Guillain-Barr é Syndrome Heralding the Diagnosis of Hodgkin Lymphoma: A Case Report
Lymphoma is a prevalent type of lymphoid tissue malignancy that is seldom associated with Guillain-Barr é syndrome (GBS). In the majority of instances, both Hodgkin’s and non-Hodgkin’s lymphoma are not proceeded by GBS. Here, we report on a case of a young patient with a manifestation and investigation suggestive of GBS, signaling an unconfirmed diagnosis of Hodgkin’s lymphoma. A cerebrospinal fluid test revealed an albuminocytological dissociation with a noteworthy rise in protein (2.32 g/L). The patient was initiated on intravenous immunoglobulin (IVIG) treatment and then showed dramatic improvem...
Source: Case Reports in Neurology - October 30, 2020 Category: Neurology Source Type: research

Avoiding Anchoring Bias in the Times of the Pandemic!
We present a patient of COVID-19 pneumonia who presented with dyspnea and acute confusional state. His initial workup was suggestive of tuberculous meningoencephalitis with lymphocytic pleocytosis, high protein in CSF analysis, and suspicious MRI findings, which was later confirmed with a positive CSF culture. To the best of our knowledge, it is the first such case. Anchoring to the diagnosis of COVID-19 may deter clinicians from considering other concurrent diagnoses and a poor outcome consequently.Case Rep Neurol 2020;12:359 –364 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - October 9, 2020 Category: Neurology Source Type: research

Exploding Head Syndrome: A Case Series of Underdiagnosed Hypnic Parasomnia
Exploding head syndrome (EHS) is an under-recognized parasomnia characterized by a complaint of sudden loud noise or a sense of explosion in the head that usually occurs at sleep onset. This paper is a report of 6 patients diagnosed with EHS through a structured clinical interview and video-polysomnography (vPSG) recordings. We also reviewed the available literature that addressed the presentation and clinical and PSG characteristics of EHS. The case series included 4 men and 2 women of a mean age of 44.2 years (between 13 and 77 years). Their episodes were variable in expression, between a sudden firecracker-like explosio...
Source: Case Reports in Neurology - October 8, 2020 Category: Neurology Source Type: research

Paraneoplastic Stiff Person Syndrome in Early-Stage Breast Cancer with Positive Anti-Amphiphysin Antibodies
We present the case of a patient with paraneoplastic SPS, presenting with muscle cramps of lower extremities that progressed to severe muscle rigidity and spasms, associated with a right breast tumor and positive anti-amphiphysin antibodies. Paraneoplastic SPS is a rare neurological disorder, challenging for the physicians both to diagnose and treat.Case Rep Neurol 2020;12:339 –347 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - October 2, 2020 Category: Neurology Source Type: research

A Case of HaNDL with Low Cerebrospinal Fluid Level of Neurofilament Light Chain
We present a case presenting with characteristic features of HaNDL and an MRI lesion in the splenium of corpus callosum. CSF neurofilament light chain (NFL) levels were assessed in this patient together with 7 additional HaNDL patients, 18 multiple sclerosis (MS) patients, and 15 primary headache patients. Both HaNDL and primary headache patients showed significantly lower NFL levels than MS patients. Our results suggest that increased CSF levels of NFL and neuroaxonal loss are not characteristic features of HaNDL. Neurological disorders mimicking HaNDL often present with increased levels of NFL, and thus CSF measurement o...
Source: Case Reports in Neurology - October 2, 2020 Category: Neurology Source Type: research

A Pyramidal Cause of a Cerebellar Ataxia: HSP-7
A 43-year-old man presented with a slowly progressive fatigue and coordination problems, coupled with a radiological appearance of diffuse atrophy, especially in the cerebellar hemispheres. The diagnostic process was challenging because initially the additional investigations were focused on a cerebellar ataxia. In the following months, his ataxic gait developed in a more spastic pattern and whole exome sequencing revealed mutations in the SPG7 gene, confirming a diagnosis of hereditary spastic paraplegia. Therefore, the authors call for an extension of genetic panels in ataxia patients.Case Rep Neurol 2020;12:329 –3...
Source: Case Reports in Neurology - October 2, 2020 Category: Neurology Source Type: research

