Showing papers in "Case Reports in Neurology in 2022"

Parsonage-Turner Syndrome Following COVID-19 Vaccination: Clinical and Electromyographic Findings in 6 Patients

Abstract: Parsonage-Turner syndrome (PTS) following COVID-19 infection or vaccination is rare. The pathophysiology may involve an immune-mediated inflammatory reaction against brachial plexus nerve fibers in a genetically predisposed individual. We describe the clinical and electromyographic features of 6 patients presenting with the clinical picture of PTS following COVID-19 vaccination. All patients were referred for electromyographic studies to evaluate the acute onset of pain in the shoulder girdle/upper limb accompanied by muscle weakness in the distribution of one or more branches of the brachial plexus. Each patient had received the COVID-19 vaccine within a few weeks prior to the onset of symptoms. Patients underwent detailed neurological examinations followed by nerve conduction and EMG studies. The patients developed symptoms after a mean duration of 17 days (5 days–8 weeks) after receiving the COVID-19 vaccine. The initial symptom was pain in the shoulder girdle/upper limb, followed within days by muscle weakness. Physical examinations and EMG studies showed upper trunk brachial plexopathy in 2 patients, lower trunk plexopathy in 1 patient, posterior cord brachial plexopathy in 1 patient, and anterior/posterior interosseous nerve involvement in 2 patients. All patients either improved or attained complete resolution of the arm pain at follow-up. Three (50%) patients did not have any improvement in the arm/hand weakness, while 3 (50%) patients had some recovery in strength. PTS may occur after the COVID-19 vaccine and should be suspected in patients with symptoms and signs suggestive of acute brachial plexopathy. Studies of a larger series may provide insight into predisposing factors.

Small-Fiber Neuropathy Possibly Associated with COVID-19

Abstract: COVID-19 has caused several neurological complications by affecting the central and peripheral nervous systems (PNS). Studies on the PNS involvement in COVID-19 are limited. These complications are likely unreported, given the difficulty of obtaining further diagnostic information, such as expert neurologist evaluation, electrodiagnostic testing, and skin biopsy. Herein, we report 2 cases of possible COVID-19-related small-fiber neuropathy (SFN). These cases are reported to increase awareness of a possible link between COVID-19 and SFN. Additional investigation, including neurology consultation, nerve conduction studies, and skin biopsy, should be considered in patients who develop paresthesia during and after COVID-19 infection. Further research is also needed to determine a possible underlying neuropathology mechanism and the role of immunomodulatory treatment, such as intravenous immunoglobulin, in COVID-19-related SFN.

Plasmapheresis for Extrapontine Myelinolysis: A Case Series and a Literature Review

Abstract: Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are syndromes of osmotic demyelination attributed to the rapid correction of hyponatraemia. Isolated EPM is a rare clinical entity which poses a significant diagnostic challenge especially in the absence of a rapid rise in sodium. Typical MRI findings aid in the diagnosis. Treatment for established osmotic demyelination syndrome (ODS) is nonstandardized and the prognosis is considered poor. Therefore, different strategies including plasmapheresis (TPE), immunoglobulins (IVIG), and steroids have been used. We present our findings from a series of successfully treated patients at a high-volume tertiary care center in Sri Lanka, with an appraisal of available literature. A total of 21 patients with established ODS are analyzed here, including 5 cases of EPM managed by the authors over a 2-year period. Thirteen (40.2%) patients were treated with plasmapheresis alone, 6 (28.5%) received dual therapy (TPE + IVIG or steroids) and 2 (9.5%) received triple therapy (TPE + IVIG + steroids). There was complete or near complete response in 18 (85.7%) and complete response in 10 (47.6%) patients. We conclude that although the management of CPM/EPM is largely symptomatic, patients may show a significant response to immunomodulatory therapy. The marked improvement in motor, cognitive, and functional domains supports an immune basis for osmotic demyelination. Plasmapheresis, in particular, leads to favorable outcomes in ODS which is supported by previously published case reports. We propose its utility as standard treatment.

