Correlation of Clinical and Electrophysiological Criteria in the Diagnosis of Guillain-Barré Syndrome: A Prospective Observational Study.

INTRODUCTION: There are many criteria for diagnosing Guillain-Barré syndrome (GBS), and the yield of these diagnostic criteria varies. Each criterion requires some laboratory data and nerve conduction studies (NCS). Although supportive laboratory data are reassuring when present in suspected cases of GBS, when absent, they can potentially cause further delay in diagnosis and treatment. There is no gold standard test for the diagnosis of GBS, and there are multiple diagnostic criteria for GBS to date. The aim of the study is to know the sensitivity of different criteria, clinical and electrophysiological, for the diagnosis of GBS and to study the clinical spectrum and electrophysiological spectrum of GBS in our cohort of patients. MATERIALS AND METHODS: We studied a total of 43 cases who presented with one or more of the following symptoms: relatively symmetrical and progressive flail-type weakness of more than one limb, with or without ataxia and/or ophthalmoplegia, and were diagnosed with GBS according to clinical criteria at the time of admission to Government Stanley Medical College Hospital. GBS mimics were ruled out. In all patients, the demographic data, cerebrospinal fluid (CSF) analysis (if done), and electrophysiological findings fitting into the diagnostic criteria of National Institute of Neurological Disorders and Stroke (NINDS), Dutch, and Brighton criteria were recorded. The need for assisted mechanical ventilation, neurology intensive care unit (NICU) stay, any complications, and treatment outcome details were recorded in a structured proforma. RESULTS: Most of the patients in our study were in their fourth decade of life, with a mean age of 41.37 years, similar to previous studies from India. Men are more frequently affected compared to women, similar to what has been observed in most studies done previously worldwide. In our study, electrophysiological criteria by Dutch criteria (87.5%), Brighton criteria (87.5%), and NINDS criteria (85.6%) had low sensitivity compared to the clinical criteria. CONCLUSION: In the present study, electrophysiological criteria proposed by the NINDS, Dutch, and Brighton criteria are less sensitive compared to clinical criteria in diagnosing GBS at early stages. Clinical criteria alone may be useful in resource-poor countries and at peripheral healthcare systems where NCS are not always readily available.

Neuromyelitis Optica Presenting as Trigeminal Autonomic Cephalgia-Trigeminal Neuralgia (Tic) Overlap.

A 32-year-old lady presented with acute paroxysmal facial pain in the V1-V2 distribution, lower cranial nerve dysfunction, and mild long-tract neurological signs. Paroxysmal facial pain with features of trigeminal autonomic cephalgia (TAC) and trigeminal neuralgia (TN) (Tic) was the unique presentation in this case, explained by involvement of the somatotopically arranged spinal trigeminal nucleus. She had two attacks of area postrema syndrome (APS) preceding the current symptoms, evaluated as a gastroenterological disorder, causing a delay in imaging and AQP4-seropositive diagnosis of neuromyelitis optica spectrum disorder (NMOSD). She recovered from brainstem symptoms with immunosuppression but continued to have painful tonic spasms and neuropathic pain as immediate sequelae. The untreated first attack, AQP4 positivity, and relapse within the 1st year are all risk factors for further relapses and long-term morbidity, as was the case with our patient.

Clinical, Etiological, and Imaging Study of Patients with Cerebral Sinus Venous Thrombosis in a Tertiary Care Hospital.