COVID-19 Related Cerebrovascular Thromboembolic Complications in Three Young Patients
Coronavirus disease 2019 (COVID-19) is a viral illness, caused by the novel severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2). It is currently affecting millions of people worldwide and is associated with coagulopathy, both in the venous and arterial systems. The proposed mechanism being excessive inflammation, platelet activation, endothelial dysfunction, and stasis. As an ongoing pandemic declared by WHO in March 2020, health systems worldwide are experiencing significant challenges with COVID-19-related complications. It has been noticed that patients with COVID-19 are at greater risk of thrombosis.Case Rep N...
Source: Case Reports in Neurology - September 28, 2020 Category: Neurology Source Type: research

A Case of Cervical Spondylotic Amyotrophy Mimicking Amyotrophic Lateral Sclerosis
We present the case of a 57-year-old female who presented with a 1-year history of left-hand weakness and wasting with no sensory deficits. She denied any involvement of her other hand or bilateral lower limbs, and she was referred to our clinic with the potential diagnosis of amyotrophic lateral sclerosis (ALS). An elaborate history, physical examination, electrophysiological studies, and imaging assisted us in reaching the diagnosis of CSA, 1 year after the onset of symptoms.Case Rep Neurol 2020;12:314 –320 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - September 18, 2020 Category: Neurology Source Type: research

An Atypical Presentation of CLIPPERS, a Challenging Diagnosis of Reversible Early-Onset Dementia
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory disorder featured by pontocerebellar dysfunctions and, in some cases, later cognitive disturbances. Here, we describe an atypical presentation of CLIPPERS, characterized by clinical onset with neuropsychiatric and cognitive symptoms. A 45-year-old man was referred to our Memory Clinic due to difficulties at work for over a month, caused by confusion and asthenia. Furthermore, insomnia and mood changes appeared. These disturbances were unresponsive to antipsychotic and antidepressant drugs. At admi...
Source: Case Reports in Neurology - September 18, 2020 Category: Neurology Source Type: research

Effect of Flunarizine on Alternating Hemiplegia of Childhood in a Patient with the p.E815K Mutation in ATP1A3: A Case Report
Alternating hemiplegia of childhood (AHC) (MIM 104290) is characterized by transient repeated attacks of paresis on either or both sides of the body, oculomotor and autonomic abnormalities, movement disorders, and cognitive impairment. Preventing paroxysmal attacks, such as paresis and spasm, in patients with AHC is often difficult. An 8-month-old girl presented to our institution with intractable epilepsy. She developed AHC, with left-right alternating or bilateral recurrent plegia upon waking, involuntary movements, eye movement abnormalities, and psychomotor retardation. She had a heterozygous de novo p.E815K mutation i...
Source: Case Reports in Neurology - September 18, 2020 Category: Neurology Source Type: research

Monomelic Amyotrophy (Hirayama Disease): A Rare Case Report and Literature Review
We report a rare case of a 20-year-old Kuwaiti patient presenting with a 10-month duration of gradual left upper limb weakness and wasting. We describe his electrophysiological and radiological findings that confirmed the diagnosis, and conducted a literature review. Hirayama disease is rarely encountered in clinical settings and should be suspected in male patients presenting with unilateral or asymmetrical bilateral lower motor weakness of hands and forearms. It is a benign entity, and cervical collar is usually the only treatment needed in most cases.Case Rep Neurol 2020;12:291 –298 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - September 17, 2020 Category: Neurology Source Type: research

Volume-Staged Radiosurgery for Large Arteriovenous Malformation
L аrge аrteriovenous mаlformаtions (АVMs) аre chаllenges in mаnаgement becаuse of outcomes аnd аdverse аffects. Volume-stаged rаdiosurgery hаs been аn аppropriаte аpproаch when removаl resection аnd embolizаtion аre not recommended. А 53-yeаr-old gentlemаn wаs diаgnosed w ith а lаrge intrаcrаniаl АVM with persistent heаdаche аnd short-term seizure. Brаin mаgnetic resonаnce аnd аngiogrаph showed а bulky volume of АVM nidus. Removаl resection аnd embolizаtion were not recommended becаuse of high risk of аdverse аffects. The pаtient wаs treаted by volume-stаged rаdiosu...
Source: Case Reports in Neurology - September 17, 2020 Category: Neurology Source Type: research

