Recurrent cryptogenic strokes in a young female as a novel presentation of Parry Romberg syndrome: a case report and review.

INTRODUCTION: Parry Romberg Syndrome (PRS) is a less common genetic condition presenting with progressive hemifacial atrophy involving skin, underlying connective tissue, muscle and facial bone. Neurological manifestations include seizures, headaches, deafness and trigeminal neuralgia refractory to medications, while stroke is a less common presentation. MATERIALS AND METHODS: A 43-year-old right-handed female with previous history of Lower Motor Neuron (LMN) type facial palsy, seizure disorder and linear scleroderma, presented to our clinic with recurrent cryptogenic strokes. She developed progressive hemifacial atrophy on the left side and left eye ectropion and was eventually diagnosed with rare Parry Romberg Syndrome. RESULTS: Patient underwent extensive work up for stroke to rule out etiologies like hyperlipidemia, diabetes, lupus and vasculitis. Peripheral labs for inflammatory markers and Cerebrospinal fluid (CSF) studies were unremarkable. Brain imaging at different points in time showed progressive atrophy of brain parenchyma, overlying bone, connective tissue and facial muscles on the left side. Central Nervous System (CNS) vessel imaging and diagnostic cerebral angiogram was unremarkable. CONCLUSION: This novel case underscores the potential CNS involvement in PRS, which is a rare disease entity. Neurological manifestations are not uncommon, including stroke. Further research is needed to understand the mechanisms of stroke in this rare disease process, that could help develop potential therapeutic targets.

When a Painful Rash Keeps Recurring: A Case of Seronegative Amyopathic Dermatomyositis Without Neurological Sequelae.

We present a case of seronegative amyopathic dermatomyositis (SADM). This clinical entity should be considered in the differential diagnosis of patients with recurring, painful erythematous skin manifestations, and requires close monitoring for the development of neurological manifestations and malignancy. SADM is a rare autoimmune disease that affects the skin and muscles. It is considered a subtype of dermatomyositis (DM), which is a systemic autoimmune disease. The exact cause of SADM is not fully understood but is believed to involve a complex interplay between genetic, environmental, and immunological factors. The diagnosis of SADM is typically made based on clinical evaluation, blood tests, muscle biopsy, and skin biopsy. Treatment options for SADM may include corticosteroids, immunosuppressive drugs, and other supportive measures to manage symptoms and prevent disease progression. A 30-year-old female presented with symptoms of intermittent burning, painful rash primarily on the hands and face. Her medical history was remarkable for a six-year history of multifocal joint pain, chronic low back pain, and intermittent, painful recurring rash in the upper body (face, neck, and chest). Neurological examination revealed scalp tenderness and arthralgia in the upper extremities, with normal motor strength examination. Skin findings included described an erythematous rash on the arms and hands bilaterally. Skin punch biopsy showed compact orthokeratosis, atrophy of the epidermis, interface changes, and increased dermal mucin on the colloidal iron stain, which are suggestive of DM. Electromyography and nerve conduction study were normal. The MRI of the left thigh was normal. C3 and C4 levels were reduced. The extended muscle-specific myositis panel including MDA5 was negative. The patient was placed on a multidrug regimen, including methotrexate, hydroxychloroquine, and prednisone. Within one year of follow-up, she was found to have reductions in skin manifestation and flare-ups. Clinicians should consider amyopathic DM (ADM) in the differential diagnosis of patients with recurring, painful skin manifestations. This condition can be easily overlooked as the development of neurological sequelae may be present much later in the course. We highlight the need for a multi-disciplinary management approach for patients with this unique diagnosis. Close monitoring for the development of neurological manifestations and associated sequelae including malignancy is recommended.

Cefepime-Induced Neurotoxicity or Nonconvulsive Status Epilepticus (NCSE): A Controversy Revisited.

Neurotoxicity is a well-known side effect of cefepime among patients commonly present with altered mental status and typical electroencephalogram (EEG) findings of generalized periodic discharges (GPDs). Some practitioners consider this pattern as encephalopathy and often treat it with the withdrawal of cefepime only, while others are at times concerned with non-convulsive status epilepticus (NCSE) and treat it with antiseizure medications (ASMs) in addition to the withdrawal of cefepime to accelerate the recovery. We present a case series of two patients who developed cefepime-induced altered mental status and EEG findings of GPDs at a rate of 2-2.5 Hz concerning for the ictal-interictal continuum (IIC). Both cases were treated as possible NCSE with ASMs in addition to the withdrawal of cefepime, resulting in different clinical outcomes. The first case showed clinical and EEG improvement shortly after the administration of parenteral benzodiazepines and ASMs. The other case showed electrographic improvement but did not show significant improvement in mentation, and the patient died eventually.

