Multiple Cranial Nerve Palsies Without Limb Weakness: A Rare Cranial Variant of Guillain-Barré Syndrome.

We herein report a case of an unusual variant of Guillain-Barré syndrome (GBS) where the patient presented with multiple bilateral cranial nerve palsies involving nerves V, VII, IX, and X, leading to difficulties with eye closure, eyebrow-raising, chewing, swallowing, and speech. Sensation and motor examination were normal. Bilateral knee reflexes were absent. Lumbar puncture showed cerebrospinal fluid albuminoid-cytologic dissociation. Prompt initiation of plasmapheresis therapy facilitated a successful recovery. This case report underscores the significance of early identification and tailored intervention for atypical GBS presentations, highlighting the potential for improved patient outcomes through targeted management strategies.

Elsberg Syndrome With Albuminocytologic Dissociation - A Guillain-Barré Syndrome Mimic or Guillain-Barré Syndrome Variant?

Introduction: Elsberg Syndrome is a presumed infectious lumbosacral radiculitis, with or without accompanying lumbar myelitis, that is often attributed to herpes simplex virus type 2 (HSV-2). Case: A 58-year-old man presented with lower extremity anesthesia, ataxic gait, radiological evidence of radiculitis, and CSF albuminocytologic dissociation. Polymerase chain reaction testing of CSF confirmed HSV-2 infection. Conclusion: A variety of presentations are reported within the scope of Elsberg Syndrome, potentially with distinct disease mechanisms. Delayed onset of neurological symptoms after resolution of rash and absence of pleocytosis raises the possibility that some patients meeting criteria for Elsberg Syndrome have a post-infectious immune-mediated neuropathy. We advise a lower threshold for PCR testing of herpes viruses in patients with acute neuropathy and albuminocytologic dissociation, particularly in cases with early sacral involvement.

An Atypical Pediatric Presentation of a Chronic Polyradiculoneuropathy.

Here, we present a case of a 15-year-old male with polyradiculoneuropathy, which was diagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP), who was refractory to initial treatment. The patient presented with a one-and-a-half-month history of decreased strength, most notable in the bilateral hip flexors and finger flexors/extensors, and areflexia. Electromyography and nerve conduction studies did not fulfill diagnostic criteria for a demyelinating polyneuropathy; however, the cerebrospinal fluid analysis demonstrated albuminocytologic dissociation and his physical exam was otherwise consistent with the diagnosis. He was treated with IV immunoglobulin (IVIg). He relapsed less than one month later with worsening weakness. Imaging revealed increased cauda equina enhancement when compared to the MRI from the previous admission, and labs were otherwise similar to the initial presentation. He was treated with a second course of IVIg in addition to high-dose IV methylprednisolone. Upon his second discharge, he was transitioned to oral corticosteroids, and at a follow-up visit one month later, he had fully regained his strength and demonstrated normal reflexes. This case highlights the variable nature of CIDP in its initial presentation, its course, and its response to treatment, particularly in young patients. Additionally, we would like to emphasize that this case of CIDP was in the context of chronic malnutrition and significant weight loss, which made the diagnostic picture more complex.

Neuroborreliosis Presenting as Guillain-Barré Syndrome.

Lyme disease (LD) is the most common vector-borne disease in the United States. The early localized disease presents with erythema migrans and nonspecific constitutional symptoms. A neurological manifestation of LD (neuroborreliosis) is only seen in 10-15% of LD cases, and it typically presents as cranial neuritis or painful radiculitis. We report a case of a 33-year-old male who presented with progressive ascending bilateral lower extremities weakness with paresthesia in hands and feet following an upper respiratory tract infection and an abdominal rash. Cerebrospinal fluid (CSF) analysis revealed albuminocytologic dissociation. An electrodiagnostic study showed prolonged distal motor latency, conduction block, and absent F-wave response. Magnetic resonance imaging of the lumbar spine revealed enhancement of the cauda equina nerve roots. After a lack of improvement with intravenous immunoglobulin for presumed Guillain-Barré syndrome (GBS), Lyme serologies were sent and showed positive Lyme antibodies in serum and CSF as well as positive western blot IgM followed by IgG seroconversion a week later. The patient was started on IV ceftriaxone and doxycycline for four weeks with significant improvement in his symptoms. This is a rare case of LD presenting as GBS. Lyme can have diverse neurologic manifestations and should be considered in the differential diagnosis of GBS in the appropriate settings.