Isolated Cervical Myelitis in Lyme Disease: A Rare Manifestation of Acute Neuroborreliosis
We report a case of acute neuroborreliosis that manifested as extended isolated cervical myelitis. Not only the manifestation as isolated myelitis in the early stages of borreliosis represents a rarity, but also the strong contrast between mild clinical symptoms and pronounced imaging findings in this case is remarkable.Case Rep Neurol 2020;12:276 –281 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - September 17, 2020 Category: Neurology Source Type: research

The Effectiveness of Perampanel for Myoclonic Seizures in Down Syndrome with Isodicentric Chromosome 21
Epileptic seizures are common in the elderly Down syndrome population. We encountered a patient with Down syndrome in whom karyotyping showed the rare isodicentric chromosome 21 and who suffered from myoclonic seizures. A 52-year-old woman with Down syndrome experienced sudden onset of drowsiness and frequent myoclonic jerks in the upper body. Video-EEG recordings demonstrated generalized polyspike-wave discharges consistent with myoclonic jerks, which were exacerbated by photo-stimulation. Her myoclonus completely resolved with perampanel administration. Perampanel was effective for myoclonic seizures in our patient. We s...
Source: Case Reports in Neurology - September 10, 2020 Category: Neurology Source Type: research

Hypertension-Related Cerebral Microbleeds
We describe a case of cerebellar hemorrhage with cerebral microbleeds secondary to chronic hypertension.Case Rep Neurol 2020;12:266 –269 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - September 10, 2020 Category: Neurology Source Type: research

A Case of Coronavirus Disease 2019 Presenting with Seizures Secondary to Cerebral Venous Sinus Thrombosis
We report the first case of COVID-19 presenting with generalized seizure secondary to cerebral venous sinus thrombosis.Case Rep Neurol 2020;12:260 –265 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - August 4, 2020 Category: Neurology Source Type: research

A Novel Duplication Mutation in the < b > < i > Myelin Protein Zero < /i > < /b > Gene Causing Mild, Nonprogressive Demyelinating Neuropathy
We present a father and daughter with neuropathy carrying a novel 31 base pair duplication mutation in the 5 ′ untranslated region of theMPZ gene, c.-23_8dup31. Genetic analysis and protein modeling indicated that this is a frameshift mutation resulting in premature truncation of the encoded protein. The daughter underwent repeat neurological examination and electromyography testing over an 11-year time span demonstrating no clinical or electrophysiological change. Our study expands the clinical and genetic spectrum of mutations that can cause CMT type 1B disease and demonstrates the value of sequence analysis of non...
Source: Case Reports in Neurology - July 29, 2020 Category: Neurology Source Type: research

Fibrosing Inflammatory Pseudotumor Presenting as Cranial Neuropathy
We report two cases of biopsy-corroborated “fibrosing inflammatory pseudotumor” to illustrate that the entity, rarely described in the neurological literature, should be included in the differential diagnosis of either a cranial mononeuropathy or, certainly, in the case of progressive cranial neuropathies. A broad differential diagnosis arises in certain contexts. Early steroid treatment can be effective, and perhaps later-generation immune-modulating agents may confer further options, although there is no known definitive treatment.Case Rep Neurol 2020;12:247 –254 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - July 1, 2020 Category: Neurology Source Type: research

Virtual Reality Training Helpful in Motor and Cognition in Corticobasal Syndrome: A Case Report PET Study
Corticobasal syndrome (CBS) is a rare progressive neurodegenerative disease characterized by a combination of asymmetric motor symptoms and non-motor symptoms. CBS is poorly responsive to levodopa. Non-pharmacological strategies have been found to be useful as components of a multidisciplinary therapeutic approach for patients with CBS. The purpose of this study was to investigate the effects of virtual reality training in addressing symptoms of CBS. A 64-year-old man was clinically diagnosed as CBS one year after the onset of complaints. Positron emission tomography (PET) scan showed asymmetrical hypometabolism in the rig...
Source: Case Reports in Neurology - July 1, 2020 Category: Neurology Source Type: research

Colocalization of Tau but Not β-Amyloid with Cortical Superficial Siderosis in a Case with Probable CAA
We report on an 80-year-old male patient who was diagnosed with probable CAA according to modified Boston criteria and underwent longitudinal magnetic resonance imaging, amyloid positron emission tomography (PET), and additional t au PET imaging. Amyloid deposition presented predominantly in the contralateral hemisphere not affected by cSS. In contrast, tau deposition was predominantly overlapping with brain regions affected by cSS. Amyloid deposition was not different in the vicinity of cSS whereas tau depositions were eleva ted in the vicinity of CSS-affected regions compared to non-cSS-affected brain regions. This case ...
Source: Case Reports in Neurology - June 29, 2020 Category: Neurology Source Type: research

