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Case Reports in Neurological Medicine 2020Chronic immune sensory polyradiculopathy (CISP) is an uncommon and treatable inflammatory disorder of the proximal sensory nerve roots. Patients typically present with...
Chronic immune sensory polyradiculopathy (CISP) is an uncommon and treatable inflammatory disorder of the proximal sensory nerve roots. Patients typically present with severe sensory ataxia, normal motor examination, unsteady gait, and normal nerve conduction studies (NCS). We describe an elderly man who presented with a two-week history of progressive numbness of both legs and recurrent falls. He had hyporeflexia, normal strength, severe proprioceptive, and vibration sense loss in both lower limbs and was unable to stand or walk because of severe sensory ataxia. The NCS and MR scan of the spine were normal. Tibial somatosensory evoked potentials revealed proximal conduction defect and localized the pathology to the lumbar sensory nerve roots proximal to the dorsal root ganglion. Cerebrospinal fluid showed cytoalbuminergic dissociation suggestive of inflammation. CISP was diagnosed; he was given aggressive immunotherapy consisting sequentially of corticosteroids with mycophenolate mofetil and three cycles of intravenous immunoglobulin after which he regained independent mobility. Unlike previous reports where patients presented months-years after symptom onset and improved after single-line immunotherapy, our patient presented fairly acutely and made dramatic improvement only after aggressive combination therapy. We urge physicians to recognize this uncommon neurologic cause of sensory ataxia where early aggressive treatment is crucial for better functional outcomes.
PubMed: 32148984
DOI: 10.1155/2020/6595086 -
Expert Opinion on Drug Safety Apr 2020: Immune checkpoint inhibitors (ICI) are associated with a wide spectrum of neurologic immune-related adverse events (irAEs) including meningo-encephalitis, myasthenia... (Review)
Review
: Immune checkpoint inhibitors (ICI) are associated with a wide spectrum of neurologic immune-related adverse events (irAEs) including meningo-encephalitis, myasthenia gravis and various neuropathies. Although relatively rare, they often present significant diagnostic complexity and may be under-recognized. Permanent neurologic deficits and/or fatality have been described but improvement is noted in most cases with ICI discontinuation and immunosuppressive therapy.: This review highlights the most frequently reported ICI-associated neurologic toxicities with a particular focus on those that may be more severe and/or fatal. Data from case series and pharmacovigilance studies is leveraged to provide an overview of associated clinical features, expected outcomes and appropriate management. Various immunobiologic triggers have been proposed to explain why certain patients might develop neurologic irAEs and are also briefly discussed.: All providers who care for patients with cancer should be made aware of common neurologic irAEs and able to recognize when prompt evaluation and consultation with appropriate specialists are indicated. Symptoms suggestive of encephalitis, myasthenia-gravis or an acute polyradiculopathy such as Guillain-Barre Syndrome (GBS) in patients exposed to these agents warrant immediate attention with a low threshold for hospitalization to expedite work-up and monitor for severe and/or life-threatening manifestations.
Topics: Animals; Antineoplastic Agents, Immunological; Humans; Immune Checkpoint Inhibitors; Neoplasms; Neurotoxicity Syndromes
PubMed: 32126176
DOI: 10.1080/14740338.2020.1738382 -
Turkish Journal of Physical Medicine... Jun 2019Peripheral nervous system complications such as acute demyelinating polyradiculopathy and mononeuropathy may rarely develop after substance use. A 27-year-old man used...
Peripheral nervous system complications such as acute demyelinating polyradiculopathy and mononeuropathy may rarely develop after substance use. A 27-year-old man used illegal drugs the day before his admission to the emergency service. Initially, he was suspected for rhabdomyolysis, due to elevated blood urea nitrogen, creatinine, aspartate aminotransferase, alanine aminotransferase, myoglobin, and creatine kinase levels. On Day 4, generalized edema and flask paralysis were noted in both upper limbs. The patient was diagnosed with bilateral brachial pan-plexopathy based on electrophysiological study results. He underwent a rehabilitation program. After eight months, repeated electrophysiological study revealed a significant improvement in all bilateral upper limb muscles, except for the right abductor pollicis brevis and abductor digiti minimi muscles. The underlying cause of bilateral brachial pan-plexopathy was rhabdomyolysis secondary to substance use. In conclusion, substance use in patients with non-traumatic plexopathy should always be questioned.
PubMed: 31893280
DOI: 10.5606/tftrd.2019.3157 -
Clinical Neurology and Neurosurgery Nov 2019Lyme disease is a common vector-borne illness in the U.S. caused by Borrelia species spirochetes. Neuroborreliosis has variable presentations, rarely manifesting as...
