{"id":8538,"date":"2024-07-08T12:18:15","date_gmt":"2024-07-08T12:18:15","guid":{"rendered":"https:\/\/www.kauveryhospital.com\/ima-journal\/?p=8538"},"modified":"2025-04-09T10:29:21","modified_gmt":"2025-04-09T10:29:21","slug":"a-rare-form-of-a-spectrum-of-guillain-barre-syndrome","status":"publish","type":"post","link":"https:\/\/www.kauveryhospital.com\/ima-journal\/ima-journal-july-2024\/a-rare-form-of-a-spectrum-of-guillain-barre-syndrome\/","title":{"rendered":"A RARE FORM OF A SPECTRUM OF GUILLAIN BARRE SYNDROME"},"content":{"rendered":"<p class=\"caps\">[vc_row][vc_column][vc_column_text]<span style=\"font-weight: 400;\">A 58-year-old lady presented to ER on 24<\/span><span style=\"font-weight: 400;\">th<\/span><span style=\"font-weight: 400;\"> April 2023 at around 7.30 pm with\u00a0<\/span><\/p>\n<ul>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">History of low back pain x 2 days followed by sudden onset of giddiness, headache,\u00a0<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">double vision involving both eyes with photosensitivity, and\u00a0<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">bilateral numbness of hand and fingers, perioral numbness\u00a0<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">decreased sensation over both feet\u00a0<\/span><\/li>\n<\/ul>\n<h2><b>PAST MEDICAL HISTORY:<\/b><\/h2>\n<ul>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">She is known Diabetic ,\u00a0<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">Guillian barre syndrome-treated 28 yrs back,\u00a0<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">cervical spondylosis and anxiety disorder<\/span><\/li>\n<\/ul>\n<h2><b>INITIAL VITALS &amp; EXAMINATION DONE AT ER<\/b><span style=\"font-weight: 400;\">:<\/span><\/h2>\n<p><span style=\"font-weight: 400;\">She was conscious, oriented, alert, afebrile. Vitals were within normal limits.<\/span><\/p>\n<h2><b>Systemic Examination<\/b><b> :\u00a0<\/b><\/h2>\n<p><b>Respiratory system:<\/b><span style=\"font-weight: 400;\"> B\/L air entry present, NVBS<\/span><\/p>\n<p><b>CVS: <\/b><span style=\"font-weight: 400;\">S1,S2 heard, no murmur, JVP normal<\/span><\/p>\n<p><b>Per abdomen: <\/b><span style=\"font-weight: 400;\">soft, non-tender, no org, BS+\u00a0<\/span><\/p>\n<p><b>Central nervous system:<\/b><span style=\"font-weight: 400;\"> GCS- 15\/15, b\/l pupil reacting to light 3mm sluggish<\/span><\/p>\n<p><b>EOM<\/b><span style=\"font-weight: 400;\">&#8211; bilateral lateral rectus palsy- complete restriction<\/span><\/p>\n<p><span style=\"font-weight: 400;\">\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0Bilateral medial rectus palsy- partial restriction<\/span><\/p>\n<p><span style=\"font-weight: 400;\">\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0Upward and downward gaze restriction<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Bilateral partial ptosis<\/span><\/p>\n<p><span style=\"font-weight: 400;\">No pronator drift\u00a0<\/span><\/p>\n<table>\n<tbody>\n<tr>\n<td><\/td>\n<td><b>RIGHT<\/b><\/td>\n<td><b>LEFT<\/b><\/td>\n<\/tr>\n<tr>\n<td><b>POWER<\/b><span style=\"font-weight: 400;\"> BOTH UL AND LL<\/span><\/td>\n<td><span style=\"font-weight: 400;\">5\/5<\/span><\/td>\n<td><span style=\"font-weight: 400;\">5\/5<\/span><\/td>\n<\/tr>\n<tr>\n<td><b>REFLEXES<\/b><\/td>\n<td><\/td>\n<td><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Biceps<\/span><\/td>\n<td><span style=\"font-weight: 400;\">+<\/span><\/td>\n<td><span style=\"font-weight: 400;\">reduced<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Triceps\u00a0<\/span><\/td>\n<td><span style=\"font-weight: 400;\">+<\/span><\/td>\n<td><span style=\"font-weight: 400;\">reduced<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Supinator\u00a0<\/span><\/td>\n<td><span style=\"font-weight: 400;\">+<\/span><\/td>\n<td><span style=\"font-weight: 400;\">reduced<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Knee\u00a0<\/span><\/td>\n<td><span style=\"font-weight: 400;\">absent<\/span><\/td>\n<td><span style=\"font-weight: 400;\">absent<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Ankle\u00a0<\/span><\/td>\n<td><span style=\"font-weight: 400;\">absent<\/span><\/td>\n<td><span style=\"font-weight: 400;\">absent<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Plantar\u00a0<\/span><\/td>\n<td><span style=\"font-weight: 400;\">mute<\/span><\/td>\n<td><span style=\"font-weight: 400;\">mute<\/span><\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<h2><b>Initial workup and investigations<\/b><b>:<\/b><\/h2>\n<p><span style=\"font-weight: 400;\">MRI BRAIN with MRA and MRV: no evidence of hemorrhage, mass lesion, demyelination.