TABLE 1
FROM:
Acute chorea and bilateral basal ganglia lesions in a hemodialysis patient
K Kiryluk, F Khan and A Valeri
BACK TO ARTICLETable 1. Differential diagnosis for basal ganglia lesions in a hemodialysis patient
| Cause | Mechanism/Comments | Workup in this case |
|---|---|---|
| Hereditary | ||
| Late-onset Wilson's disease | Autosomal recessive defect in cellular copper transport | No hepatic disease, no cognitive changes, no corneal rings, spontaneous resolution |
| Late-onset Huntington's disease and related genetic disorders2 | Progressive neurodegenerative disorders characterized by chorea, cognitive decline, and behavioral changes | Negative family history, no behavioral or cognitive impairment, spontanous resolution |
| Endocrine | ||
| Hyperthyroidism | Unknown mechanism | Normal thyroid function tests |
| Fahr's syndrome | Calcification of the basal ganglia | No calcifications in the basal ganglia by imaging |
| Electrolyte/metabolic disorders | ||
| Hypo/hypernatremia3, 4 | Basal ganglia edema/extrapontine myelinolysis | Normal serum Na levels, no history of dysnatremia |
| Hypo/hyperglycemia5, 6, 7, 8 | Very rare cause of isolated chorea | Normal serum glucose levels, HgbA1C 7.8% |
| Organic acidosis9, 10 | Unclear mechanism, rare | Normal bicarbonate, no anion gap |
| Uremia/hemodialysis1, 11, 12, 13, 14, 15, 16 | Chronic kidney disease, underdialysis | Stable and adequate dialysis treatments |
| Thiamine deficiency17 | Malnutrition | Normal serum thiamine levels |
| Drugs and toxins | ||
| Pharmacologic agents2 | Antipsychotic, anti-Parkinsonian, and antiepileptic agents, verapamil, baclofen, tricyclics, lithium, theophylline, digoxin, cyclosporine, steroids, OCPs | Not taking |
| Psychostimulants | Cocaine, amphetamines | Negative toxicology screen |
| Alcohols | Ethanol, methanol,18 ethylene glycol19 | Negative toxicology screen |
| Carbon monoxide20 | Hypoxic basal ganglia injury | Normal carboxyhemoglobin levels |
| Manganese,21 Aluminum,22 Lead | Direct basal ganglia toxicity | Normal serum levels |
| Vascular | ||
| Cerebrovascular disease | Thrombosis/infarction | Normal MRA, symmetric bilateral lesions |
| Polycythaemia vera | Hyperviscosity/infarction | Normal hematocrit |
| HUS/TTP | Microthrombosis/infarction | No hemolysis, normal platelet count |
| Moyamoya disease | Progressive arterial occlusive disease, likely hereditary | Normal MRA, no family history |
| Post-pump chorea23 | Complication of extracorporeal circulation | No documented intradialytic hypotension |
| Autoimmune/inflammatory | ||
| SLE | Vasculitis/inflammation | Negative autoimmune serologies, normal complement levels, no systemic symptoms. |
| APS | Thrombosis/infarction | Negative APL/ACL serologies |
| Small vessel vasculitis | Vasculitis/inflammation | Negative ANCA, normal CRP, no systemic symptoms. |
| Multiple sclerosis plaques | Demyelination/inflammation | No evidence of plaques on imaging, CSF fluid negative for oligoclonal bands |
| Sydenham's chorea | Rheumatic fever, typically in childhood, antibody-mediated (molecular mimicry between streptococcal and CNS antigens) | Negative ASO titers, advanced age, no evidence of rheumatic fever |
| Postinfectious or postviccinal encephalitis | Autoimmune reaction following exposure to foreign antigens (molecular mimicry) | No history of recent infections or vaccinations |
| Neurosarcoidosis24 | Granulomatous inflammation may rarely involve basal ganglia | No evidence of sarcoidosis on MRI, lack of systemic symptoms, normal ACE levels |
| Infections | ||
| HIV/AIDS | Possible direct HIV infection of subcortical structures | Negative HIV test |
| Neurosyphilis | Direct infection in the basal ganglia | Negative RPR and CSF VDRL |
| Bacterial endocarditis | Typically unilateral septic embolic disease | Bilateral lesions, no evidence of endocarditis |
| Parasitic infections and TB | Toxoplasmosis, cysticercosis, tuberculous granulomas | No evidence by imaging, negative PPD |
| Creutzfeldt–Jakob Disease | Sporadic human prion disease | Negative CSF 14-3-3 protein, spontaneous resolution |
| Tumors | ||
| Mass lesions | CNS lymphoma or metastatic lesions | No evidence of tumor on imaging |
| Paraneoplastic syndromes | Anti-Hu and anti-CRMP5 antibodies | Negative paraneoplastic antibody panel |
ACE, angiotensin converting enzyme; ACL, anti-cardiolipin; ANCA, anti-neurtophilic cytoplasmic antibodies; APL, anti-phospholipid; APS, anti-phospholipid syndrome; ASO, anti-streptolysin O; CNS, central nervous system; CRP, C-reactive protein; CSF, cerebrospinal fluid; Hgb, hemoglobin; HUS, hemolytic uremic syndrome; MRA, magnetic resonance angiogram; MRI, magnetic resonance imaging; OCPs, oral contraceptive pills; PPD, purified protein derivative; RPR, rapid plasma reagin; SLE, systemic lupus erythematosus; TB, tuberculosis; TTP, thrombotic thrombocytopenic purpura; URR, urea reduction ratio; VDRL, veneral disease research laboratory.
Only selected relevant causes are referenced.
