Atypical Guillain-Barre Syndrome with Focal Seizures and CSF Pleocytosis: A Rare Entity

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Sir, We present an unusual case of Guillain-Barre Syndrome (GBS) with focal seizures and cerebrospinal fluid (CSF) pleocytosis in the absence of hypertension or radiological abnormalities. GBS, an immune-mediated polyneuropathy, primarily affects the peripheral nervous system, but central nervous system (CNS) involvement remains rare. Reported CNS complications include posterior reversible encephalopathy syndrome (PRES), reversible cerebral vasoconstriction syndrome (RCVS), and dysautonomia, usually linked to the hypertension or characteristic neuroimaging findings, and often present with seizures.[1-3] This case broadens the clinical spectrum of GBS and highlights the importance of recognizing its atypical presentations. A previously healthy 10-year-old boy developed progressive limb weakness following gastroenteritis. He initially experienced lower limb (LL) pain and tingling, which evolved into weakness that later involved the upper limbs (UL). Two weeks before, he experienced episodes of loose stools, which were managed symptomatically. He was initially hospitalized at a local facility for symptomatic treatment; however, due to persistent pain and weakness, he was referred to our center for further evaluation. On examination, the patient was conscious, oriented, and afebrile. Neurological assessment revealed bilateral sixth cranial nerve palsy and lower motor neuron-type seventh cranial nerve palsy, while other cranial nerves remained unaffected. Muscle strength was graded as 2/5 in the UL and 1/5 in the LL. His sensory function was intact, his deep tendon reflexes were absent, and his single-breath count was 10 sec. There was no bowel or bladder dysfunction or any respiratory or bulbar muscle involvement. Higher cognitive functions were within normal limits. Routine investigations, including nerve conduction studies (NCS) were indicative of the acute motor axonal neuropathy (AMAN) variant of GBS, with no evidence of conduction block. CSF analysis revealed pleocytosis without albumin cytological dissociation, an atypical finding for GBS, as detailed in Table 1. The CSF viral panel, including PCR testing for common neurotropic viruses (such as HSV, VZV, CMV, EBV, and enteroviruses), as well as GeneXpert testing for Mycobacterium tuberculosis, returned negative. Routine laboratory investigations, including complete blood count, liver and renal function tests, serum electrolytes, C-reactive protein (CRP), and procalcitonin, were all within normal limits.Table 1: Cerebrospinal fluid analysis of the patientOn second day of hospitalization, the patient developed focal seizures, effectively controlled with levetiracetam. Electroencephalography and MRI brain with screening of spine were normal. Given the absence of hypertension or MRI abnormalities, PRES and RCVS were excluded. The patient showed significant improvement following intravenous immunoglobulin (IVIG) therapy and supportive care. Seizures are rare in GBS, typically reported in association with PRES, RCVS, or metabolic disturbances. Several mechanisms have been proposed, including endothelial dysfunction, cerebral hypoperfusion, and immune-mediated processes. PRES, a reversible neurological syndrome, is frequently associated with acute hypertension, leading to vasogenic edema and seizures. Similarly, RCVS is characterized by transient vasospasms of cerebral arteries, often triggered by hypertensive episodes or immune-mediated responses. PRES and RCVS, share overlapping features, including headaches, altered mental status, and seizures. PRES is also a recognized complication of IVIG therapy, necessitating MRI evaluation in suspected cases. In our patient, the absence of hypertensive episodes and normal neuroimaging findings argue against these diagnoses. CSF pleocytosis is another unusual feature in GBS, as classic cases typically exhibit albumin cytological dissociation. Significant pleocytosis in the CSF raises concerns about alternative diagnoses, such as infectious or inflammatory conditions. However, extensive workup excluded viral, bacterial, and autoimmune etiologies. Previous reports have documented cases of aseptic meningitis post-IVIG administration, but our patient’s CSF abnormalities were noted before IVIG initiation.[4] The presence of pleocytosis in GBS remains poorly understood but may reflect an exaggerated immune response or concurrent CNS involvement.[5] Other potential differential diagnoses, such as CNS infections and autoimmune conditions like sarcoidosis and Sjögren’s syndrome, can present with polyradiculopathy and mild CSF pleocytosis. However, the absence of elevated inflammatory markers, constitutional symptoms (e.g. fever, weight loss), respiratory involvement, a normal chest radiograph, and negative PCR testing of the CSF for neurotropic viruses made these diagnoses unlikely. Assessment for central nervous system autoimmunity, including anti-MOG, anti-GFAP, ganglioside antibodies, and other autoimmune markers would have provided valuable insights for expanding the differential diagnosis, however, these tests were not performed due to financial limitations. Previous literature has described rare CNS manifestations of GBS, including encephalopathy, dystonia, visual disturbances, nystagmus, myoclonus, and cranial neuropathy. Koul et al. reported a 10-year-old girl with the Fisher variant of GBS experiencing recurrent myoclonic seizures.[6] Myokymia and fasciculations have also been observed in GBS, primarily affecting facial and limb muscles. Additionally, cases of opsoclonus and bilateral ballism have been reported. These manifestations suggest a possible CNS component in GBS pathophysiology. The standard management of GBS includes supportive care, respiratory monitoring, thromboprophylaxis, nutritional support, and immunotherapy with IVIG or plasmapheresis. IVIG is administered at a dose of 400 mg/kg/day for 5 days, whereas plasmapheresis is performed in multiple sessions. Both therapies accelerate recovery and reduce the need for mechanical ventilation, when initiated early. Our patient demonstrated remarkable improvement following IVIG treatment, highlighting the effectiveness of timely immunotherapy in GBS management. There are reported cases of seizures occurring in patients with Guillain-Barré syndrome during the course of illness, despite normal neuroimaging and characteristic findings on nerve conduction studies.[7] Awareness of such rare manifestations can facilitate early diagnosis and appropriate management, leading to improved patient outcomes, while preventing unnecessary extensive testing. Further research is needed to elucidate the mechanisms driving CNS involvement in GBS. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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