<\/span><\/p>\n<h2><b>Blood Investigation<\/b><span style=\"font-weight: 400;\">:<\/span><\/h2>\n<ul>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">Hb- 13.8\u00a0 WBC- 10,500\u00a0 platelet- 3,98,000 CRP-75<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">UREA-28, CREAT- 0.7<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">NA-136, K-4.5, CL-98<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">S.CORTISOL- 13.4<\/span><\/li>\n<\/ul>\n<p><span style=\"font-weight: 400;\">S.ganglioside, lyme serology, scrub typhus were all negative<\/span><\/p>\n<p><b>EEG<\/b><span style=\"font-weight: 400;\">&#8211; slow wave activity over anterior region<\/span><\/p>\n<p><b>NCS<\/b><span style=\"font-weight: 400;\">&#8211; both motor and sensory axonal demyelination<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Initially patient was admitted in ward during late evening . On the same day over night\u00a0 patient progressed to bilateral complete ptosis, loss of voice and loss of power in both upper limb with preserved muscle power in knee and ankle.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">A diagnosis of Guillian barre syndrome- <\/span><b>MILLER FISHER <\/b><span style=\"font-weight: 400;\">variant suspected.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">With acute onset symptoms and based on clinical examination she was started on IV immunoglobulin (immunogrel) 30 gram\/day x 5 days. Meanwhile she started to desaturate and was immediately shifted to Neuro ICU. There she was maintained with NIV support. Her\u00a0 blood reports showed high white cell count, CRP, procalcitonin, high glucose level, ABG- severe metabolic acidosis and CT chest shows aspiration pneumonia so started on IV broad spectrum antibiotics and DKA correction done.\u00a0<\/span><\/p>\n<p><b>On Day-2 <\/b>T<span style=\"font-weight: 400;\">here was a drop in GCS \u2013 she was intubated after obtaining consent.\u00a0<\/span><\/p>\n<p><b>On Day-4<\/b><span style=\"font-weight: 400;\"> In view of progression of her symptoms and prolonged ventilatory assistance she underwent tracheostomy.\u00a0<\/span><\/p>\n<p><b>On Day-5<\/b><span style=\"font-weight: 400;\"> She developed complete quadriplegia with absence of light and cough reflex.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Due to rapid progression of her symptoms , multidisciplinary discussion was made and decided to initiate plasmapheresis.<\/span><\/p>\n<p><b>On DAY-7<\/b><span style=\"font-weight: 400;\"> Right IJV was secured and first cycle of PLEX was initiated. She underwent 7 cycles of plasmapheresis (alternate days from 30<\/span><span style=\"font-weight: 400;\">th<\/span><span style=\"font-weight: 400;\"> march to 12<\/span><span style=\"font-weight: 400;\">thapril).<\/span><\/p>\n<p><b>On Day- 9<\/b><span style=\"font-weight: 400;\"> Her pupillary response to light and cough reflex was observed. Gradually improvement in her symptoms over the next 2 weeks.<\/span><\/p>\n<p><b>On Day- 17<\/b><span style=\"font-weight: 400;\"> Mild motor activity of eyelid and foot movements, nodding of head, bilateral spontaneous lower limb movements, jaw &amp; trunk movement.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Chair mobilization and physiotherapy was done. Next dose of IV IG was given, eyeball movement improved and initiated lip movements to communicate and gradually improved to being able to talk with speaking valve.\u00a0<\/span><\/p>\n<p><span style=\"font-weight: 400;\">Her cough reflex and respiratory efforts improved and she was weaned off and decannulation done on May 30th. She was tolerating orals.