Differential Impact of Cognitive Impairment in MCI Patients: A Case-Based Report
Mild cognitive impairment (MCI) traditionally refers to an intermediate stage between healthy individuals and early Alzheimer disease. Evidence shows grey and white matter volume changes and decrease in several executive functions, albeit the relation between cognitive performance and brain volume remains unclear. Here, we discuss 3 individual cases of MCI by investigating their MRI scans and cognitive test performance. We also recruited age-matched healthy older adults serving as gold standard for both grey and white matter volume and cognitive test outcomes. Our results show the impact of cognitive impairment on cognitiv...
Source: Case Reports in Neurology - June 29, 2020 Category: Neurology Source Type: research

Epstein-Barr Virus-Associated Encephalopathy Presenting with Nonconvulsive Status Epilepticus in an Immunosuppressive State
Epstein-Barr virus (EBV) infection is occasionally accompanied by central nervous system (CNS) complications, particularly in immunosuppressed patients. However, the symptoms and clinical features of EBV infection in the CNS are rather heterogeneous and remain unknown. We herein describe the first reported adult case manifesting nonconvulsive status epilepticus (NCSE), possibly associated with reactivation of EBV in an immunosuppressive state. A 63-year-old man with a history of acute myeloid leukemia and taking immunosuppressants was admitted due to progressively impaired consciousness without any focal neurological signs...
Source: Case Reports in Neurology - June 22, 2020 Category: Neurology Source Type: research

Multifocal Stroke Complicating Anti-NMDA Receptor Encephalitis
We describe here a preadolescent female, whose course of NMDARE was complicated by a unilateral stroke, resulting in permanent deficits. The imaging characteristics suggest a vascular (thrombotic) etiology. To our knowledge, th is is the first report of stroke in the setting of NMDARE.Case Rep Neurol 2020;12:210 –213 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - June 12, 2020 Category: Neurology Source Type: research

Cerebrovascular Disease in Patients with COVID-19: A Review of the Literature and Case Series
COVID-19 has been associated with a hypercoagulable state causing cardiovascular and neurovascular complications. To further characterize cerebrovascular disease (CVD) in COVID-19, we review the current literature of published cases and additionally report the clinical presentation, laboratory and diagnostic testing results of 12 cases with COVID-19 infection and concurrent CVD from two academic medical centers in Houston, TX, USA, between March 1 and May 10, 2020. To date, there are 12 case studies reporting 47 cases of CVD in COVID-19. However, only 4 small case series have described the clinical and laboratory findings ...
Source: Case Reports in Neurology - June 11, 2020 Category: Neurology Source Type: research

Sequential Bilateral Vertebral Artery Dissections with Prompt Resolution of Initial Insult
Vertebral arterial dissection is a known cause of stroke in young adults. There has been a multitude of cases of bilateral vertebral dissections, including progression from one vertebral artery to another. This case reports the curious sequential nature of the healing of a previously dissected vertebral artery with subsequent dissection of the collateral vertebral artery. Follow-up neuroimaging evaluation performed several months later showed healed bilateral vertebral artery. The potential trigger was neck cracking.Case Rep Neurol 2020;12:189 –198 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - June 11, 2020 Category: Neurology Source Type: research

Cluster-Like Headache Revealing Polycythemia Vera: A Case Report
Herein, we report on a 44-year-old man who presented with cluster headache (CH)-like pain triggered by polycythemia vera (PV). He had severe unilateral head pain attacks lasting about 30 min not associated with cranial autonomic symptoms. After the exclusion of secondary etiologies, the patient was screened for a neoplastic process through biological markers, and the diagnosis of PV was established. The results of the initial laboratory examination showed hemoglobin at 18.1 g/L and Hct at 54%. JAK2 mutation analysis was positive at 54%, and marrow biopsy confirmed the hematopoietic clonal expansion, without myelofibrosis. ...
Source: Case Reports in Neurology - June 10, 2020 Category: Neurology Source Type: research

