LETTER TO THE EDITOR
|Year : 2021 | Volume
| Issue : 6 | Page : 939-941
A case of relapsing - remitting CIDP with sixth nerve palsy
Arikrishnan Thelengana1, Vinay Goyal2
1 Department of Neurology, All India Institute of Medical Sciences, New Delhi; Department of Neurology, Sri Manakula Vinayagar Medical College, Pondicherry, India
2 Department of Neurology, All India Institute of Medical Sciences, New Delhi, India
|Date of Submission||07-Jul-2020|
|Date of Acceptance||14-Apr-2021|
|Date of Web Publication||11-Oct-2021|
Director Neurology, Room No 6, Neurosciences Institute, Medanta The Medicity Gurugram, Haryana
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Thelengana A, Goyal V. A case of relapsing - remitting CIDP with sixth nerve palsy. Ann Indian Acad Neurol 2021;24:939-41
| Introduction|| |
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neurological illness affecting the peripheral nervous system. It has a diversified clinical presentation categorised as monophasic, relapsing-remitting, or chronic progressive. Cranial nerve involvements are not uncommon in CIDP. Cranial nerves are usually irreversibly affected., We report a case of relapsing-remitting CIDP with recurrent sixth nerve palsy.
| Case Summary|| |
A 30-year-old male patient presented with a recurrent neurological deficit for the past 13 years. Initially, he developed bilateral lower limb proximal weakness and about two weeks later developed distal weakness of the lower limbs and proximal weakness of upper limbs followed by distal weakness of upper limbs. Simultaneously the patient also developed paresthesia involving bilateral lower limb followed by upper limb. After one month of onset of illness, symptoms started improving, and in about two months, he recovered completely.
In the next five years, he had five similar events, which improved without any treatment. In the next five years, he was symptom-free. In the past three years, he has had four similar events, in one event in addition to his usual symptoms he also had horizontal diplopia. The diplopia was worse on looking at distance and towards the left side. For the above complaints, he was treated with pulsed steroids, and he improved completely within one week. He was on an oral steroid for six months following which he stopped the treatment on his own. After two months of stopping steroids, he developed horizontal diplopia. The diplopia was worse on looking at distance and towards the right side. He also had paresthesia involving both upper limbs and distal weakness of all four limbs. There was no other cranial nerve involvement. No h/o joint pain/rash/hypopigmented or hypoesthetic skin patches/long-term drug use/toxin exposure. No h/o headache/vomiting/fever/bowel/bladder symptoms/erectile dysfunction/lightheadedness/addiction/any comorbid illness. There was no significant family history. His physical examination showed a right sixth nerve palsy, distal weakness of all four limbs and absent deep tendon reflexes. He had a remarkable response to intravenous pulsed steroid therapy and was discharged on oral steroid therapy and then started on azathioprine in the follow-up. The oral steroid was gradually tapered and stopped.
Motor nerve conduction study revealed reduced velocities and reduced amplitude with prolonged latencies in the right median and right tibial nerve. There were reduced velocity and prolonged latencies with normal CMAP with conduction block in the left median, right peroneal and right tibial nerve. All other nerves tested were not recordable. F waves and sensory nerve conduction study revealed were not recordable in all nerves tested. The nerve conduction study was suggestive of demyelinating sensory-motor polyneuropathy involving all four limbs [Table 1]. CSF study showed albumino-cytological dissociation (cell count 4 lymphocytes, Protein: 98 mg% Sugar: 111.2 mg%). Other routine hemogram and biochemistry, HbA1C, serum protein electrophoresis for M band and serum ACE were normal. Serum viral markers, vasculitis profile, and ELISA for Borrelia burgdorferi and brucella were negative. In MRI brain, 3rd, 5th, and 9th cranial nerves appeared hyperintense on T2W and showed post-contrast enhancement [Figure 1]. X-ray chest, USG abdomen, ECG was normal.
|Figure 1: T1W post contrast images show (a) thickening and enhancement of the left maxillary nerve (b) enhancement of cisternal segment of the left trigeminal nerve (c) enhancement of cisternal segment of the right trigeminal nerve (d) enhancement of right 9th cranial nerve|
Click here to view
| Discussion|| |
A young male patient, without any comorbid illness presented with 13 years history of recurrent lower motor neurone type quadriparesis with sensory involvement without bowel-bladder involvement. He also had two events of lateral rectus palsy (left side followed by right side). Initial events had a spontaneous recovery. Later events were treated with pulsed steroid therapy. The nerve conduction study (NCS) revealed a sensory and motor demyelinating polyneuropathy with conduction block in multiple nerves consistent with CIDP. Cerebrospinal fluid demonstrated albumino-cytologic dissociation. The patient was managed as a case of relapsing-remitting CIDP with recurrent sixth nerve palsy (Left followed by right).