BACKGROUND
Lyme disease is a common vector-borne illness in the U.S. caused by Borrelia species spirochetes. Neuroborreliosis has variable presentations, rarely manifesting as meningoradiculitis or "Bannwarth Syndrome", characterized by painful radiculopathy, neuropathy, varying degrees of motor weakness, peripheral facial nerve palsy and cerebrospinal fluid (CSF) lymphocytic pleocytosis. We present a case of Bannwarth Syndrome manifesting with transaminitis and significant weight loss.
CASE PRESENTATION
A 60-year-old man with history of hypertension presented with 3 weeks of progressive back pain, bilateral arm and leg weakness, bilateral hand numbness and a right facial droop in absence of sphincter dysfunction. He reported an 11.3 kg unintentional weight loss and recent holiday to Egypt. Patient was afebrile with normal vital signs but with profound transaminitis on presentation. Exam revealed a lower motor neuron right facial nerve palsy, diffuse quadriparesis, areflexia but isolated brisk ankle reflexes. A left complete facial palsy developed shortly after admission. Concern for leptomeningeal plus peripheral nerve involvement led to consideration of oncologic, infectious and inflammatory etiologies, along with Guillain-Barre variants. Contrasted MRI of the brain and total spine was normal. CSF revealed lymphocytic pleocytosis (cell count 134), elevated protein (156) with normal glucose, cytology, AFB culture, viral PCRs and paraneoplastic antibodies. Serum and CSF Lyme IgG and IgM were positive. IV Ceftriaxone 2 g daily was started one day after admission. EMG/Nerve conduction studies showed diffuse polyradiculopathy without evidence of Guillain-Barre syndrome. Babesia co-infection was considered given unexplained transaminitis but PCR and quantitation were negative. CSF following 1 week of antibiotics showed improving cell and protein counts with resolving transaminitis. On follow-up at 2 months, facial paralysis, pain, motor and sensory deficits had resolved with return to baseline weight and liver function tests.
CONCLUSIONS
Bannwarth syndrome, a subacute painful meningoradiculitis caused by Borrelia species infection, is an uncommon presentation of neuroborreliosis in the U.S. Our case demonstrates previously unreported features such as profound transaminitis and weight loss without evidence of co-infection. Clinical manifestations of neuroborreliosis are variable, thus it is important to consider Bannwarth syndrome in the differential of meningoradiculitis in areas where Lyme Disease is prevalent.
Topics: Borrelia; Humans; Lyme Neuroborreliosis; Male; Middle Aged; Radiculopathy; Syndrome
PubMed: 31574359
DOI: 10.1016/j.clineuro.2019.105532 -
International Medical Case Reports... 2019Peripheral neuropathy is a common complication of Lyme disease. Cranial mononeuropathy, particularly that affecting the facial nerve, can be a presenting symptom, and at...
BACKGROUND
Peripheral neuropathy is a common complication of Lyme disease. Cranial mononeuropathy, particularly that affecting the facial nerve, can be a presenting symptom, and at times, it can be associated with polyradiculopathies or plexopathies. However, isolated femoral neuropathy has not yet been reported in Lyme disease; therefore, we felt the need to present this case.
CASE PRESENTATION
Laboratory investigations were performed on a 67-year-old man living in a region at high risk for Lyme disease after he developed erythema migrans on his chest, accompanied by the swelling of his left knee joint. A Western blot immunoglobulin assay was performed, including a screening for connective tissue disorders. Positive serological test results led to the administration of oral doxycycline therapy at a dosage of 100 mg twice daily. Shortly afterwards, he developed gait difficulties and frequent falls. The clinical examination and electrodiagnostic studies were consistent with femoral neuropathy. To look for etiologies other than Lyme disease, radiographic studies of his lumbar spine, pelvic cavity, retroperitoneal compartment, and hips were conducted. In addition, he was screened for diabetes. However, no other etiologies were found to explain the femoral neuropathy. Eventually, he recovered, and he was able to return to work.
CONCLUSION
We firmly believe that the femoral neuropathy and Lyme disease seen in this patient were causally related.
PubMed: 31534373
DOI: 10.2147/IMCRJ.S207889 -
Neurologia Sep 2020
Topics: Cauda Equina Syndrome; Humans; Intervertebral Disc Displacement; Leriche Syndrome; Polyradiculopathy
PubMed: 30926230
DOI: 10.1016/j.nrl.2018.12.015