Nerve Ultrasound Findings before and after Surgery in a Patient with Charcot-Marie-Tooth Disease Type 1A and Comorbid Carpal Tunnel Syndrome

Abstract: The diagnosis of comorbid carpal tunnel syndrome (CTS) in patients with Charcot-Marie-Tooth (CMT) disease is challenging due to the overlapping symptoms and inconclusive electrodiagnostic studies (EDX). This case report is aimed at illustrating the value of ultrasonography (US) in a patient with CMT1 disease and comorbid CTS. A 28-year-old woman presented with symptoms of painful paresthesia and weakness of both hands. EDX demonstrated a demyelinating sensory-motor polyneuropathy in the upper and lower extremities, consistent with CMT1 disease. US showed an increased cross-sectional area (CSA) of the median nerve at the carpal tunnel inlet (CTI) with a significant drop in the diameter within the carpal tunnel, confirming concurrent CTS. Genetic testing confirmed PMP22 duplication consistent with CMT1A. Bilateral carpal tunnel releases were performed with partial symptom resolution within 3 weeks. Postoperative EDX demonstrated improved motor conduction across the wrist, but the sensory potentials continued to be unrecordable. US showed a significant reversal of the diameter-drop of the median nerve within the carpal tunnel and decrease in CSA at the CTI. US imaging is a valuable technique for identifying comorbid CTS in patients with CMT and directing appropriate treatment.

The Combined Use of Neuropsychiatric and Neuropsychological Assessment Tools to Make a Differential Dementia Diagnosis in the Presence of “Long-Haul” COVID-19

Abstract: The longer term neurocognitive/neuropsychiatric consequences of moderate/severe COVID-19 infection have not been explored. The case herein illustrates a complex web of differential diagnosis. The onset, clinical trajectory, treatment course/response, serial neuroimaging findings, and neuropsychological test data were taken into account when assessing a patient presenting 8 months post-COVID-19 (with premorbid attention-deficit hyperactivity disorder, diabetes mellitus, mood difficulties, and a positive family history of vascular dementia). Her acute COVID-19 infection was complicated by altered mental status associated with encephalopathy and bacterial pneumonia. After recovery from COVID-19, the patient continues to experience persisting cognitive and emotive difficulties despite an ongoing psychopharmacotherapy regimen (16 + years), psychotherapy (15 + sessions), and speech-language pathology SLP; 2 × week/for 12 weeks). The purpose of her most recent and comprehensive neuropsychological evaluation was to determine the presence/absence of neurocognitive disorder. The patient is a 62-year-old Caucasian woman. Cognitive screening was completed 3 months post-acute COVID-19 as part of an SLP evaluation, and a full neuropsychological evaluation was conducted 8 months post-COVID-19 recovery on an outpatient basis (in person). The patient had serial neuroimaging. Initial neurological evaluation during acute COVID-19 included unremarkable brain computed tomography (CT)/magnetic resonance imaging. However, follow-up CT (without contrast) revealed, in part, “asymmetric perisylvian atrophy on the left.” Full neuropsychological evaluation at 8 months post-COVID-19 recovery revealed a dysexecutive syndrome characterized by language dysfunction and affective theory-of-mind deficit, consistent with dementia. There is need for careful use of differential diagnosis in COVID-19 patients with multiple risk factors that make them more susceptible to long-term neurological complications post-COVID-19. Differential diagnosis should involve multidisciplinary assessment (e.g., neuropsychology, SLP, neurology, and psychiatry).

Acute Transient Encephalopathy after Moderna COVID-19 Vaccine

Abstract: Although mRNA vaccine responses following previous coronavirus disease 2019 (COVID-19) infection have not been assessed in trials, it has been shown that serological evidence of previous COVID-19 generates strong humoral and cellular responses to one dose of mRNA vaccine. We describe a patient with prior COVID-19 infection who developed acute transient encephalopathy with elevated inflammatory markers within 24 h of her first injection of Moderna COVID-19 vaccine. A 69-year-old cognitively normal woman presented with intermittent inattention, disorientation, left/right confusion, weakness, gait instability, and decreased speech. Head CT, brain MRI and MRA, complete blood count, liver enzymes, hepatitis B serology, ammonia, thyroid function, vitamin B12, and pulse oximetry were normal. Electroencephalography performed 48 h after symptom onset showed diffuse triphasic waves, diffuse theta and delta slowing, and no posterior dominant rhythm. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) IgG was positive and inflammatory markers were elevated. On day 5 post-vaccine, she returned to her baseline, without neurological sequelae. The reported patient likely developed a transient inflammatory encephalopathy associated with an abnormal immunologic reaction to one dose of COVID-19 vaccine, in the setting of remote COVID-19 infection (1 year prior), SARS-CoV-2 IgG-positivity, and multiple comorbidities. Physicians should be alert to possible postvaccination reactogenicity in individuals with SARS-CoV-2 IgG-positivity, including risk of neuro-inflammation.