AIM OF THE STUDY: To study the clinical profile, etiology, and imaging features of subjects presenting with cerebral sinus venous thrombosis (CVST) and to correlate the clinical findings with radiological findings. MATERIALS AND METHODS: The study included 120 patients admitted with the diagnosis of CVST to Stanley Medical College and Hospital, Chennai. The study included patients of all age-groups presenting with cerebral venous thrombosis. The diagnosis was confirmed by imaging studies. RESULTS: The mean age of presentation is 24.4, predominantly males. Headache (90.8%) was the most common presentation, followed by seizures (25%), and paresis (12%). The superior sagittal sinus (SSS) is the most common sinus involved, with multiple sinuses involved in 47% of patients. Magnetic resonance imaging (MRI) brain with magnetic resonance venography (MRV) is the investigation of choice. The most common risk factor for CSVT was alcohol intake, especially in males, and females, anemia, pregnancy, usage of oral contraceptive (OC) pills, and puerperium were the common risk factors. The causes of CSVT in this study were hyperhomocystenemia in 8%, anemia in 13%, and prothrombotic state in 6%. CONCLUSION: Cerebral sinus venous thrombosis is a cause of stroke in young individuals. Even though uncommon, it is underdiagnosed because of its extremely varied clinical presentation. The correct diagnosis of CSVT relies on a high index of suspicion and the availability of advanced imaging like MRI with MR venography.

A Study on Neurological Manifestations in Coronavirus Disease 2019 Patients Admitted to a Tertiary Care Hospital.

AIM: The coronavirus disease 2019 (COVID-19) is considered a pandemic by the World Health Organization (WHO). Although diffuse alveolar damage and acute respiratory failure are the main features of COVID-19, the involvement of other organs needs to be explored. Thus, this study is undertaken to analyze the neurological manifestations in patients with COVID-19 infection. To analyze the neurological manifestations in patients with COVID-19 infection. MATERIALS AND METHODS: All COVID-19-positive patients who got neurology referrals from March 2020 to June 2021 were included in the study. Laboratory confirmation of COVID-19 infection was done by real-time reverse transcriptase-polymerase chain reaction (RT-PCR) of throat swabs in patients who present with symptoms suggestive of COVID-19. Demographic characteristics, neurological complaints, comorbid conditions, neurological examination, and requisite investigations were analyzed. RESULTS: Among 160 patients, 107 (67%) were men, and the mean age was 61 years. Comorbidities included diabetes mellitus (51%) of subjects followed by hypertension (28%), chronic kidney disease (10%), and coronary artery disease (5%). Considering the COVID-19 severity, 28.75% had mild; 8.75% had moderate; and 62.5% had severe disease. The most common neurological symptoms included altered sensorium (62.5%), focal neurological symptoms (29.4%), anosmia (13.1%), headache (10.6%), and seizures (7.5%). The most prevalent neurological signs and/or syndromes were acute encephalopathy (62.5%), stroke (21.3%%), and mucormycosis (12.5%). The mortality rate in our study population was 16.3%, encephalopathy being the most common cause. CONCLUSION: In our study, encephalopathy was the major cause of morbidity and mortality among the COVID-19-related neurological manifestations. Encephalopathy was most seen in severe COVID-19 infection and was associated with increased neutrophil-to-lymphocyte (NL) ratio raised inflammatory markers. Stroke constituted 29.4% of the neurology referrals in COVID-19 patients confirming COVID-19 infection predisposes to thrombotic events. We found an increased incidence of Mucormycosis in COVID-19 patients, but early debridement and timely treatment with antifungal medications had reduced the mortality.

Spinocerebellar ataxia 46 in a young female.

Spinocerebellar ataxias (SCAs) are a group of both clinically and genetically heterogeneous neurodegenerative disorders. SCA 46 is a rare autosomal dominant ataxia initially described in a Dutch family, clinically characterized by ataxia, peripheral neuropathy, cerebellar dysarthria, and varied oculomotor abnormalities. SCA 46 has recently been discovered to be associated with a mutation in phospholipase D 3 gene.

AMAN with Ophthalmoparesis: A Rare Presentation.

