ABSTRACT
We present a patient with peritoneal carcinosarcoma who was treated with the alkylating agent ifosfamide and experienced a rapid decline in mental status. Electroencephalogram (EEG) displayed generalized periodic epileptiform discharges, which raised suspicion for nonconvulsive status epilepticus (NCSE). Following administration of midazolam, the patient's clinical condition and EEG improved. We review the 8 documented cases of ifosfamide-induced NCSE, and demonstrate the similarity in clinical features when compared with ifosfamide neurotoxicity that is not classified as NCSE. EEG findings suggesting an ictal pattern are subtle and heterogeneous, but they are essential for a diagnosis. Since it is unlikely that EEGs are uniformly obtained in instances of ifosfamide neurotoxicity, many cases of NCSE may go unrecognized.
Subject(s)
Carcinosarcoma/drug therapy , Electroencephalography/drug effects , Epilepsy, Generalized/chemically induced , Ifosfamide/adverse effects , Ifosfamide/therapeutic use , Peritoneal Neoplasms/drug therapy , Signal Processing, Computer-Assisted , Cerebral Cortex/drug effects , Cerebral Cortex/physiopathology , Diagnosis, Differential , Epilepsy, Generalized/diagnosis , Epilepsy, Generalized/drug therapy , Evoked Potentials/drug effects , Fatal Outcome , Female , Humans , Midazolam/therapeutic use , Middle AgedABSTRACT
Autoimmune autonomic ganglionopathy (AAG) and myasthenia gravis (MG) are both autoimmune channelopathies mediated by antibodies directed against nicotinic acetylcholine receptors. While both diseases target acetylcholine receptors, skeletal muscle and ganglionic receptor subtypes have key immunologic and genetic distinctions, and reports of patients with both AAG and MG are rare. We report a patient with antibody-confirmed AAG and elevated levels of ACh binding antibodies that did not meet clinical or electrodiagnostic criteria for MG. We presume that his skeletal muscle nAChR seropositivity was a false positive, perhaps due to the cross reactivity of the patient's ganglionic nAChR antibodies with skeletal nAChR subtypes.
Subject(s)
Autoantibodies/biosynthesis , Autoimmune Diseases of the Nervous System/diagnosis , Autoimmune Diseases of the Nervous System/immunology , Autonomic Nervous System Diseases/immunology , Myasthenia Gravis/immunology , Receptors, Nicotinic/immunology , Aged, 80 and over , Autoantibodies/blood , Autoimmune Diseases of the Nervous System/blood , Autonomic Nervous System Diseases/blood , Autonomic Nervous System Diseases/diagnosis , Cross Reactions/immunology , Diagnosis, Differential , False Positive Reactions , Ganglia, Autonomic/immunology , Ganglia, Autonomic/pathology , Ganglia, Autonomic/physiopathology , Humans , Male , Myasthenia Gravis/blood , Myasthenia Gravis/diagnosis , Receptors, Nicotinic/classificationABSTRACT
Acquired cerebellar tonsillar herniation is a known complication of lumboperitoneal shunt (LPS) for any indication, including idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri.(1) While the underlying pathophysiology of IIH remains unknown, increasing body mass index is a clear risk factor for the development of IIH. We describe an obese patient with IIH unresponsive to LPS who developed symptoms of intracranial hypotension and cerebellar tonsillar herniation after bariatric surgery and a 50-kg weight loss.