Late-Onset Guillain-Barré Syndrome and Right Facial Nerve Palsy after COVID-19 Infection

Abstract: Here, we present a case of late-onset Guillain-Barré syndrome (GBS) associated with COVID-19. A 70-year-old woman presented with ascending paralysis and right lower motor neuron facial weakness 2 months after COVID-19 infection. Test results for SARS-CoV-2 immunoglobulin were positive at the time of presentation. Lumbar puncture showed albuminocytological dissociation, and electrophysiology showed features of demyelination with secondary axon loss. In the published literature on GBS associated with COVID-19, almost all patients presented with neurological symptoms 1–4 weeks after the infection. GBS can be an early or late manifestation after COVID-19. Patients with signs of paraparesis and facial weakness after COVID-19 should be carefully evaluated for immune-mediated central and peripheral nervous system disorders.

Belly Dancer's Dyskinesia: 3 Cases of a Rare Entity

Abstract: Involuntary, undulating, or circular movements of abdominal wall muscles are a rare condition descriptively termed belly dancer syndrome or belly dancer’s dyskinesia. Differential diagnoses range from cerebral, spinal, peripheral, hormonal/drug-induced effects to local or even unknown/idiopathic and functional/psychogenic causes. Thorough diagnostic work-up is mandatory and therapeutic options depend on etiology and include surgical approaches and symptomatic treatment. We present 3 cases of belly dancer’s dyskinesia, in which differential diagnostic work-up did not uncover known etiologies for this movement disorder. Strikingly, 2 patients manifested vitamin B12 deficiency while 1 case showed values close to the lower limit of normal. While relevance of vitamin B12 levels remains elusive, all patients improved substantially following a combined therapeutic approach of clonazepam treatment and vitamin B12 supplementation.

Donepezil-Induced Complex Multimodal Hallucinations: Two Cases and a Review of the Literature

Abstract: Hallucinations are common in neurodegenerative dementias, being present in a significant proportion of patients. Most of the available studies show that acetylcholinesterase inhibitors may be beneficial in preventing and treating hallucinations in patients with neurodegenerative and even psychiatric disorders, even though there are reports that they might also develop as an adverse effect of such therapy. However, a clear causal relationship for the latter association was not previously established. Here we describe 2 cases of patients treated with donepezil who developed complex multimodal hallucinations, which could be causally linked to the drug by means of a challenge-dechallenge (and rechallenge in one case) paradigm. We also provide a narrative review of the literature regarding donepezil and hallucinations and propose a hypothesis to explain the occurrence of this phenomenon.

Stiff Person-Like Syndrome: An Unusual Presentation of Pituitary Macroadenoma with Panhypopituitarism

Abstract: Pituitary adenoma can manifest as pituitary hypofunction, which can cause symptoms of panhypopituitarism. Commonly, symptoms of hormonal deficiencies such as lethargy, weight change, cold intolerance, and sexual dysfunction are reported. Optic chiasmal compression leads to visual field changes and the discovery of the pituitary lesion. However, limb stiffness is a rare presentation of hypopituitarism, especially hypocortisolism. We report a 68-year-old man who presented with progressive lower limb stiffness associated with truncal instability mimicking a stiff person syndrome (SPS). Hypoglycaemia and hyponatraemia prompted the discovery of pituitary macroadenoma with panhypopituitarism. Investigation showed pituitary macroadenoma on magnetic resonance imaging with hypocortisolism, hypothyroidism, and hypogonadotropic hypogonadism. After initiating hydrocortisone replacement, the patient had complete resolution of lower limb stiffness with no permanent neurological sequelae. It is postulated that hypocortisolism and hyponatraemia disrupt the metabolic function of muscle leading to stiffness. As a result, lower limb rigidity, flexion deformities, and pain are more common. Differentiating adrenal insufficiency associated with rigidity and SPS is important as the response to treatment for both conditions differs. Prompt treatment leads to fast resolution and prevents contractures in adrenal insufficiency-associated rigidity. Thus, recognizing limb rigidity as the first presentation of hypopituitarism is important to avoid long-term complications.