Probable Creutzfeldt-Jakob Disease Presenting Dementia and Urinary Retention
We describe the case of an 80-year-old woman with probable Creutzfeldt-Jakob disease (CJD) presenting dementia and urinary retention. Although the number of patients previously examined, including ours, is small for conclusion, provided that other etiologies of urinary retention are carefully excluded, urinary retention seems to become a feature in CJD, presumably reflecting spinal cord pathology in CJD. Physicians are advised to evaluate pelvic floor function in CJD particularly by checking post-void residuals.Case Rep Neurol 2020;12:180 –183 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - June 10, 2020 Category: Neurology Source Type: research

Efficacy of Deep Brain Stimulation in a Patient with Genetically Confirmed Chorea-Acanthocytosis
Chorea-acanthocytosis (ChAc) is a rare autosomal recessive neurodegenerative disease due to mutation of the VPS13A gene encoding the protein chorein. ChAc is a slowly progressive disorder that typically presents in early adulthood, and whose clinical features include chorea and dystonia with involuntary lip, cheek, and tongue biting. Some patients also have seizures. Treatment for ChAc is symptomatic. A small number of ChAc patients have been treated with bilateral deep brain stimulation (DBS) of the globus pallidus interna (GPi), and we now present an additional case. Patient chart, functional measures, and laboratory fin...
Source: Case Reports in Neurology - June 26, 2019 Category: Neurology Source Type: research

Effect of Repetitive Transcranial Magnetic Stimulation on the Right Superior Temporal Gyrus for Severe Aphasia Caused by Damage to the Left Inferior Frontal Gyrus
This study attempted to perform low-frequency rTMS on the right posterior superior temporal gyrus (BA22), which is the center for language reception for aphasia patients with a drastic decline in verbal expression due to damage to the left inferior frontal gyrus and a considerable decline in language perception. The participants performed a language task that was displayed on a computer monitor during rTMS. In addition, intensive speech-language and hearing therapy was performed by the therapist after rTMS. This study reports that a resultant improvement in language perception was observed in the activated brain regions ba...
Source: Case Reports in Neurology - May 28, 2019 Category: Neurology Source Type: research

Intradural Lipoma at the Craniocervical Junction Presenting with Progressing Hemiparesis: A Case Report
We report a rare case of spinal lipoma without dysraphism and with progressing hemiparesis. A 60-year-old woman had incidental lipoma at the craniocervical junction observed for more than 5 years. Recently, she developed right-sided hemiparesis and sensory disturbance. Radiological studies revealed a large lipoma compressing the dorsal medulla and C1 –C2 spinal cord. Standard midline suboccipital craniotomy and C1 laminectomy were performed, and the lipoma was removed subtotally. The lipoma showed severe adhesion to the dorsal medulla and C1 spinal cord; therefore, the excision was limited as internal debulking. Her ...
Source: Case Reports in Neurology - May 23, 2019 Category: Neurology Source Type: research

Positive Effect of Steroids in Posterior Reversible Encephalopathy Syndrome
We present a case of posterior reversible encephalopathy syndrome with severe clinical manifestation. Apart from initial aphasia, hemiparesis, and a generalized seizure, the patient had a prolonged loss of consciousness. Although blood pressure was normalized, the clinical status deteriorated continuously. After adding steroids to the therapy, the patient recovered rapidly, suggesting that this could have been a useful therapeutic approach. Even the vasogenic edema in the cerebral magnetic resonance imaging disappeared shortly within 6 days.Case Rep Neurol 2019;11:173 –177 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - May 22, 2019 Category: Neurology Source Type: research

Severe Daily Headache as an Uncommon Manifestation of Widespread Skull Base Osteomyelitis
We report a 70-year-old man with diabetes who presented to our outpatient clinic with severe chronic daily complaints of headache. The headache was located frontoparietally and kept him awake at night. Imaging (nonenhanced computed tomography [CT], magnetic resonance imaging, and positron emission tomography/CT) showed a hypermetabolic mass on the right side of the skull base, in the middle ear, and in the mastoid process, with invasion and partial destruction of the surrounding elements of the petrous bone, the occipital bone, and the sphenoid bone on the right, with extension by way of the clivus into the apex of the lef...
Source: Case Reports in Neurology - May 22, 2019 Category: Neurology Source Type: research

