. Routine hematological and biochemical parameters, including serum lactate, creatine phosphokinase and thyroid profile, were within normal limits. Nerve conduction study, concentric needle electromyography and repetitive nerve stimulation test were unremarkable. Echocardiography showed asymmetrical septal hypertrophy without outflow obstruction. Magnetic resonance imaging (MRI) brain and ultrasonography of abdomen were normal. Muscle biopsy specimens were taken from left vastus lateralis. Microscopic examination showed minimal variation in fiber size without any endomysial and perimysial fibrosis. Occasional atrophied and bluish fibers were seen [Figure 1]a. Histochemistry for ATPase at different pH levels showed normal fiber typing. Oxidative stains revealed subsarcolemmal accentuation of staining [Figure 1]b and c. Numerous COX-negative fibers were seen. A significant number of ragged red fibers were present [Figure 1]d. Ultrastructural examination revealed variation in the mitochondrial size with para-crystalline inclusions, at places showing characteristic Zipper-like pattern [Figure 2]. Pathological diagnosis was mitochondrial myopathy.
Figure 1 :Histopathology of muscle showing no endomysial and perimysial fibrosis with occasional atrophied and bluish fibres (Figure 1a, H and E stain). Oxidative stains revealed subsarcolemmal accentuation of staining (Figure 1b, COX with SDH and Figure 1c, NADH-TR). Numerous COX negative fibres were seen. A significant number of ragged red fibres were present (Figure 1d, MGT stain)

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Figure 2 :Electron microscopy showing variation in the mitochondrial size with para crystalline inclusions, along with characteristic zipper like pattern

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Our patient had mild proximal weakness with progressive external ophthalmoplegia (PEO), cardiac septal hypertrophy and presence of ragged red fibers (RRF) or ragged blue fiber (SDH equivalents) in muscle biopsy. These features were suggestive of mitochondrial myopathy, which was further supported by ultrastructural findings. Unusual presentation in our case was respiratory involvement and recurrent relapses. Respiratory involvement is not a common feature in mitochondrial myopathy [1] but has been reported rarely in PEO and Leigh's disease. [2] To the best of our knowledge, recurrent quadriplegia due to muscle involvement, requiring prolonged ventilator support, has not been described in mitochondrial myopathy. The most appropriate clinical syndrome in the present patient would be " sporadic progressive external ophthalmoplegia with ragged red fibers with relapses (sporadic PEO with RRF with relapses) ." Present case adds relapsing quadriplegia to the already known wide clinical spectrum of mitochondrial myopathy.

  Acknowledgment Top

We thank Dr. U. K. Misra, Professor & Head, Department of neurology, SGPGIMS, Lucknow for referring this patient to us.

  References Top

1.Howard RS, Wiles CM, Hirsch NP, Spencer GT. Respiratory involvement in primary muscle disorder: assessment and management. Q J Med 1993;86:175-89.   Back to cited text no. 1
2.Carlayne E, Jackson MD. A clinical approach to muscle disease. Semin Neurol 2008;28:228-40.  Back to cited text no. 2


  [Figure 1], [Figure 2]


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