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1.
Parkinsonism Relat Disord ; 126: 107075, 2024 Sep.
Article in English | MEDLINE | ID: mdl-39067320

ABSTRACT

A 32-year old lady with AQP4-Antibody positive neuromyelitis optica developed a new-onset complex movement disorder after therapeutic plasma exchange, which was initially suspected to be hypocalcemic carpo-pedal spasm. However, when her bilateral, distal predominant, paroxysmal, stereotypic, wrist and finger flexor tonic contractions did not respond to serum calcium correction, other diagnoses were considered. The patient had a dramatic response to oral carbamazepine suggesting that the tonic spasms were likely a spinal movement disorder due to the primary demyelinating pathology.


Subject(s)
Plasma Exchange , Humans , Female , Adult , Plasma Exchange/methods , Movement Disorders/etiology , Neuromyelitis Optica/physiopathology
2.
Clin Neurol Neurosurg ; 240: 108260, 2024 05.
Article in English | MEDLINE | ID: mdl-38564992

ABSTRACT

A 63-year-old man with type 2 diabetes mellitus, alcohol consumption in moderation, and three episodes of hepatic encephalopathy presented with symmetrical lower limb distal weakness, sensory ataxia, thickened palpable nerves, mood disturbances for seven years, and a family history of schizophreniform disorders. Nerve conduction studies showed demyelinating sensorimotor polyradiculoneuropathy. CSF analysis showed mild albumino-cytological dissociation. MRI brain and lumbosacral plexus showed thickened fifth cranial nerves and lumbosacral roots. He was treated with steroids for a provisional diagnosis of chronic inflammatory polyneuropathy and became encephalopathic. EEG showed triphasic waves. Serum ammonia was 201 micrograms/dL. Further evaluation suggested ornithine transcarbamylase (OTC) deficiency. The patient underwent hemodialysis with a low protein diet, rifaximin, and sodium benzoate, with subsequent recovery.


Subject(s)
Neural Conduction , Ornithine Carbamoyltransferase Deficiency Disease , Humans , Male , Middle Aged , Ornithine Carbamoyltransferase Deficiency Disease/complications , Ornithine Carbamoyltransferase Deficiency Disease/diagnosis , Neural Conduction/physiology , Ataxia , Polyneuropathies/diagnosis , Magnetic Resonance Imaging , Diabetes Mellitus, Type 2/complications , Electroencephalography , Hepatic Encephalopathy/diagnosis , Renal Dialysis
4.
Qatar Med J ; 2022(4): 45, 2022.
Article in English | MEDLINE | ID: mdl-36304065

ABSTRACT

Hypernatremia (serum sodium>160 meq/L) present with diverse neurological manifestations, ranging from flaccid paralysis to impaired cognition, encephalopathy, and even deep coma. Osmotic demyelination refers to changes in brain cells because of an acute change in plasma osmolality. It is further divided into two types, i.e., central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM). Patients with EPM, besides spasticity, may also present with other movement disorders such as catatonia, parkinsonism, and dystonia. We present a case of a postpartum woman bought to the emergency department by her relatives in an unconscious state. In view of poor sensorium (Glasgow coma scale < 7), she was intubated and received mechanical ventilatory support. On admission, computed tomography ofthebrain was normal, and the patient was transferred to the intensive care unit (ICU) for further management. The preliminary work-up in the ICU showed hypernatremia (serum sodium of 182 mEq/L) with hyper-osmolality (359 mOsm/kgH2O). She was managed as per the ICU protocol for hypernatremia. During her ICU stay, her sensorium improved, but she developed flaccid paralysis and was quadriplegic. Thus, a tracheostomy was performed, and she was weaned from the ventilator. After ventilator weaning, she was transferred to the ward for further rehabilitation. During rehabilitation, the patient was able to sit and takefoodorally.To date, only a few cases are reported in postpartum women with acute severe hypernatremia caused by high-grade fever and loss of body fluids leading to extra-pontine demyelination and flaccid paralysis. This case highlightsthat prompt recognition and appropriate intervention can improve the outcomes in these patients.

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