The relapsing-remitting course of CIDP is seen, ranging from 5% to 51% in various studies., It was predominant seen in the juvenile age group. Our patient also had the juvenile onset of symptoms. The prognosis regarding response to IVIg was better in the relapsing-remitting course of CIDP. Our case report describes a rare presentation of CIDP, i.e., relapsing-remitting CIDP with recurrent sixth nerve palsy. Cranial nerve involvement is well reported in CIDP., Ophthalmoplegia in CIDP is seen in about 3-8% of cases in various case series. They might be restricted to cranial nerve involvement or followed by subsequent limb involvement. IgG anti-GQlb gangliosides, which is seen in the Guillain-Barre syndrome with ophthalmoplegia is usually not seen in CIDP. One case of CIDP with recurrent ophthalmoplegia had reported the presence of anti-GM1 antibodies. We did not test for antiganglioside antibody profile in our patient.
In previous case reports of CIDP with cranial nerve involvement, the patients had a complete recovery with IVIg., Our patient also had a full recovery, but with pulsed steroid alone. The published randomised trials and systematic review suggested that pulsed steroids are better than IVIg in attaining remission and lower rates of serious adverse effects.,, The patient was compliant with the treatment of oral steroid. He was started on azathioprine, and the oral steroid was gradually tapered and stopped. The patient is in follow up with us for the past 1.5 years without any new relapse.
| Conclusion|| |
In conclusion, CIDP with cranial nerve involvement needs detailed evaluation, including neuroimaging, to rule out the varied differential diagnosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Dyck PJ, Lais AC, Ohta M, Bastron JA, Okazaki H, Groover RV. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 1975;50:621-37.
Lunn M, Manji H, Choudhary P, Hughes R, Thomas P. Chronic inflammatory demyelinating polyradiculoneuropathy: A prevalence study in south east England. J Neurol Neurosurg Psychiatry 1999;66:677-80.
Maisonobe T, Chassande B, Vérin M, Jouni M, Léger JM, Bouche P. Chronic dysimmune demyelinating polyneuropathy: A clinical and electrophysiological study of 93 patients. J Neurol Neurosurg Psychiatry 1996;61:36-42.
Pieh C, Rossillion B, Heritier-Barras AC, Chofflon M, Landis T, Safran AB. Isolated unilateral adduction deficit and ptosis as the presenting features of chronic inflammatory demyelinating polyradiculoneuropathy. J Neuroophthalmol 2002;22:92-4.
McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy. A clinical and electrophysiological study of 92 cases. Brain 1987;110:1617-30.
McLeod JG, Pollard JD, Macaskill P, Mohamed A, Spring P, Khurana V. Prevalence of chronic inflammatory demyelinating polyneuropathy in New South Wales, Australia. Ann Neurol 1999;46:910-13.
Hattori N, Misu K, Koike H, Ichimura M, Nagamatsu M, Hirayama M, et al
. Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci 2001;184:57-63.
Waddy HM, Misra VP, King RH, Thomas PK, Middleton L, Ormerod IE. Focal cranial nerve involvement in chronic inflammatory demyelinating polyneuropathy: Clinical and MRI evidence of peripheral and central lesions. J Neurol 1989;236:400-5.
Hickman SJ, Allen JA, Baisre A, Batty R, Lari HB, Melen O, et al
. Neuro-ophthalmological complications of chronic inflammatory demyelinating polyradiculoneuropathy. Neuroophthalmology 2013;37:146-56.
Ryo M, Saito T, Kunii N, Hasegawa H, Kowa H. [A case of chronic inflammatory demyelinating polyneuropathy with recurrent ophthalmoplegia, persistent conduction block, antibody activity against gangliosides GM1]. Rinsho Shinkeigaku 1994;34:702-6.
Al-Bustani N, Weiss MD. Recurrent isolated sixth nerve palsy in relapsing-remitting chronic inflammatory demyelinating polyneuropathy. J Clin Neuromuscul Dis 2015;17:18-21.
Trip SA, Saifee T, Honan W, Chandrashekar H, Lunn MP, Yousry T, et al
. Chronic immune sensory polyradiculopathy with cranial and peripheral nerve involvement. J Neurol 2012;259:1238-40.
Press R, Hiew FL, Rajabally YA. Steroids for chronic inflammatory demyelinating polyradiculoneuropathy: Evidence base and clinical practice. Acta Neurol Scand 2016;133:228-38.
van Schaik IN, Eftimov F, van Doorn PA, Brusse E, van den Berg LH, van der Pol WL, et al
. Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): A double-blind, randomised, controlled trial. Lancet Neurol 2010;9:245-53.
Nobile-Orazio E, Cocito D, Jann S, Uncini A, Beghi E, Messina P, et al
. Intravenous immunoglobulin versus intravenous methylprednisolone for chronic inflammatory demyelinating polyradiculoneuropathy: A randomised controlled trial. Lancet Neurol 2012;11:493-502.