Motor Band Sign in Primary Lateral Sclerosis: A Case Report Proposing the Need for an Imaging Biomarker.

Motor neuron disease is a degenerative condition involving both upper motor neurons (UMN) and lower motor neurons (LMN). While amyotrophic lateral sclerosis (ALS) is an overlap of upper and lower motor neuron involvement, primary lateral sclerosis (PLS) is predominantly an upper motor neuron involvement with lower motor involvement seen in the later stages of illness. Diagnostic criteria rely on clinical features and electrodiagnostic tests such as electromyography (EMG). EMG predominantly helps in determining lower motor neuron involvement. No definitive objective measures are currently available to determine upper motor neuron involvement. We describe a patient diagnosed with PLS based on consensus diagnostic criteria. The patient had absent LMN features both clinically and on EMG. Magnetic resonance imaging (MRI) was significant for hypointense signals in the bilateral motor strip area on susceptibility weighted sequence, suggesting a surrogate marker of degeneration involving motor neurons in the brain. Early recognition of this MRI pattern called motor band sign (MBS) can help determine the earlier diagnosis of this neurodegenerative condition, potentially translating to better treatment and outcome measures.

Spinal Cord Infarction as a Cause of Acute Myelopathy.

Spinal cord infarction is an uncommon and often perplexing condition for emergency doctors to diagnose. Its initial symptoms are general and non-distinct, leading to frequent misdiagnosis. This case report is about a 56-year-old woman who presented to the hospital with substernal tightening chest pain and rapidly progressing bilateral lower-extremity weakness. Initially, she was diagnosed with spinal cord infarction based on magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) studies, with all other differential diagnoses ruled out. This article explores the utility of advanced MRI techniques, particularly diffusion-weighted imaging (DWI) sequence, in diagnosing spinal cord infarction. This is especially pertinent in patients who present with atypical symptoms and do not have conventional risk factors for spinal cord ischemia.

A Devastating Neurological Disorder: Anti-Dipeptidyl-Peptidase-Like Protein 6 (DPPX) Encephalitis Causing Rapidly Progressive Dementia.

Rapidly progressive dementia (RPD) is caused by a heterogeneous group of neurological disorders, and the prototype is Creutzfeldt-Jakob disease (CJD). However, treatable causes including autoimmune encephalitis are often underrecognized and undertreated. A 72-year-old female patient was admitted with a 10-month history of rapidly progressive cognitive decline, visual hallucinations, paranoid behavior, diarrhea, and an 18-kg unintentional weight loss. On the physical exam, she was only oriented to the person and demonstrated an exaggerated startle response with diffuse rigidity. The initial clinical suspicion included CJD versus autoimmune encephalitis. Comprehensive laboratory testing, thyroid peroxidase, thyroglobulin antibodies, and autoimmune encephalitis panel were negative. The EEG showed mild to moderate diffuse slowing without any epileptiform abnormalities. An MRI brain revealed mild hippocampal atrophy. CSF testing revealed mild lymphocytic pleocytosis; RT-QuIC analysis and 14-3-3 protein were negative. There was no clinical improvement after treatment with IV steroids and IVIG. Repeated autoimmune encephalitis panel testing performed on a research basis was positive for dipeptidyl-peptidase-like protein 6 (DPPX) antibodies in serum and CSF. Unfortunately, our patient passed away before additional treatment could be attempted. Anti-DPPX encephalitis is a rare autoimmune disorder and an unrecognized cause of RPD. Early diagnosis and rapid escalation of treatment are imperative to avoid devastating neurological consequences.

Antiganglioside antibodies and paraneoplastic neuromuscular junction disorder?

Based on the instructions in "Guide for authors", our manuscript is a case reports and was submitted under "Letters to the Editor", which should not include an abstract.

Multiple Sclerosis: Clinical Updates in Women's Health Care Primary and Preventive Care Review.

