Acute Demyelinating Encephalomyelitis (ADEM) in COVID-19 Infection: A Case Series.

OBJECTIVE: The purpose of this study was to report three patients COVID-19 infection with severe respiratory syndrome requiring intubation, who developed acute demyelinating encephalomyelitis (ADEM). METHODS: Patient data were obtained from medical records from the North Memorial Hospital, Robbinsdale, MN, USA. RESULTS: Three patients (two men and one woman, aged 38-63) presented with fatigue, cough, and fever leading to acute respiratory distress syndrome secondary to COVID-19 infection requiring ventilatory support. Two patients were unresponsive and the third patient had severe diffuse weakness. MRI in all patients showed findings consistent with ADEM. CSF showed elevated protein in all patients with normal cell count and no evidence of infection, including negative COVID-19 PCR. All three patients were treated with intravenous corticosteroids and one improved markedly. The other two had minimal response to steroids and no further improvement after IVIG. CONCLUSION: Neurological complications from COVID-19 are being rapidly recognized. Our three cases highlight the occurrence of ADEM as a postinfectious/immune-mediated complication of COVID-19 infection, which may be responsive to corticosteroid treatment.

Fingolimod-associated macular edema controlled with nepafenac non-steroidal anti-inflammatory opthalmologic applications.

BACKGROUND: Fingolimod, an immunomodulatory agent, is used for the treatment of relapsing-remitting multiple sclerosis (RRMS). Fingolimod-associated macular edema (FAME) is a known complication with an incidence of 0.4%. The current recommendation for treatment of FAME is cessation of fingolimod. There are few case reports with management of FAME with steroid eye drops. CASE PRESENTATION: A 38-year-old Caucasian female patient with history of relapsing-remitting multiple sclerosis (RRMS) and treated with fingolimod developed Fingolimod-associated macular edema (FAME). Nevertheless, FAME was successfully treated with nonsteroidal anti-inflammatory eye drops without discontinuation of fingolimod. CONCLUSION: FAME may be managed with non-steroidal eye drops without discontinuation of fingolimod in appropriate patient monitored with close follow up.

Safety, tolerability, and cerebrospinal fluid penetration of ursodeoxycholic Acid in patients with amyotrophic lateral sclerosis.

OBJECTIVE: Amyotrophic lateral sclerosis is a progressive degenerative disease, which typically leads to death in 3 to 5 years. Neuronal cell death offers a potential target for therapeutic intervention. Ursodeoxycholic acid is a cytoprotective, endogenous bile acid that has been shown to be neuroprotective in experimental Huntington and Alzheimer diseases, retinal degeneration, and ischemic and hemorrhagic stroke. The objective of this research was to study the safety and the tolerability of ursodeoxycholic acid in amyotrophic lateral sclerosis and document effective and dose-dependent cerebrospinal fluid penetration. METHODS: Eighteen patients were randomly assigned to receive ursodeoxycholic acid at doses of 15, 30, and 50 mg/kg of body weight per day. Serum and cerebrospinal fluid were obtained for analysis after 4 weeks of treatment. Treatment-emergent clinical and laboratory events were monitored weekly. RESULTS: Our data indicated that ursodeoxycholic acid is well tolerated by all subjects at all doses. We also showed that ursodeoxycholic acid is well absorbed after oral administration and crosses the blood-brain barrier in a dose-dependent manner. CONCLUSIONS: These results show excellent safety and tolerability of ursodeoxycholic acid. The drug penetrates the cerebrospinal fluid in a dose-dependent manner. A large, placebo-controlled clinical trial is needed to assess the efficacy of ursodeoxycholic acid in treating amyotrophic lateral sclerosis.

Treatment of chronic inflammatory demyelinating polyneuropathy with pulsed oral steroids.

BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy that responds to various immunosuppressive treatments. Oral daily prednisone therapy is effective and inexpensive, but the long-term treatment that is usually necessary leads to serious adverse effects. Consequently, intravenous immunoglobulin and plasma exchange have been widely used to treat CIDP, making treatment expensive and inconvenient. A steroid regimen that reduces adverse effects but preserves efficacy would simplify treatment. Pulsed steroids have nongenomic actions not seen with low-dose steroids, including rapid inhibition of arachidonic acid release and of calcium and sodium cycling across plasma membranes of immune cells. OBJECTIVE: To study the efficacy, safety, and tolerability of pulsed oral methylprednisolone therapy in patients with CIDP. DESIGN: Open-label prospective study. SETTING: University of Minnesota Neuropathy Center, Minneapolis. PATIENTS: Ten patients (3 women and 7 men) with CIDP followed up for at least 22 months. MAIN OUTCOME MEASURES: Neuromuscular score and Inflammatory Neuropathy Cause and Treatment (INCAT) disability score were used as outcome measures for efficacy; weight, blood pressure, changes in bone density, and steroid-related adverse effect questionnaire were used as outcome measures for safety. RESULTS: This steroid regimen leads to significant improvement in weakness and disability in all patients treated and to off-treatment remission in 60% of patients. Treatment was fairly well tolerated, and only 1 patient discontinued treatment because of adverse effects. Steroid-induced osteoporosis remained a problem, especially in older patients. CONCLUSIONS: Pulsed oral methylprednisolone may be efficacious in the long-term treatment of CIDP and is relatively well tolerated. Remission can be induced in most patients, especially those with a shorter duration of disease.