COVID-19 Infection and Guillain-Barre Syndrome: A Case Series.

The coronavirus disease 2019 (COVID-19) pandemic brought about an unprecedented time. Multiple systemic complications have been recognized with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) as it can do much more than affect the respiratory system. One of the intriguing neurological complications is Guillain-Barre syndrome (GBS). We reviewed three cases in which patients presented with GBS following COVID-19 infection. All three cases had positive lumbar puncture results with albumino-cytological dissociation. Each patient was treated with plasmapheresis and improved clinically. Although an exact causal relationship between COVID-19 and GBS cannot be drawn from this case series alone, it signifies the importance of this complication. It warrants further studies to establish the causal relationship. One should have a high suspicion for acute inflammatory demyelinating polyneuropathy (AIDP) in patients presenting with acute onset of ascending weakness following COVID-19 infection.

Study of Guillain-Barre syndrome etiology in Pakistani patients.

OBJECTIVE: To examine clinical features, biochemical markers, demographic features, antecedent infections, frequency and treatment strategies related to Guillain-Barré syndrome. METHODS: The case-control study was conducted at the Pakistan Institute of Medical Sciences, Islamabad, Pakistan, and the District Headquarters Hospital, Rawalpindi, Pakistan, from 2018 to 2020, and comprised Guillain-Barré syndrome patients in group A and healthy controls in group B. The patients were diagnosed on the basis of clinical presentation, nerve conduction study, electromyography, cerebrospinal fluid analysis and biochemical profile. Data was analysed using SPSS 23. RESULTS: Of the 167 subjects, 90(54%) were in group A and 77(46%) were in group B. The mean age of group A was 40.20±14.90 years, while there were 61(67.7%) males and 29(32.2%) females compared to 50 (64.93%) males and 27 (35.06%) females with mean age 38.40±12.34 years in group B. Acute inflammatory demyelinating polyneuropathy was the most common electrophysiological variant of Guillain-Barré syndrome 41(46%). There was significant difference in mean interleukin-17 levels between group A 23.12±3.41 pg/ml and group B 8.82±2.49 (p<0.05). Gastrointestinal infection was the most common preceding infection 51(56.66%). The mean cerebrospinal fluid protein was 100.83±51.32g/dl and albumiocytologic dissociation was found in all the four variants (p= 0.005). CONCLUSION: Guillain-Barré syndrome affected patients regardless of age, while males were more affected than females. Majority of the patients had an antecedent infection before disease onset. Increased levels of interleukin-17 showed involvement of autoimmunity. Albuminocytologic dissociation differentiated it from poliomyelitis.

West Nile Neuroinvasive Disease With Atypical CSF Findings: A Case Report.

West Nile Neuroinvasive Disease (WNND) is a rare complication of West Nile Virus infection with the capability of mimicking other neurologic diseases. This infection should be considered in the differential diagnosis for patients presenting in the late summer months with altered mentation, fever, and focal neurologic deficits without an otherwise clear etiology. A 63-year-old male presented with acute onset fever, confusion, falls, ataxia, vertical nystagmus, and right leg weakness. Although magnetic resonance imaging of the brain and cervical spine were unremarkable, the lumbar spine revealed enhancement of ventral nerve roots in the cauda equina. Cerebrospinal fluid (CSF) analysis was significant for elevated protein without pleocytosis, which was more suggestive of albuminocytologic dissociation. Both serum and CSF IgM labs testing for West Nile Virus were positive. Despite a 5 day course of immunoglobulin therapy, his symptoms did not significantly improve. He eventually was transferred to inpatient rehabilitation for several days prior to returning home. This case highlights the variable presentations of acute West Nile Virus infection in the rare setting of neuroinvasive disease, which can make diagnosis difficult. The CSF analysis may also not always show results consistent with an acute viral infection, which can make determining the underlying etiology more challenging.