Unusual Pattern of Reading Errors in a Patient with Posterior Cortical Atrophy
Posterior cortical atrophy (PCA) is a degenerative condition characterized by a progressive deterioration of visual processing. Dyslexia constitutes an early and frequent visual symptom of the disease and previous comprehensive investigations in series of individuals have extensively documented a characteristic abundance of visual errors as the most prevalent error category in this population. Here we describe the profile of a patient with PCA, C.P., who presents an unusual prevalence of phonological, instead of purely visual, errors in his reading, in the context of an otherwise classic PCA phenotype. In keeping with the ...
Source: Case Reports in Neurology - May 8, 2019 Category: Neurology Source Type: research

Cerebellar Transcranial Magnetic Stimulation Improves Ataxia in Minamata Disease
Minamata disease (MD) is a form of intoxication involving the central nervous system and is caused by ingesting seafood from methylmercury-contaminated areas in Japan. In MD, cerebellar ataxia is a cardinal feature observed in approximately 80% of MD patients. Although cerebellar transcranial magnetic stimulation (TMS) has recently been used for treating cerebellar ataxia, the optimal stimulation conditions remain unclear. Here, we report the first case of cerebellar ataxia in an MD patient that was significantly improved after high-frequency cerebellar TMS. To determine the optimal stimulation conditions, we examined the ...
Source: Case Reports in Neurology - May 8, 2019 Category: Neurology Source Type: research

Atypical Lyme Neuroborreliosis, Guillain-Barr é Syndrome or Conversion Disorder: Differential Diagnosis of Unusual Neurological Presentations
We report the case of a 62-year-old woman presenting with asymmetrical tetraparesis and hyporeflexia. Initially the presumed diagnosis of Guillain-Barré syndrome with a possible functional component was suspected and treatment with intravenous immunoglobulin was initiated. Due to partial response to therapy and further test results including positive serologies and cerebrospinal fluid antibodies for Borrelia, the diagnosis of neuroborreliosis was considered. Furthe r exploring revealed the possibility of exposure to ticks although there was no report of typical skin lesions. Daily physical therapy and appropriate an...
Source: Case Reports in Neurology - May 5, 2019 Category: Neurology Source Type: research

Unusual Course of an Aggressive Pituitary Prolactinoma: Case Report and Review of the Literature
We describe the case of a 35-year-old male who presented with an invasive prolactinoma, managed initially with a transsphenoidal resection, postsurgical radiotherapy and DA agonists. The patient posteriorly presented a sole metastatic lesion to the lumbar spine that was later managed with local radiotherapy. Due to pituitary recurrence of the lesion, multiple surgical resections were needed until further treatment was declined. The clinical course in this patient was unusual. He lived for 13 years after initial diagnosis, with a very invasive tumor without systemic chemotherapy. Radiotherapy is used in pituitary tumors in ...
Source: Case Reports in Neurology - May 5, 2019 Category: Neurology Source Type: research

When Nothing Goes Right: An Unexpected Tongue Deviation in Internal Carotid Artery Dissection
We describe a case of a 55-year-old man who presented with right internal carotid artery dissection and deviation to the left of the protruded tongue. The direction of the deviation of the protruded tongue was unexpected in this patient, because if the XII nerve palsy was due to mass effect related to the intramural hematoma of the dissected artery, a deviation to the right should have happened. Anyway, a subsequent magnetic resonance revealed also an acute ischemic lesion in the right tongue area in the primary motor cortex of the patient, providing a rare, but a fitting neuroanatomical explanation of the deviation and al...
Source: Case Reports in Neurology - April 24, 2019 Category: Neurology Source Type: research

Gastrointestinal Beriberi and Wernicke ’s Encephalopathy Triggered by One Session of Heavy Drinking
An otherwise healthy 30-year-old male acquired gastrointestinal beriberi and subsequent Wernicke ’s encephalopathy after 1 session of heavy drinking. Nausea, vomiting, and anorexia relentlessly progressed. The patient developed external ophthalmoplegia after 2 months. Intravenous 1,000 mg thiamine reversed both neurologic and gastrointestinal symptoms within hours. It is hard to diagnose gast rointestinal beriberi since the symptoms are nonspecific. The patient underwent 11 emergency room visits, 3 hospital admissions, and laparoscopic cystectomy within 2 months, but the gastrointestinal symptoms continued to progres...
Source: Case Reports in Neurology - April 18, 2019 Category: Neurology Source Type: research