Cervical Myelopathy Associated with Deep Neck Muscle Rhabdomyolysis after Polysubstance Abuse: A Case Report

Abstract: When used appropriately, buprenorphine and oxycodone are safe drugs. They are, however, widely abused in combination with other drugs. Here we describe a case series of 8 patients with cervical myelopathy and rhabdomyolysis of the adjacent deep neck muscles after using an opioid in combination with other drugs. All patients were young males who had a previous history of polysubstance abuse. Six of the patients had used buprenorphine in combination with pregabalin and/or benzodiazepines, and one patient had used oxycodone with pregabalin and/or benzodiazepines. One patient used buprenorphine with amphetamine. After taking the drugs, they all reported feeling drowsy and then falling asleep. On waking, they noticed weakness in their extremities. However, only one patient woke with his head in a flexed position. A varying degree of tetraparesis was observed. Cerebrospinal fluid analysis revealed elevated protein levels and white blood cell count. Blood creatine kinase was elevated in 7 patients. Spinal cord MRI showed a hyperintense spinal lesion at the level of C1 – Th3 vertebrae associated with rhabdomyolysis in the adjacent, paravertebral deep neck muscles. We suggest that polysubstance abuse, especially the combination of an opioid with another drug with GABA-agonistic properties, caused a compartment syndrome of the deep paravertebral muscles without excessive head flexion. This subsequently led to compression of the external vascular structures, resulting in venous congestive myelopathy.

Effectiveness of Transcranial Direct Current Stimulation in Chronic Daily Headaches: An Experimental Single Case Report with a Novel Protocol

Abstract: Chronic daily headaches are often refractory to prescribed or non-prescribed medications. Transcranial direct current stimulation (tDCS) is a new technological-based intervention with various modes of applicability. Therefore, we aimed to study the effectiveness of tDCS in an individual with symptoms of chronic daily headaches and the associated comorbidities like depression, anxiety, stress, and RLS or sleepiness, numbness, a sensation of fullness, or ringing in the ears. Based on DASS-21 and Epworth questionnaires, headache diary, and semi-structured interviews, we used repeated measures for assessing the symptoms such as frequency, duration, intensity, or severity of chronic daily headaches and associated comorbidities at baseline, after tDCS-intervention, and at a 12-month follow-up. The results showed that tDCS-intervention reduced the frequency, duration, intensity, or severity of chronic daily headaches and associated symptoms after tDCS-intervention and at a 12-month follow-up.

Case Report: Three Case Reports of Rapidly Progressive Dementias and Narrative Review

Abstract: Rapidly progressive dementia (RPD) is a heterogeneous group of diseases characterized by cognitive impairment and other neurological disorders developed in a short span of fewer than 2 years. Currently viewed as new and infrequent entities, most medical personnel have little understanding of it. Nevertheless, they significantly compromise many patients’ quality of life. Here, we drive 3 clinical cases that evolve as RPD with different etiologies. Case 1:70-year-old woman presented to the emergency with neuropsychiatric syndrome for 18 days. The researchers identified inflammatory cerebrospinal fluid (CSF), protein 14-3-3-positive T-tau protein, MRI: T2 and FLAIR hyperintensities in bilateral caudate nuclei with diffusion restriction, EEG shows a generalized periodic pattern with triphasic wave morphology. Case 2:29-year-old man with cognitive impairment and faciobrachial dystonia seizure. The diagnosis was confirmed by achieving elevated antibodies against voltage-gated potassium channels. Case 3:A 49-year-old woman with encephalopathy and myoclonic seizures; EEG and MRI showed subtle changes. The patient also had a normal CSF but a positive CBA serologic NMDA-R antibody test. We described fundamental aspects of RPD to allow made differential diagnoses in patients with cognitive impairment and encephalopathy. Establishing an early and accurate diagnosis can benefit patients with RPD etiologies that are treatable and even reversible, decreasing in morbidity and mortality.

Rhabdomyolysis after COVID-19 Comirnaty Vaccination: A Case Report

Abstract: Rhabdomyolysis is an acute disruption in skeletal muscle integrity, leading to the rapid release of 4 muscle contents into the bloodstream, such as creatine kinase (CK). It can have various causes, including infections. Throughout the pandemic, multiple cases of rhabdomyolysis following COVID-19 infections have been reported. However, rhabdomyolysis subsequent to COVID-19 vaccinations appears to be relatively rare. Here, we report such a case after a second COVID-19 Comirnaty (BioNTech/Pfizer) vaccination. Our patient developed rhabdomyolysis 1 day after the second Comirnaty vaccination with high creatine kinase (CK) levels, generalized weakness, and kidney failure. CK levels and muscle weakness resolved after treatment with intravenous fluids, but unfortunately, he remained hemodialysis dependent after discharge. To our knowledge, this is one of the first case reports describing a patient with rhabdomyolysis after a Comirnaty vaccination. However, as millions of people have received the Comirnaty vaccine, it is unclear whether the rhabdomyolysis in our patient is a rare side effect or an unrelated, coincidental event. Large observational studies are needed to elucidate the causality between the Comirnaty vaccination and rhabdomyolysis. Awareness is warranted in patients with myalgia and muscle weakness shortly after COVID-19 vaccination, in order to initiate treatment early and prevent life-threatening complications.