Acute motor axonal neuropathy (AMAN) is a variant of Guillain-Barré syndrome (GBS), characterized by acute areflexic flaccid quadriparesis with motor axonal changes and absence of demyelinating findings in electrophysiological studies. A 30-year-old man presented with acute onset flaccid type of weakness involving all four limbs, along with drooping of eyelids. Examination revealed ptosis with restricted horizontal and vertical eye movements. Spinomotor system examination revealed acute flaccid areflexic quadriparesis. Nerve conduction studies (NCS) showed features suggestive of motor axonal neuropathy changes. Cerebrospinal fluid (CSF) revealed albuminocytological dissociation. The diagnosis of AMAN was made, and the patient was treated with intravenous immunoglobulin (IVIg). His weakness gradually improved over 1 month, with partial improvement in ptosis and eye movements. This case highlights the occurrence of ophthalmoparesis in the AMAN variant of GBS. The presence of ophthalmoparesis and areflexia makes it necessary to exclude Miller-Fisher syndrome. But, the presence of axonal changes in nerve conduction study and the profound weakness with negative serum anti-GQ1b antibody profile, supports the diagnosis of AMAN. How to cite this article: Budumuru U, Muralidharan K, Sowmini PR, et al. AMAN with Ophthalmoparesis: A Rare Presentation. J Assoc Physicians India 2023;71(11):103-104.

An Unusual Case of Recurrent Raised Intracranial Pressure Headache.

INTRODUCTION: Raised intracranial pressure (ICP) can be due to varied etiology. Differentiating among these various etiologies is crucial in making appropriate therapeutic decisions. A patient with a known past history of the primary or secondary headache of any etiology, when presenting with new onset severe headache, needs to be evaluated with imaging to rule out an alternative diagnosis. DISCUSSION: Here, we describe the case details of a young lady who presented with recurrent raised ICP headaches due to three different etiologies. At her third visit, isolated intracranial hypertension (IH) was the only manifestation of cerebral venous sinus thrombosis (CVST), which could have been missed if a repeat magnetic resonance imaging (MRI) brain and venogram were not done. CONCLUSION: Our case highlights the importance of having a high degree of suspicion for CVST in the clinical setting of raised ICP headache in view of its crucial therapeutic implications.

Myokymia in a Patient with Chronic Inflammatory Demyelinating Polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired, immune-mediated neuropathy affecting peripheral nerves and nerve roots. It is characterized by symmetric weakness involving both proximal and distal muscles; it can be relapsing-remitting or progressive in course. The clinical manifestations of CIDP are various and may present with atypical features, like myokymia, tremor, or tremor-like phenomena, which may mislead the clinician in diagnosis.

Management of Dyslipidaemia for the Prevention of Stroke: Clinical Practice Recommendations from the Lipid Association of India.

Stroke is the second most common cause of death worldwide. The rates of stroke are increasing in less affluent countries predominantly because of a high prevalence of modifiable risk factors. The Lipid Association of India (LAI) has provided a risk stratification algorithm for patients with ischaemic stroke and recommended low density lipoprotein cholesterol (LDL-C) goals for those in very high risk group and extreme risk group (category A) of <50 mg/dl (1.3 mmol/l) while the LDL-C goal for extreme risk group (category B) is ≤30 mg/dl (0.8 mmol/l). High intensity statins are the first-line lipid lowering therapy. Nonstatin therapy like ezetimibe and proprotein convertase subtilisin kexin type 9 (PCSK9) inhibitors may be added as an adjunct to statins in patients who do not achieve LDL-C goals with statins alone. In acute ischaemic stroke, high intensity statin therapy improves neurological and functional outcomes regardless of thrombolytic therapy. Although conflicting data exist regarding increased risk of intracerebral haemorrhage (ICH) with statin use, the overall benefit risk ratio favors long-term statin therapy necessitating detailed discussion with the patient. Patients who have statins withdrawn while being on prior statin therapy at the time of acute ischaemic stroke have worse functional outcomes and increased mortality. LAI recommends that statins be continued in such patients. In patients presenting with ICH, statins should not be started in the acute phase but should be continued in patients who are already taking statins. ICH patients, once stable, need risk stratification for atherosclerotic cardiovascular disease (ASCVD).