Trismus as a Presenting Symptom in a Case of Progressive Encephalopathy with Rigidity and Myoclonus
In this report we present a clinical case of trismus. The patient in question showed symptoms of trismus for 3 days, rapidly leading to respiratory insufficiency. Afterwards she developed myoclonus and progressive encephalopathy. Neurological workup showed no relevant abnormalities. A CT of the abdomen revealed a mass in the lower abdomen, which turned out to be an ovarian teratoma. Progressive encephalopathy with rigidity and myoclonus (PERM) was diagnosed clinically. Treatment with corticosteroids, benzodiazepines, and levetiracetam did not ameliorate the patient ’s condition. Only after the introduction of plasmap...
Source: Case Reports in Neurology - April 18, 2019 Category: Neurology Source Type: research

Herpes Simplex Virus Type 2 Radiculomyelitis Disguised as Conversion Disorder
We report the case of a patient with a lengthy psychiatric history who presented with lower extremity pain and weakness. Cervical, thoracic, and lumbar MRI scans with and without gadolinium contrast revealed no significant stenosis, neural compression, or other abnormal findings, and the brain MRI with and without gadolinium contrast was normal. The initia l diagnosis was conversion disorder due to myriad psychological stressors. Polymerase chain reaction (PCR) of CSF detected HSV-2 and a lymphocytic pleocytosis, and the diagnosis of radiculomyelitis was confirmed. She was treated with i.v. acyclovir for 3 weeks followed b...
Source: Case Reports in Neurology - April 17, 2019 Category: Neurology Source Type: research

Horner Syndrome as Complication of Acute Sphenoid Sinusitis
Horner syndrome is described in this case report as a rare complication of bacterial sphenoid sinusitis. A patient presented with miosis, ptosis, and ophthalmic nerve palsy with acute sphenoid sinusistis and cavernous sinus thrombosis on MRI. The impairment of sympathetic fiberscan can be explained through the direct septic effects of the sphenoid sinusitis and indirectly through thrombosis of the cavernous sinus at the level of the carotid plexus.Case Rep Neurol 2019;11:112 –116 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - April 15, 2019 Category: Neurology Source Type: research

Juvenile Muscular Atrophy of the Proximal Upper Extremity as So-Called Proximal-Type Hirayama Disease: Case Report and Review of the Literature
We report herein a case with Hirayama disease-like juvenile muscular atrophy involving proximal muscles in the upper extremities. In this case, in the flexion position of the neck, cervical magnetic resonance imaging revealed that the spinal cord was compressed by expansion of the posterior extradural space with forward displacement of the dura matter. These neuroimaging results are identical to those of Hirayama disease. However, the involved muscles in this case were the proximal muscles, unlike Hirayama disease. Five previous cases have displayed this rare subtype of Hirayama disease. The cause of the unique phenotype m...
Source: Case Reports in Neurology - March 21, 2019 Category: Neurology Source Type: research

Bilateral Chronic Subdural Hematoma in the Posterior Fossa Treated with a Burr Hole Irrigation: A Case Report and Review of the Literature
We report a case of bilateral CSH in the posterior fossa successfully treated with a single-burr hole surgery. A 74-year-old man under anticoagulation and antiplatelet therapy developed headache and nausea during observation for an asymptomatic supratentorial CSH. Radiological examinations revealed appearance of bilateral CSH in the posterior fossa associated with hydrocephalus. Upon rapid deterioration of the patient ’s consciousness, an urgent treatment was required. A burr hole was made near the transverse-sigmoid junction on the left side to access the hematoma. No ventricular drainage was placed as his conscious...
Source: Case Reports in Neurology - March 20, 2019 Category: Neurology Source Type: research

Citalopram Improves Obsessive-Compulsive Crossword Puzzling in Frontotemporal Dementia
Behavioral variant frontotemporal dementia (bvFTD) is characterized by severe changes in personality/behavior. Recent studies have provided evidence that a decrease in serotonin receptors and neuronal loss in the raphe nuclei play a role in the bvFTD pathology. Serotonergic antidepressants have been reported to diminish behavioral disturbances in bvFTD, particularly repetitive behaviors, disinhibition, apathy, sexually inappropriate behaviors, and hyperorality. Here, we present the case of an 80-year-old Caucasian male patient with clinically and biomarker supported bvFTD ( “probable” bvFTD; disease-specific al...
Source: Case Reports in Neurology - March 20, 2019 Category: Neurology Source Type: research