Right Tegmental Hemorrhage with Urinary Retention: A Case Report

Abstract: The upper brainstem tegmentum is dense and complex, making it difficult to localize functions to specific subregions. In particular, the precise location and possible laterality of subregions supporting basic functions like consciousness and urinary continence remain unclear. Here, we describe a patient who presented with a right pontine tegmental syndrome caused by intraparenchymal hemorrhage. Despite hemorrhage extension into the fourth ventricle and expansion of both hemorrhage and edema into a large region of the caudal midbrain and right-sided pontine tegmentum, this patient did not lose consciousness. Instead, he developed new and total urinary retention, with residual bladder volumes of more than 1,000 mL. We conclude that injury to the right pontine tegmentum is sufficient to disrupt the micturition reflex pathway.

Cerebral Venous Thrombosis due to Cryptococcus in a Multiple Sclerosis Patient on Fingolimod

Abstract: Fingolimod is a disease-modifying treatment utilized in the treatment of relapsing-remitting multiple sclerosis. Fingolimod has been associated with an increased risk in herpes simplex and varicella infection in clinical trials. We report a case of cerebral venous thrombosis secondary to cryptococcus in a patient receiving fingolimod. A 61-year-old male with multiple sclerosis treated with fingolimod presented with a 2-week history of headache, chills, and night sweats. An MRI of the brain revealed a left transverse and sigmoid sinus thrombosis. Two blood cultures revealed Cryptococcus neoformans; a serum cryptococcal antigen was also positive. HIV testing was negative. A lumbar puncture was deferred as the patient was placed on heparin and, subsequently, warfarin for the cerebral venous thrombosis. The patient received antifungal therapy for 14 days with liposomal amphotericin B and flucytosine, followed by oral fluconazole for 8 weeks. He was subsequently readmitted 60 days later with bilateral papilledema; his anticoagulation was reversed, and a lumbar puncture revealed a negative cryptococcal antigen and an intracranial pressure of 20. A repeat MRI revealed worsening superior sagittal sinus thrombosis, thought to be the cause of the papilledema; his anticoagulation was reinitiated. He received a brief course of intravenous methylprednisolone, but as his multiple sclerosis was well-controlled, further therapy was deferred. His symptoms had resolved at a 3-month follow-up appointment. This is the first report of a multiple sclerosis patient treated with fingolimod to develop cerebral venous thrombosis secondary to cryptococcal fungemia. The risks of opportunistic infections should be considered in patients managed with fingolimod.

Refractory Seizures Secondary to Vitamin B6 Deficiency in Parkinson Disease: The Role of Carbidopa-Levodopa

Abstract: Carbidopa-levodopa has been used for more than 50 years in the treatment of Parkinson disease (PD) and other movement disorders. Pyridoxal 5′-phosphate (PLP), an active form of vitamin B6 (pyridoxine), is involved in the decarboxylation of levodopa to dopamine; carbidopa, which is combined with levodopa to reduce peripheral levodopa conversion and minimize peripheral dopamine side effects, binds irreversibly with PLP. As a result, carbidopa-levodopa may cause vitamin B6 deficiency and associated sequelae, including seizures, especially in high doses. A 78-year-old gentleman with a 6-year history of PD on carbidopa-levodopa therapy and recent weight loss presented with new-onset myoclonus and focal to bilateral tonic-clonic seizures. Workup for vascular, infectious, malignant, metabolic, and autoimmune causes of seizure was unrevealing. The folate level was critically low at <2.20 ng/dL. Video EEG studies showed moderate cerebral dysfunction and seizures with diffuse onsets. Several anti-seizure medications (ASMs) were unsuccessfully tried, so empiric treatment with high-dose steroids was initiated eventually alongside intravenous vitamin B6 therapy. Following introduction of these interventions, the patient had no further epileptic events. The vitamin B6 level came back as undetectable at <1 μg/dL. The patient was discharged to a rehabilitation center for improved strength and function. At the time of writing, he remained on two ASMs as well as IV B6 supplementation. Vitamin B6 is a required cofactor in the decarboxylation of levodopa to dopamine, and high levodopa dosages may cause B6 deficiency; in addition, carbidopa binds B6 irreversibly. We recommend screening of vitamin B6 levels in PD patients, especially those requiring high or increasing doses of carbidopa-levodopa and those with poor nutrition.