<\/span><\/p>\n<h2><b>Condition at discharge:<\/b><\/h2>\n<ul>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">Sensory perception- intact<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">Bowel and bladder sensation present<\/span><\/li>\n<li style=\"font-weight: 400;\" aria-level=\"1\"><span style=\"font-weight: 400;\">Cranial nerve- intact<\/span><\/li>\n<\/ul>\n<table>\n<tbody>\n<tr>\n<td><\/td>\n<td><b>RIGHT<\/b><\/td>\n<td><b>LEFT<\/b><\/td>\n<\/tr>\n<tr>\n<td><b>POWER<\/b><\/td>\n<td><\/td>\n<td><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Shoulder abduction\/adduction\/<\/span><\/td>\n<td><span style=\"font-weight: 400;\">3+\/5<\/span><\/td>\n<td><span style=\"font-weight: 400;\">3+\/5<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Shoulder flexion\u00a0<\/span><\/td>\n<td><span style=\"font-weight: 400;\">2\/5<\/span><\/td>\n<td><span style=\"font-weight: 400;\">2\/5<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Shoulder extension<\/span><\/td>\n<td><span style=\"font-weight: 400;\">3+\/5<\/span><\/td>\n<td><span style=\"font-weight: 400;\">3+\/5<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Elbow flexion<\/span><\/td>\n<td><span style=\"font-weight: 400;\">1\/5<\/span><\/td>\n<td><span style=\"font-weight: 400;\">1\/5<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Elbow extension<\/span><\/td>\n<td><span style=\"font-weight: 400;\">3+\/5<\/span><\/td>\n<td><span style=\"font-weight: 400;\">3+\/5<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Wrist flexion\/extension<\/span><\/td>\n<td><span style=\"font-weight: 400;\">3\/5<\/span><\/td>\n<td><span style=\"font-weight: 400;\">3\/5<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Finger flexion\/extension<\/span><\/td>\n<td><span style=\"font-weight: 400;\">2\/5<\/span><\/td>\n<td><span style=\"font-weight: 400;\">2\/5<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Hip&amp; knee flexion\/extension<\/span><\/td>\n<td><span style=\"font-weight: 400;\">4\/5<\/span><\/td>\n<td><span style=\"font-weight: 400;\">4\/5<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Ankle dorsiflexion and plantar flexion<\/span><\/td>\n<td><span style=\"font-weight: 400;\">4\/5<\/span><\/td>\n<td><span style=\"font-weight: 400;\">4\/5<\/span><\/td>\n<\/tr>\n<tr>\n<td><b>REFLEXES<\/b><\/td>\n<td><\/td>\n<td><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Biceps\/triceps\/supinator<\/span><\/td>\n<td><span style=\"font-weight: 400;\">mute<\/span><\/td>\n<td><span style=\"font-weight: 400;\">mute<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">Knee\/ankle<\/span><\/td>\n<td><span style=\"font-weight: 400;\">mute<\/span><\/td>\n<td><span style=\"font-weight: 400;\">mute<\/span><\/td>\n<\/tr>\n<tr>\n<td><span style=\"font-weight: 400;\">plantar<\/span><\/td>\n<td><span style=\"font-weight: 400;\">flexor<\/span><\/td>\n<td><span style=\"font-weight: 400;\">mute<\/span><\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p><span style=\"font-weight: 400;\">She was discharged and transferred to rehabilitation centre .<\/span><\/p>\n<h2><b>DISCUSSION<\/b><span style=\"font-weight: 400;\">:<\/span><\/h2>\n<p><span style=\"font-weight: 400;\">Miller Fisher Syndrome (MFS) is a rare, acquired nerve disease that is considered a variant of Guillain-Barr\u00e9 Syndrome (GBS). It typically presents with a triad of symptoms: ophthalmoplegia (paralysis or weakness of the eye muscles), ataxia (lack of voluntary coordination of muscle movements), and areflexia (absence of reflexes).<\/span><\/p>\n<p><b><img loading=\"lazy\" decoding=\"async\" class=\"size-full wp-image-8540 alignnone\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/07\/img.jpg\" alt=\"\" width=\"446\" height=\"211\" srcset=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/07\/img.jpg 446w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/07\/img-300x142.jpg 300w\" sizes=\"auto, (max-width: 446px) 100vw, 446px\" \/><\/b><\/p>\n<p style=\"text-align: left;\"><span style=\"font-weight: 400;\"><img loading=\"lazy\" decoding=\"async\" class=\"size-full wp-image-8544 alignnone\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/07\/img-2.jpg\" alt=\"\" width=\"580\" height=\"237\" srcset=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/07\/img-2.jpg 580w, https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2024\/07\/img-2-300x123.jpg 300w\" sizes=\"auto, (max-width: 580px) 100vw, 580px\" \/><\/span><\/p>\n<p style=\"text-align: left;\"><span style=\"font-weight: 400;\">Ocular Movement showed total restriction on the both eyes<\/span><\/p>\n<h2><b>Key Features of Miller Fisher Syndrome:<\/b><\/h2>\n<p><b><i>Ophthalmoplegia<\/i><\/b><span style=\"font-weight: 400;\">: This involves weakness or paralysis of the muscles that control eye movements, leading to double vision and difficulty moving the eyes<\/span><\/p>\n<p><b><i>Ataxia<\/i><\/b><span style=\"font-weight: 400;\">: Patients experience unsteady gait and coordination problems, making it difficult to walk or perform fine motor tasks<\/span><\/p>\n<p><b><i>Areflexia<\/i><\/b><span style=\"font-weight: 400;\">: Reflexes, especially in the legs, are often absent or significantly diminished.