ABSTRACT
The association of focal motor seizures with cerebral hemiatrophy is a recognised rare paediatric syndrome known as 'hemiconvulsion, hemiatrophy and epilepsy' (HHE). To date, HHE has not been reported in adults. We present four adult patients with striking similarities to HHE, following alcohol withdrawal in chronic alcoholics. We document the imaging findings in the acute and subacute phases, discuss the underlying mechanisms and present a hypothesis regarding the pathophysiology.
Subject(s)
Alcoholism , Epilepsy , Substance Withdrawal Syndrome , Humans , Adult , Child , Brain/diagnostic imaging , Brain/pathology , Hemiplegia/complications , Hemiplegia/pathology , Substance Withdrawal Syndrome/complications , Substance Withdrawal Syndrome/pathology , Atrophy , Magnetic Resonance ImagingABSTRACT
BACKGROUND: Osmotic demyelination syndrome (ODS) is non-inflammatory demyelination in response to an osmotic challenge. It can be pontine or extrapontine in presentation. AIMS: To retrospectively review cases involving ODS and define the spectrum of causes, risk factors, clinical and radiological presentations, and functional outcomes. RESULTS: The study utilised data from 15 patients with a mean age of 53.6 years. Malnutrition (9; 60%) and chronic alcoholism (10; 66.7%) were the most common associated disorders. Two (13.3%) patients had severe hyponatraemia (<120 mmol/L). The average highest single-day change was 5.1 mmol/L. Radiologically, 14 (93.3%) had pontine and 6 (40%) had extra-pontine lesions. Hypokalaemia (14; 93.3%) and hypophosphataemia (9; 60%) were commonly associated. Common clinical manifestations include altered consciousness/encephalopathy (9; 60%), dysphagia (4; 26.7%) and limb weakness (4; 26.7%). At 3 months, two (14.3%) had died and six (40%) were functionally independent (modified Rankin scale 0-2). CONCLUSION: We found that ODS occurred despite appropriate correction rates of hyponatraemia. Factors such as malnutrition, chronic alcoholism, hypokalaemia and hypophosphataemia are thought to play a role in its pathogenesis. Approximately half of the patients survived and became functionally independent.
Subject(s)
Alcoholism , Hypokalemia , Hyponatremia , Hypophosphatemia , Malnutrition , Myelinolysis, Central Pontine , Humans , Middle Aged , Alcoholism/complications , Alcoholism/epidemiology , Myelinolysis, Central Pontine/diagnostic imaging , Myelinolysis, Central Pontine/epidemiology , Myelinolysis, Central Pontine/etiology , Hyponatremia/epidemiology , Hypokalemia/epidemiology , Retrospective Studies , Risk Factors , Hypophosphatemia/complications , Magnetic Resonance ImagingABSTRACT
An 82-year-old man was treated with neo-adjuvant nivolumab (programmed cell death protein 1 or PD-1 inhibitor) for local recurrence of melanoma developed myositis, myocarditis and a myasthenic-like syndrome with a fatal outcome. The occurrence of these three conditions may constitute a new immune checkpoint-induced syndrome. The relevance of the clinical features and the immunology is discussed. This case highlights the special role of anti-striated muscle antibodies as a predictor of mortality.
Subject(s)
Antineoplastic Agents, Immunological/therapeutic use , Melanoma/drug therapy , Muscle, Striated/drug effects , Myasthenia Gravis/chemically induced , Myocarditis/chemically induced , Myositis/chemically induced , Nivolumab/adverse effects , Aged, 80 and over , Antineoplastic Agents, Immunological/adverse effects , Humans , Male , Myocarditis/diagnosis , Myositis/diagnosis , Neoadjuvant Therapy , Neoplasm Recurrence, Local , Nivolumab/therapeutic useABSTRACT
A patient with metastatic melanoma developed myasthenia-like syndrome and paraspinal myositis with subsequent extraocular muscle atrophy associated with immune checkpoint inhibitor treatment. MRI scan of the ocular muscles on admission was normal, however 3 months later revealed significant extraocular muscle atrophy.