Severe Hemophilia A and Moyamoya Syndrome in a 19-Year-Old Boy Caused by Xq28 Microdeletion

Abstract: Severe hemophilia A and moyamoya (SHAM) syndrome is a rare condition that combines hemophilia A and moyamoya disease (MMD) due to an Xq28 microdeletion encompassing the F8 and BRCC3 genes. Here, we report the case of a 19-year-old male patient with hemophilia A and hypogonadism that presented with right-sided hemiparesis and dysarthria. Brain magnetic resonance imaging and angiography revealed an ischemic lesion in the left lobe and stenosis of both middle cerebral arteries with a concomitant thick vascular network, compatible with moyamoya disease. Next-generation sequence revealed a large Xq28 deletion compatible with SHAM syndrome. The patient was treated with acetylsalicylic acid and neurosurgical intervention was scheduled. Our patient is one of the few cases reported in the literature with Xq28 microdeletion encompassing the F8, hemophilia A causative gene, and BRCC3, responsible for MMD, presenting with a compound phenotype that included neurological manifestations and hypogonadism. In conclusion, diagnosis of MMD should be considered in any male, young patient with symptoms of ischemic stroke with no obvious explanation, and especially in patients with known hemophilia, since a relationship between the two conditions has been documented.

Frontotemporal Brain Sagging Syndrome as a Treatable Cause Mimicking Frontotemporal Dementia: A Case Report

Abstract: Frontotemporal brain sagging syndrome (FBSS) is a progressive disorder characterized by symptoms similar to the behavioral variant of frontotemporal dementia (FTD), with a sagging appearance of the brain on imaging similar to that observed in spontaneous intracranial hypotension (SIH). The onset of behavioral and cognitive symptoms of FBSS is insidious and progressive, similar to those of FTD. Here, we report a case involving a 53-year-old man with progressive hypersomnolence, apathy, forgetfulness, and personality changes but without headache or auditory symptoms. The combination of frontotemporal dysfunction, hypersomnolence, and the appearance of a sagging brain on magnetic resonance imaging suggested a diagnosis of FBSS. Although a definite site of cerebrospinal fluid leakage could not be identified in our case, clinical symptoms and imaging findings were improved after an epidural blood patch. Considering FBSS as a differential diagnosis of FTD is important even in the absence of typical SIH symptoms, such as headache or auditory symptoms.

Sensory Predominant Guillain-Barré Syndrome Concomitant with Dengue Infection: A Case Report

Abstract: Guillain-Barre syndrome is an acute demyelinating polyneuropathy disease which is autoimmune in nature and usually follows gastrointestinal or respiratory infections. Dengue fever is however not a common trigger to the condition. Here, we report a patient who developed sensory predominant demyelinating polyradiculopathy during febrile phase of dengue fever. It was later confirmed with serology test and nerve conduction study. He was successfully treated with intravenous immunoglobulin and discharged home well. The purpose of this case report is to highlight that Guillain-Barré syndrome can occur as an uncommon neurological complication of dengue fever which can occur during any phase of the illness.

Cerebral Amyloid Angiopathy-Related Inflammation following Multiple Cancers and Chemotherapies

Abstract: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare autoimmune encephalopathy of aging caused by an autoantibody immune response against Aβ protein deposited in the brain of older adults affected by cerebral amyloid angiopathy (CAA) and Alzheimer’s disease pathology. Its most common clinical manifestations are (sub)acute-onset cognitive and behavioral abnormalities, focal deficits, seizures, and headaches. Brain magnetic resonance imaging shows characteristic extensive and confluent white matter hyperintensities and CAA features. The response to immunosuppressive treatment is generally good. Here, we report the case of a 62-year-old patient with CAA-ri confirmed on biopsy, who had previously repeatedly received chemotherapy for multiple cancers. We summarize his clinical data, neuroradiological features, and therapeutic response and comment on the potential mechanisms connecting multiple cancers and chemotherapies with CAA-ri.

Status Epilepticus Secondary to Hypocalcemia Due to Vitamin D Deficiency

Abstract: Vitamin D deficiency is becoming more common around the world, owing to reduced sunshine exposure and an imbalanced diet. However, severe hypocalcemia as a result of vitamin D insufficiency is a rare occurrence, and it rarely leads to seizures in children. We present such a case in a 6-month-old infant who presented with status epilepticus secondary to hypocalcemia due to vitamin D deficiency, which was first misdiagnosed as epilepsy. Thereby, we want to emphasize that hypocalcemia secondary to vitamin D deficiency can lead to convulsion and the importance of vitamin D supplementation.