<\/span><\/p>\n<h2><b>Pathophysiology:<\/b><\/h2>\n<p><span style=\"font-weight: 400;\">MFS is believed to be an autoimmune disorder in which the body\u2019s immune system mistakenly attacks its own nerves. The exact cause is unknown, but it is often preceded by a viral or bacterial infection. One common trigger is infection with Campylobacter jejuni, a bacterium that causes Gastroenteritis.<\/span><\/p>\n<h2><b>Diagnosis<\/b><span style=\"font-weight: 400;\">:<\/span><\/h2>\n<p><b><i>Clinical Examination<\/i><\/b><b>:<\/b><span style=\"font-weight: 400;\"> Identifying the classic triad of symptoms is the first step<\/span><\/p>\n<p><b><i>Lumbar Puncture<\/i><\/b><span style=\"font-weight: 400;\">: This may show elevated protein levels in cerebrospinal fluid without a significant increase in white blood cells (albuminocytologic dissociation).<\/span><\/p>\n<p><b><i>Nerve Conduction Studies:<\/i><\/b> <span style=\"font-weight: 400;\">These can show signs of nerve dysfunction.<\/span><\/p>\n<p><b><i>Anti-GQ1b Antibodies<\/i><\/b><b>: <\/b><span style=\"font-weight: 400;\">The presence of these antibodies in the blood supports the diagnosis, as they are found in a significant number of MFS cases.<\/span><\/p>\n<h2><b>Treatment<\/b><span style=\"font-weight: 400;\">:<\/span><\/h2>\n<p><b><i>Intravenous Immunoglobulin<\/i><\/b><i><span style=\"font-weight: 400;\"> (IVIG):<\/span><\/i><span style=\"font-weight: 400;\"> Helps reduce the immune system\u2019s attack on the nervous system.<\/span><\/p>\n<p><b><i>Plasmapheresis<\/i><\/b><i><span style=\"font-weight: 400;\"> (Plasma Exchange): <\/span><\/i><span style=\"font-weight: 400;\">This procedure removes antibodies from the blood.<\/span><\/p>\n<p><b><i>Supportive Care<\/i><\/b><span style=\"font-weight: 400;\">: Managing symptoms such as pain, preventing complications, and providing physical therapy to assist with mobility and coordination.<\/span><\/p>\n<h2><b>Prognosis:<\/b><\/h2>\n<p><span style=\"font-weight: 400;\">The prognosis for MFS is generally good. Most patients experience a significant recovery within weeks to months, although some may have lingering symptoms such as mild coordination issues or residual eye movement abnormalities.<\/span><\/p>\n<p><span style=\"font-weight: 400;\">MFS is a rare and complex condition, so specialized medical care is essential for proper diagnosis and treatment.<\/span><\/p>\n<p><b>Case presentation done by <\/b><b>Dr. Niveda R<\/b><\/p>\n<h2><b>Acknowledgement<\/b><span style=\"font-weight: 400;\">:<\/span><\/h2>\n<p><span style=\"font-weight: 400;\">I would like to thank Dr.Aslesha Vijaay sheth (Head of Emergency Medicine Department)<\/span><\/p>\n<p>&nbsp;<\/p>\n<p><img loading=\"lazy\" decoding=\"async\" class=\"alignleft wp-image-2943 size-thumbnail\" src=\"https:\/\/www.kauveryhospital.com\/ima-journal\/wp-content\/uploads\/2021\/07\/dr.nivetha-150x150.jpg\" alt=\"\" width=\"150\" height=\"150\" \/><\/p>\n<p><strong>Dr. Niveda R<\/strong><br \/>\n<em>Dept. of Emergency Medicine<\/em><br \/>\nKauvery Hospital Chennai[\/vc_column_text][\/vc_column][\/vc_row]<\/p>\n","protected":false},"excerpt":{"rendered":"<p>[vc_row][vc_column][vc_column_text]A 58-year-old lady presented to ER on 24th April 2023 at around 7.30 pm with\u00a0 History of low back pain x 2 days followed by sudden onset of giddiness, headache,\u00a0<\/p>\n","protected":false},"author":2,"featured_media":8539,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[77],"tags":[],"class_list":["post-8538","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-ima-journal-july-2024"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v24.0 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>A RARE FORM OF A SPECTRUM OF GUILLAIN BARRE SYNDROME<\/title>\n<meta name=\"description\" content=\"A 58-year-old lady presented to ER on 24th April 2023 at around 7.30 pm with History 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