Cerebrospinal fluid analysis in Guillain-Barré syndrome: value of albumin quotients.

BACKGROUND: Albuminocytologic dissociation in cerebrospinal fluid (CSF) is a diagnostic hallmark of Guillain-Barré syndrome (GBS). Compared to CSF total protein (TP), the CSF/serum albumin quotient (Qalb) has the advantage of method-independent reference ranges. Whether the diagnostic yield differs between Qalb and CSF-TP is currently unknown. METHODS: We retrospectively analyzed the diagnostic yield (i.e., a value above the URL indicating blood-nerve barrier dysfunction) of Qalb and CSF-TP levels in patients with GBS. We evaluated two different equations (Reiber's and Hegen's) for age-adjusted URLs of Qalb and compared results to CSF-TP using the standard URL of 0.45 g/L as well as age-adjusted URLs (by decade of age). Additionally, multivariable logistic regression analysis was used to assess the effect of clinical factors on the diagnostic yield. RESULTS: We analyzed 110 patients [62% males; median age 48 (IQR 35-58)] with sensorimotor (68), motor (16), sensory (12) and localized (8) GBS as well as Miller Fisher syndrome (6). Qalb and CSF-TP were highly correlated (r = 0.95, p < 0.001). The diagnostic yield of Qalb was 65% with Reiber's and 47% with Hegen's age-adjusted URLs compared to 66% with the fixed CSF-TP URL of 0.45 g/L and 49% with age-adjusted CSF-TP URLs. A longer duration from clinical onset to lumbar puncture was associated with a higher diagnostic yield. CONCLUSION: Qalb strongly correlates with CSF-TP in patients with GBS with a similar diagnostic yield for the detection of a blood-nerve barrier dysfunction. However, the diagnostic yield of both values is lower when using more recent age-adjusted URLs and at earlier timepoints.

Cerebrospinal fluid total protein in Guillain-Barré syndrome variants: correlations with clinical category, severity, and electrophysiology.

The discriminative value of CSF total protein (CSF-TP) in subtypes of Guillain-Barré syndrome has not been well documented in North-American patients. We reviewed 173 cases from a single institution, comprising the following clinical categories of neuropathy: 134 Sensorimotor (SM) GBS, 13 Motor (M) GBS, 8 Localized (L) GBS, and 18 Miller Fisher syndrome (MFS). We grouped the electrophysiological interpretation in primarily demyelinating, primarily axonal and normal / equivocal categories. Mean CSF-TP were substantially higher for SM and L-GBS, as well as cases classified as Acute-onset chronic inflammatory demyelinating polyneuropathy. They were lower for M-GBS and L-GBS. The most statistically significant correlation was found for elevated CSF-TP in GBS cases showing an electrophysiologic pattern classified as demyelinating (1.56 g/L) compared with axonal (0.68 g/L) or normal/ equivocal patterns (0.65 g/L). There was a correlation between CSF-TP and time interval between symptom onset and lumbar puncture. There was a weak correlation between CSF-TP and maximal overall-clinical severity grade, which was likely mostly determined by the electorphysiological pattern. Though CSF-TP is a sensitive test for GBS in the second week after onset, it may not be a reliable predictor of clinical severity. There is a robust association of CSF-TP elevation and a demyelinative electrophysiologic pattern and a suggestion that lower mean CSF-TP values can be expected in GBS-spectrum disorders thought to represent nodo-paranodopathies.

Progressive flaccid paraparesis with albuminocytologic dissociation: It's not always Gullain-Barre syndrome.

CONTEXT: Spondylodiscitis, or vertebral osteomyelitis, is an unusual infection of the vertebral bodies and intervertebral discs that can occasionally present with neurological signs. FINDINGS: We present a patient with subacute flaccid paraparesis with associated albuminocytologic dissociation who was eventually diagnosed with spondylodiscitis. CONCLUSION: The case presented depicts a diagnostic difficulty encountered in clinical practice: Albuminocytologic dissociation in CSF is not always attributed to Guillain-Barre syndrome and other possible causes such as obstructive spinal cord lesions must always be considered.

Adult CSF total protein: Higher upper reference limits should be considered worldwide. A web-based survey.