Cetuximab-Induced Aseptic Meningitis in a Patient with Colorectal Cancer: A Case Report and Review of Literature

Abstract: Cetuximab is a chimeric IgG1 monoclonal antibody against epidermal growth factor receptor. It is approved by the European medical agency for the treatment of RAS wild-type metastatic colorectal cancer and metastatic squamous cell cancer of the head and neck. Few cases of aseptic meningitis, primarily associated with the first administration of cetuximab in patients with squamous cell cancer, have been reported. So far, there was only 1 case in a patient with metastatic colorectal cancer. We report on a 50-year-old Caucasian patient with metastatic rectum carcinoma who suffered from headache, fever, and neck stiffness 3 h after the first administration of cetuximab (400 mg/m²). CSF examination revealed an excessive pleocytosis with a white blood cell count of 2,433/µL. He was diagnosed with cetuximab-induced aseptic meningitis since clinical symptoms and CSF pleocytosis resolved within days, and further diagnostic workup revealed no infectious cause. Cetuximab-induced aseptic meningitis is a rare and severe drug reaction with predominance in treating squamous cell cancer of the head and neck. Clinical presentation and CSF findings suggest acute meningoencephalitis. In all reported cases, the course of the disease was benign and self-limited. Empiric antimicrobial and antiviral therapy are suggested until infectious causes can be ruled out. A lower dosage of cetuximab and a premedication including antihistamines and glucocorticosteroids may lower the risk of a re-occurrence if cetuximab therapy is continued.

Late-Onset Pompe Disease Presenting with Isolated Tongue Involvement

Abstract: Late-onset Pompe disease (LOPD) is a rare autosomal recessive metabolic disorder that is caused by deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA), which is responsible for glycogen breakdown. It has a wide clinical spectrum but usually presents with limb girdle and respiratory muscles weakness. Tongue involvement has been rarely reported as the sole initial symptom of LOPD. A 65-year-old male presented with difficulty in speech and eating for a 4-year duration. He started to notice speech difficulty with production of particular speech sounds such as /l/, /d/, and /t/. Within 1 year, he developed difficulties in manipulating food with the tongue and oral residue in lateral sulci requiring digital manipulation, which was suggestive of tongue muscles weakness. Clinical examination showed tongue fasciculations, mild atrophic changes, and mild tongue weakness. Investigations showed mildly elevated creatine kinase levels, and electromyography of the tongue muscles revealed moderate spontaneous activity, denervation, chronic reinnervation with high-amplitude motor unit potentials, and positive sharp waves, with preserved recruitment. Given the diagnostic uncertainty, a screening for LOPD was performed using a dried blood spot, and GAA enzyme activity levels were found to be low; 1.06 μmol/L/h (reference values in adults: 2.10–29.00 μmol/L/h). Next-generation sequencing showed pathogenic variant in GAA gene, confirming the diagnosis of LOPD. This rare report of LOPD presenting with isolated tongue involvement adds to the expanding phenotypic variability of this disease. Tongue involvement is an important and early clinical sign of LOPD that needs careful evaluation and can aid in early diagnosis of this rare and treatable disease.

Wernicke Encephalopathy following Gastric Bypass: A Case Report

Abstract: Bariatric surgery is used as a treatment for morbid obesity and often results in rapid weight loss. This procedure has been associated with postoperative nutritional deficiencies. Neurological complications due to nutritional deficiencies include Wernicke encephalopathy, a disorder that affects the central and peripheral nervous system due to thiamine (vitamin B1) deficiency. Wernicke encephalopathy can lead to irreversible consequences if not treated early. Here, we present a case of a 40-year-old woman that developed Wernicke encephalopathy 2 months after gastric bypass surgery, with additional findings of flat affect and concurrent polyradiculopathy. Her diagnosis was delayed due to unique symptoms and an initial workup with negative imaging findings, making the identification of this disorder more complex.