Transcranial Direct Current Stimulation Improves Pusher Phenomenon
An 83-year-old man suffered from cerebral infarction of the right middle cerebral artery territory. In association with severe left hemiparesis and hemispatial neglect on the left side, he showed severe pusher phenomenon (PP), which made rehabilitation difficult. Transcranial direct current stimulation (tDCS) was applied to the parietal area (2 mA × 20 min/day; anode on the right and cathode on the left) for 8 days, which resulted in remarkable improvement of PP and caused prolongation of static sitting time. tDCS of the parietal area could be a novel treatment option of PP following stroke.Case Rep Neurol 2019;11:61...
Source: Case Reports in Neurology - March 7, 2019 Category: Neurology Source Type: research

Paraneoplastic opsoclonus-myoclonus syndrome secondary to melanoma metastasis form occult primary cancer
Discussion: While the incidence of melanoma of unknown primary is between 2.6 and 3.2%, with a median overall survival ranging between 24 and 127 months, when melanoma patients develop OMS their survival is markedly decreased. Although only 5 cases of paraneoplastic OMS secondary to melanoma have been reported in the literature, all had a poor prognosis, dying within 8 months of OMS onset.Case Rep Neurol 2019;11:66 –79 (Source: Case Reports in Neurology)
Source: Case Reports in Neurology - March 7, 2019 Category: Neurology Source Type: research

Insomnia and Dysautonomia with Contactin-Associated Protein 2 and Leucine-Rich Glioma Inactivated Protein 1 Antibodies: A “Forme Fruste” of Morvan Syndrome?
Morvan syndrome (MoS) is typically characterized by neuromyotonia, sleep dysfunction, dysautonomia, and cognitive dysfunction. However, MoS patients with mild peripheral nerve hyperexcitability (PNH) or encephalopathy features have been described. A 46-year-old woman presented with a 2-month history of constipation, hyperhidrosis, and insomnia. Neurologic examination revealed muscle twitching and needle electromyography showed myokymic discharges in all limbs. No clinical or electrophysiological features of neuromyotonia were present. Although the patient denied any cognitive symptoms, neuropsychological assessment reveale...
Source: Case Reports in Neurology - March 7, 2019 Category: Neurology Source Type: research

An Outbreak of Peripheral Neuropathy in a Prison
Prisoners are at risk for both physical and psychological diseases. Here, we report an outbreak of peripheral neuropathy in a prison in northeast Thailand. Between July and December 2014, there were 88 male prisoners at Bueng Kan Provincial Prison in Bueng Kan, Thailand suffering from peripheral neuropathy out of a total of 1,464 prisoners (6.01%). The common age range was 20 –39 years (58 patients; 65.91%). The three most common features were hyporeflexia/areflexia of the lower extremities (36 patients; 83.72%). On laboratory vitamin B1 deficiency was detected in 4/5 patients, positive rhinovirus polymerase chain re...
Source: Case Reports in Neurology - February 26, 2019 Category: Neurology Source Type: research

Acute Intermittent Porphyria: A Report of 3 Cases with Neuropathy
The porphyrias are metabolic disorders due to a defect in the heme biosynthetic pathway. Patients have diverse clinical presentations with neuropathy being frequent in acute intermittent porphyria (AIP). Associated symptoms are abdominal pain and seizures. Three patients presenting with neuropathy were later diagnosed with AIP on the basis of clinical features, erythrocyte porphobilinogen deaminase activity, neuropathic patterns, and nerve conduction studies. Testing for the HMBS genetic mutation confirmed the diagnosis of AIP in 1 patient. The findings from this case series confirm that porphyric neuropathy in AIP is a pr...
Source: Case Reports in Neurology - February 8, 2019 Category: Neurology Source Type: research

Acute Ischemic Stroke in Pregnancy
Stroke is an uncommon but serious potential complication of pregnancy. The management of acute ischemic stroke in pregnant women remains a complex challenge that extends beyond the limits of clinical trial evidence. Patient 1 was a 29-year-old woman 27 weeks into her first pregnancy, without remarkable past medical history or vascular risk factors. She was admitted 1 h after sudden onset of a left total anterior circulation syndrome (National Institute of Health Stroke Scale [NIHSS] score of 23). CT and angio-CT scans were normal. Thrombolysis was performed, with mild clinical improvement. Brain MRI showed multi-territoria...
Source: Case Reports in Neurology - February 8, 2019 Category: Neurology Source Type: research