Response to oral supplementation in copper deficiency myeloneuropathy.

Neurological manifestations due to copper deficiency include ataxic myeloneuropathy that resembles subacute combined degeneration due to B12 deficiency. We report our experience in the treatment of 10 patients with copper deficiency myeloneuropathy and conclude that copper supplementation leads to stabilization rather than improvement in the neurological deficits.

Isolated high-frequency jaw tremor relieved by botulinum toxin injections.

Jaw tremor can be seen as a component of various neurological disorders such as essential tremor, Parkinson's disease, dystonia, branchial myoclonus, hereditary geniospasm, task-specific tremor, and Whipple's disease, as well as in normal situations such as shivering, and subclinical physiological jaw tremor. In most of these conditions, the jaw tremor is usually associated with tremor or other abnormal involuntary movements affecting additional body parts, and its frequency is lower than 12 Hz. Schrag and colleagues reported a patient with a high-frequency idiopathic jaw tremor, and they speculated it could be related to orthostatic tremor affecting the masseter muscles. We encountered a similar patient with intermittent rapid focal jaw tremor that was successfully treated with botulinum toxin injections to the masseters.

Diabetic neuropathy.

Diabetic neuropathy is not a single entity but manifests as several different clinical syndromes. It is likely that different pathophysiological mechanisms contribute to the development of the neuropathy, including metabolic alterations, microvascular changes, and inflammatory changes. A summary of the underlying pathophysiological mechanisms and clinical aspects of different diabetic neuropathies are outlined in this article.

Myokymia in axonal disorders.

Myokymic discharges are spontaneous bursts of individual motor units occurring rhythmically or semirhythmically. Myokymia is typically seen in demyelinating disorders. We present 5 patients with myokymia in axonal disorders, including a case of myokymia after Botox injection, which has not been reported previously.

Posterior reversible encephalopathy syndrome as the initial manifestation of Guillain-Barré Syndrome.

BACKGROUND: Guillain-Barré Syndrome (GBS) frequently affects autonomic nerves with subsequent fluctuations in blood pressure. Posterior reversible encephalopathy syndrome (PRES) is a descriptive radiological term for hypertensive encephalopathy. This article describes a unique case in which autonomic neuropathy led to marked elevations in blood pressure with subsequent PRES prior to the significant motor weakness and diagnosis of GBS. METHODS: To describe a patient who presented with PRES and GBS. RESULTS: A 58-year-old female presented to the local emergency room with complaints of acute sharp thoracolumbar back pain. Within the following 2 days, her blood pressure showed marked elevation with sudden episodic falls. She subsequently became encephalopathic and had a tonic-clonic seizure. Brain MRI was consistent with PRES. By day 5 of her initial presentation, she had developed weakness and areflexia with the electrophysiological findings of GBS. Her sensorium recovered, and MRI changes reversed with control of blood pressure. She was treated with intravenous immunoglobulin with full recovery back to baseline over the next 4 to 5 months. CONCLUSION: The autonomic effects of GBS may cause hypertensive encephalopathy or PRES prior to the motor manifestations.

Sensory-predominant, painful, idiopathic neuropathy: inflammatory changes in sural nerves.

Sensory-predominant, painful, idiopathic neuropathies are a common clinical problem. In this retrospective study of 11 patients with such a neuropathy, we found axonal loss in 9, with multifocal axonal loss in 4 patients; there were large perivascular mononuclear inflammatory infiltrates (>50 cells) in 4 and smaller infiltrates (10-20 cells) in 4. These findings suggest that some cases of this neuropathy may be due to autoimmune vasculopathy and therefore may respond to immunotherapy.

Idiopathic brachial neuritis.

Idiopathic brachial neuritis is a well defined clinical condition that most commonly affects young adults, seen usually by primary care physicians, neurologists or orthopaedic surgeons. Its onset is characterized by acute, aching shoulder pain lasting a few days to weeks, followed by progressive shoulder girdle and upper extremity weakness and atrophy, with a slow but progressive recovery of motor function over 6 to 18 months. Its early recognition can help avoid unnecessary and potentially harmful diagnostic and therapeutic interventions, and avoid delays in prescribing appropriate therapies that may be helpful only early in the course of the disease. We present a case of idiopathic brachial neuritis and discuss important aspects of the disease and difficulties in reaching the correct diagnosis.

Childhood-onset chronic inflammatory demyelinating polyradiculoneuropathy with cranial nerve involvement.

A 17-year-old male presented with chronic diplopia and generalized motor weakness. He was previously diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and acute disseminated encephalomyelitis in childhood. Cranial magnetic resonance imaging (MRI) revealed a rarely reported finding of thickening and enhancement of multiple cranial nerves. Nerve conduction studies and electromyography showed peripheral nerve demyelination with axonal involvement. There was improvement in the clinical examination, MRI, and electrophysiologic studies after combined corticosteroid and plasma exchange therapy. We review the clinical presentation, course, and response to therapy among children with chronic inflammatory demyelinating polyradiculoneuropathy, with specific emphasis on the frequency and pattern of cranial nerve involvement.