Immunocompetence after SARS-CoV-2 Infection in a Patient with Multiple Sclerosis Treated with Ofatumumab: A Case Report

Abstract: Ofatumumab is the first fully human anti-CD20 monoclonal antibody that, on March 26, 2021, was approved by the EMA to treat patients with relapsing multiple sclerosis. This paper aimed to present a case confirming the ability to produce and maintain anti-SARS-CoV-2 antibodies in a patient treated with ofatumumab for over 4 years. The course of the infection was moderate, and the patient did not require hospitalization. Antibody measurements were performed five times post-COVID-19 infection. The first test was performed in the fourth month (131 days), and the last, over 1 year after the infection. To date, only 2 cases have been published describing the ability of a patient treated with the same drug to produce antibodies against SARS-CoV-2, although the observation was conducted over a shorter period. In our case study, we have 15-month follow-up data. The patient was not vaccinated and additionally received suppressive steroid therapy due to the relapse. We observed an increase in antibodies up to 10 months from the infection. The case under analysis suggests that patients treated with ofatumumab, despite complete peripheral B-cell depletion, can produce and maintain a long-lasting immune response.

Multiple Endovascular Treatments for Hemorrhagic Cerebral Proliferative Angiopathy: A Case Report

Abstract: Cerebral proliferative angiopathy (CPA) is a rare vascular abnormality characterized by transdural supply, stenoses of feeding arteries, and intermingled normal brain parenchyma in abnormal vessels. CPA is often regarded as a separate entity from “classical” brain arteriovenous malformations in angioarchitecture, natural history, clinical presentation, and treatment. Bleeding from CPA is uncommon, but once bleeding occurs, the risk of rebleeding is high. Herein, we describe a case of cerebral hemorrhage caused by CPA. We performed two different endovascular treatments: partial embolization with glue for a ruptured aneurysm and coil embolization for an unruptured growing aneurysm. To our knowledge, this is the first report of serial endovascular treatments for hemorrhagic CPA that included a ruptured aneurysm and a growing unruptured aneurysm.

Progressive Multifocal Leukoencephalopathy and Immune Reconstitution Inflammatory Syndrome after Discontinuation of Fingolimod

Abstract: Progressive multifocal leukoencephalopathy (PML) is a rare complication of immunosuppressive treatment in MS patients. Immune reconstitution inflammatory syndrome (IRIS) appears after the withdrawal of certain drugs such as natalizumab (NTZ) or fingolimod. The development of PML-IRIS after NTZ treatment has been described, and its diagnosis is made by clinical and radiological criteria and the determination of the John Cunningham virus in CSF. We present a clinical case of a patient with MS who, after the withdrawal of fingolimod, developed PML-IRIS despite sustained lymphopenia. This is important for pharmacovigilance purposes, not only for NTZ but also for alternative drugs used in MS treatment.

Myasthenia Gravis Complicated by Eosinophilic Granulomatosis with Polyangiitis: A Case Report

Abstract: A 55-year-old woman with a history of allergic sinusitis was being administered cyclosporine for ptosis and diplopia due to myasthenia gravis since age 46 years. She developed painful dysesthesia that began in her feet and later spread to her palms, leading to difficulty in walking. Eosinophils were markedly increased in the peripheral blood. Nerve conduction studies revealed mononeuritis multiplex. Nerve biopsy showed the infiltration of eosinophils in the superior neurovasculature. Based on these findings, eosinophilic granulomatous polyangiitis was diagnosed. Methylprednisolone pulse therapy was followed by oral prednisolone. Two weeks after treatment, the patient could do normal daily activities without assistance. In patients with myasthenia gravis having a history of allergic diseases, considering EGPA as a complication and monitoring prior changes in blood data are necessary for early detection before apparent tissue damage.

A Unique Constellation of Multiple Cranial Neuropathies in a Patient with Preeclampsia

Abstract: A 36-year-old nullipara at 35 weeks of gestation woke up with slurred speech and dysphagia. The next day, she developed abruption of the placenta, underwent an acute cesarean, and was diagnosed with severe preeclampsia. Neurologic examination revealed flaccid dysarthria, bilateral soft palate palsy, reduced taste of the left posterior tongue, left-sided tongue deviation, and paralysis of the left sternocleidomastoid and trapezius muscles. MRI revealed left-sided tongue edema compatible with acute left hypoglossal nerve denervation and electromyography of the left trapezius and glossal muscles showed profuse denervation potentials. In conclusion, multiple cranial neuropathies may occur in and even be a presenting symptom of preeclampsia. In this study, we report the first case of multiple cranial neuropathies involving cranial nerves IX, X, XI, and XII in a patient with preeclampsia. Possible pathogenic mechanisms of cranial neuropathy in preeclampsia include immune-mediated neuropathy with or without demyelination, microvascular thromboses, and perineural edema.