BACKGROUND: The cerebrospinal fluid total protein level (CSF-TP) is commonly used as a potential marker of infectious or immune disease of the CNS and PNS. Recent laboratory reference studies indicate that the antiquated single upper reference limit of 0.45 g/L commonly used by hospital laboratories and widely quoted in medical literature is a significant underestimation. METHODS: We distributed worldwide a web-based survey comprised of three questions: 1. What is the CSF-TP upper limit used at your institution? 2. What is the source of this upper limit? 3. Do you adjust your upper limit according to age? RESULTS: A total of 473 unique responses were obtained from North America (37.5%), South America (5.5%), Europe (29.4%), Africa (4%), Asia (21.6%) and Oceania (1.7%). A strong preponderance (86.8%) of institutions reported an upper limit of 0.45 g/L or less. Only 4% reported making age-partitioned adjustments. CONCLUSIONS: Worldwide, a strong majority of hospital laboratories presently use an underestimation of CSF-TP upper reference value, particularly for older adults. Recent well powered laboratory reference studies support higher values with age adjustment.

Spontaneous spinal epidural hematoma mimicking Guillain-Barre Syndrome.

BACKGROUND: The initial symptoms of Guillain-Barre Syndrome (GBS) can be similar to a case of spontaneous spinal epidural hematoma (SSEH) located at the cervicothoracic junction. Therefore, SSEH may be misdiagnosed as GBS. CASE REPORT: A previously healthy 6-year-old girl presented with a 2-day history of progressive pain in the lower extremities and an inability to walk. On initial evaluation, she was completely paraparetic in the lower extremities. Deep tendon reflexes were absent in the lower extremities, and Babinski reflexes were positive on both sides. She exhibited reduced response to light touch and pinprick with a sensory level below T10, and experienced difficulty during urination. However, the strength, sensation and flexion of upper extremities were normal. Because her presentation and examinations were consistent with GBS, we initiated intravenous immunoglobulin therapy. The next day, she also developed pain and muscle weakness of the right upper extremity. Three days after admission, respiratory depression progressed rapidly. Spinal MRI showed a mass extending from the level of C7-T3, with spinal cord compression. The patient underwent an emergency laminectomy with evacuation of hematoma, and was diagnosed with SSEH. Sixty days after admission, she was transferred to the rehabilitation hospital with severe neurologic sequelae of paralysis in both legs. CONCLUSION: SSEH might have severe consequences, including neurologic deficits and risk of death. This case report serves to raise the awareness of SSEH that mimics the initial presentation of GBS.

Orthostatic Hypotension and Albuminocytologic Dissociation as Primary Manifestations of the Paraneoplastic Syndrome.

BACKGROUND: Orthostatic hypotension (OH) is the key manifestation of autonomic dysfunction with many causes. Systemic neurological causes such as paraneoplastic syndrome are usually ignored. METHODS: We retrospectively analyzed clinical and examination data of 2 patients who were hospitalized, with onset symptom of OH and who were diagnosed as paraneoplastic syndrome. RESULTS: The patients were characteristic of an initial symptom of OH, positive anti-Hu antibody and albuminocytologic dissociation in the cerebrospinal fluid. Patient 2 died and Patient 1 worsened during follow-up. CONCLUSIONS: The diagnosis of paraneoplastic syndrome is usually neglected when the onset symptoms are autonomic dysfunctions such as OH. Neurologists should improve their knowledge to diagnose accurately.

Central-Variant Posterior Reversible Encephalopathy Syndrome with Albuminocytologic Dissociation.