Multiple sclerosis (MS) is a chronic inflammatory and demyelinating disease of the central nervous system. The disease affects more women than men and often is diagnosed during a woman's childbearing years. Typical clinical presentations of the disease are extensive and variable, with symptoms that include dysregulated mood, fatigue, vision problems, weakness, tremor, imbalance, abnormal sensations, bladder dysfunction, and heat sensitivity. If a woman aged 15-50 years experiences these neurologic symptoms in isolation or combination, and the symptoms are not explained by other underlying medical conditions, MS should be suspected. Multiple sclerosis can be divided into four clinical subtypes: 1) relapsing-remitting MS, 2) secondary progressive MS, 3) primary progressive MS, and 4) clinically isolated syndrome. Relapsing-remitting MS at the time of onset is the most common form and accounts for approximately 80% of all cases of MS. Relapsing-remitting MS does not affect life expectancy. However, because of the neurodegenerative and progressive course of the disease, patients accumulate physical and cognitive disabilities over time that result in impaired ability to work, increased financial burden, and slightly increased mortality. A variety of possible risk and prognostic indicators have been identified that may predict the course of disease, particularly the extent of relapses and disability. Multiple sclerosis currently is incurable, but many disease-modifying therapies are available that can reduce the frequency of clinically evident exacerbations and accumulation of disease burden as defined by the number of lesions identified on magnetic resonance imaging. The choice of disease-modifying therapies, contraception use, and treatment of symptoms should be individualized based on age at onset and disease activity and, during pregnancy, the gestational age. Proactive management of MS across the woman's life cycle reduces morbidity, improves maternal and fetal health during pregnancy and the postpartum period, and increases quality-of life-measures for patients and their families.

Use of noninvasive induction techniques in the diagnosis of PNES.

The diagnosis of psychogenic nonepileptic seizures (PNES) remains challenging. In the correct clinical setting with prolonged electroencephalography (EEG) monitoring, the specificity of provocative techniques to distinguish induced epileptic event from a nonepileptic event approaches 90%. We report our epilepsy monitoring unit (EMU) experience with the use of noninvasive verbal suggestion (VS) during hyperventilation (HV), photic stimulation (PS) as induction technique in making the diagnosis of PNES. In total, 189/423 patients were diagnosed with PNES during the EMU evaluation. Of the 189, 20 had mixed disorder and 169 patients had only PNES, 80 patients (47.3%) had a PNES with induction, and the remaining 89 of 169 patients (52.7%) had a spontaneous PNES episode that did not require induction. Verbal suggestion during HV and PS confirmed the diagnosis of PNES in 47% of the patients who otherwise did not have spontaneous events. Within the group who was diagnosed with PNES following induction, antiepileptic drugs (AEDs) were stopped in 53% of the patients. We believe that this is a large proportion of patients that would possibly remain undiagnosed if no induction were performed.

Fascicular Involvement of the Posterior Tibial Nerve as a Result of Perineural Ganglion Cyst at the Posterior Tibial Nerve in the Calf: A Case Report and Review of Literature.

We report a 19-year-old woman with a 6-month history of nontraumatic left foot numbness associated with intermittent weakness. Nerve conduction studies and electromyography localized the lesion to the posterior tibial nerve, below the innervation to the soleus and medial gastrocnemius muscles. MRI of the left leg revealed a multiloculated cystic collection near the proximal tibiofibular joint. Surgical excision and pathology confirmed the diagnosis of a ganglion cyst, in an atypical location distal to the popliteal fossa. We believe this is the first reported case of fascicular posterior tibial nerve involvement by a ganglion cyst in the calf.

Compression of lateral antebrachial cutaneous nerve in waitresses.

OBJECTIVE: In a retrospective survey of our electrophysiology laboratory, we encountered 3 cases of lateral antebrachial cutaneous nerve (LACN) compression in waitresses and propose direct compression of LACN by carrying heavy trays on the lateral bicipital tendon. LACN, a branch of musculocutaneous nerve, provides sensory innervations to lateral forearm. Causes of LACN involvement include venipuncture, elbow surgery, and trauma. METHODS: We encountered 6 cases of LACN neuropathy, 3 cases that were in slim waitresses carrying heavy trays. History and physical examination and forearm electrophysiologic studies (EPS) were performed in 3 waitresses at initial and follow-up visits. Antidromic stimulation of LACN was undertaken by Spindler and Felsenthals technique. Latency, amplitude, and conduction velocity were measured and compared with contralateral limb. RESULTS: All 3 patients were female waitresses aged 35-42 years, presented with few months of painful paresthesias of distal forearm, worse during working hours. Tinel sign at lateral cubital fossa was positive in all. EPS confirmed delayed latency, low amplitude, and slow conduction velocity of LACN on symptomatic side compared with normal. All 3 patients revealed electrophysiologic abnormalities consistent with LACN compression due to heavy trays in slim waitresses and considered occupational hazard. Treatment included nortriptyline or gabapentin and avoidance of heavy trays. Clinical and EPS findings improved over 8 months. CONCLUSIONS: We described 3 cases of LACN compression in waitresses from edges of heavy trays with typical symptoms and abnormal electrophysiological studies and improvement by avoiding compression and with analgesics. We propose inadequate fatty tissue in antecubital fossa contributed to compression of LACN.