Posterior reversible encephalopathy syndrome (PRES) is a disorder of reversible vasogenic brain edema which mainly involves the parieto-occipital lobes in various clinical settings. The main mechanism is known to be cerebral autoregulation failure and endothelial dysfunction leading to the disruption of the blood-brain barrier. We report the case of a 47-year-old woman with PRES which involved the brain stem and thalami, sparing the cerebral hemispheres. She was admitted to the emergency room because of acute-onset confusion. Her initial blood pressure was 270/220 mm Hg. Routine blood lab tests showed pleocytosis, hyperglycemia, and azotemia. Brain magnetic resonance imaging (MRI) showed a lesion of vasogenic edema involving nearly the whole area of pons, the left side of the midbrain, and the bilateral medial thalami. Cerebrospinal fluid (CSF) examination revealed an increased level of protein with normal white blood cell count. With conservative care, the patient markedly recovered 3 days after symptom onset, and a follow-up MRI confirmed complete resolution of the vasogenic edema. This case suggests that PRES can rarely involve the "central zone" only, sparing the cerebral hemispheres, which may be confused with other neurological diseases. Besides, the CSF albuminocytologic dissociation may suggest the disruption of the blood-brain barrier in patients with PRES.

Autonomic Neuropathy and Albuminocytologic Dissociation in Cerebrospinal Fluid As the Presenting Features of Primary Amyloidosis: A Case Report.

OBJECTIVE: Primary amyloidosis is a disease with a poor prognosis and multi-organ involvement. Here, we report the clinical and pathological features of a patient with primary amyloidosis featuring autonomic neuropathy as the initial symptom and albuminocytologic dissociation in the cerebrospinal fluid (CSF). METHODS: The patient was a 60-year-old Chinese male with numbness, orthostatic hypotension, and gastrointestinal symptoms. For diagnosis, we performed an electromyogram (EMG), lumbar puncture, Bence Jones protein urine test, serum electrophoresis blood test, sural nerve and rectal membrane biopsies, transthyretin (TTR) gene sequencing, and bone marrow puncture. RESULTS: Congo red staining of sural nerve and rectal membrane biopsies showed amyloid deposition and apple-green birefringence was visualized under polarized light microscopy. TTR gene sequencing showed no causative mutation. Following lumbar puncture, normal CSF cell counts and elevated CSF protein concentration (1,680 mg/L) were detected. Bone marrow puncture showed that out of the total number of whole blood cells, 0.56% were abnormal plasma cells and that 87.4% of the total number of plasma cells were abnormal. EMG results showed mixed peripheral nerve damage predominately in the sensory nerve fibers. CONCLUSION: Obvious symptoms of neuropathy, particularly autonomic neuropathy, albuminocytologic dissociation, and organ function damage suggested a diagnosis of amyloidosis. In such patients, neurologists should use caution to differentiate between chronic inflammatory demyelinating polyneuropathy, primary amyloidosis, and familial amyloid neuropathy.

An unusual case of recurrent Guillain-Barré syndrome with normal cerebrospinal fluid protein levels: a case report.

BACKGROUND: Guillain-Barré syndrome is an acquired polyradiculo-neuropathy, often preceded by an antecedent event. It is a monophasic disease but a recurrence rate of 1-6 % is documented in a subset group of patients. Patients with Guillain-Barré syndrome show cerebrospinal fluid albuminocytologic dissociation. Normal cerebrospinal fluid protein levels during both initial and recurrent episodes of Guillain-Barré syndrome is a rare occurrence and has not been described earlier in the literature. CASE PRESENTATION: Twenty-five-year-old Sri Lankan female with past history of complete recovery following an acute inflammatory demyelinating polyneuropathy (AIDP) variant of Guillain-Barré syndrome 12 years back presented with acute, ascending symmetrical flaccid quadriparasis extending to bulbar muscles, bilateral VII cranial nerves and respiratory compromise needing mechanical ventilation. Nerve conduction study revealed AIDP variant of Guillain-Barré syndrome. Cerebrospinal fluid analysis done after 2 weeks were normal during both episodes without albuminocytologic dissociation. She was treated with intravenous immunoglobulin resulting in a remarkable recovery. Both episodes had a complete clinical recovery in three and four months' time respectively, rather a faster recovery than usually expected. CONCLUSION: Recurrence of Guillain-Barré syndrome can occur in a subset of patients with Guillain-Barré syndrome even after many years of asymptomatic period. Normal cerebrospinal fluid profile does not exclude Guillain-Barré syndrome and may occur in subsequent recurrences of Guillain-Barré syndrome arising the need for further studies to identify the pathophysiology and the possibility of a different subtype of Guillain-Barré syndrome.