Neuropathology Review Questions 11/2/2012

Match the metal with the toxicity or description Arsenic Lead Mercury Manganese 1.Mees’ transverse white lines on fingernails 2.Psychological dysfunction 3.Parkinson’s symptoms 4.Red blood cell basophilic stippling 5.Brain levels increased by dimercaprol (BAL) 6.Symptoms improve with L-dopa 7.Increased urine coproporphyrin 8.Penicillamine and BAL are used in treatment Arsenic Mercury Mercury Manganese Manganese Lead Lead Lead

Arsenic Acute intoxication Acute intoxication – Encephalopathy – Peripheral neuropathy – Abdominal pain – Nausea/vomiting – Diarrhea – Shock Chronic intoxication Chronic intoxication – Malaise – Mees lines – Increased pigment & hyperkeratosis From insecticides From insecticides Detect in hair & urine Detect in hair & urine Treat with (BAL) Treat with (BAL)

Lead Kids Kids – Encephalopathy Adults Adults – Motor demyelinating peripheral neuropathy (wrist drop) – Anemia – Gingival lead line Diagnosis Diagnosis – Erythrocyte basophilic stippling – Long bone metaphyseal lead lines – Serum lead level – Increased urine proporhyrin & delta-aminolevulinic acid Treatment Treatment – EDTA – BAL – Penicillamine

Mercury Symptoms Symptoms – Psychologic dysfunction – Tremor, movement disorder, cerebellar signs – Peripheral neuropathy – GI dysfunction – Rental tubular necrosis From contaminated fish & felt hat dyes From contaminated fish & felt hat dyes Treatment Treatment – Penicillamine – BAL increases brain levels

Manganese Miners Miners Symptoms Symptoms – Parkinsonianism – Psychologic disorders – Headache Neuronal loss & gliosis in pallidum & striatum Neuronal loss & gliosis in pallidum & striatum Treatment Treatment – L-Dopa – Chelators do not help

Match the vitamin with is deficiency or toxicity 1.Wernicke’s encephalopathy 2.Korsakoff’s psychosis 3.Pellegra 4.Beriberi 5.Seen in rice eaters 6.Seen in corn eaters 7.Rickets 8.Pernicious Anemia 9.Subacute combined degeneration 10.Pseudotumor Thiamine Niacin Vitamin B 12 Vitamin A Vitamin DThiamine Thiamine Thiamine Thiamine Niacin Niacin Vitamin B 12 Vitamin A

Thiamine Deficiencies – Alcoholics – GI tumors – Dialysis – TPN – Gastric Plication – Europeans Involved in carbohydrate metabolism (transketolase) High transketolase activity in mamillary bodies

Wernicke Encephalopathy Signs Signs – Conjugate gaze & lateral rectus palsy – Nystagmus – Gait ataxia – Confusion Affected areas Affected areas – Mammilary bodies – Mediodorsal nucleus of thalamus – Periaqueductal gray – Floor of 4 th ventricle (dorsal motor nuclei of X, vestibular nuclei) – Superior cerebellar vermis Path findings Path findings – Brown-gray discoloration – Edema – Hemorrhage – Loss of purkinje cells – Reactive astrocytosis Lab findings Lab findings – Increased pyruvate & thymidine triphosphate – Decreased transketolase

Korsakoff Psychosis & Beriberi Korsakoff Korsakoff – Chronic disorder – Deterioration in memory – Lesions in dorsomedial thalamus Beriberi Beriberi – Common in rice eaters (refined) – Peripheral neuropathy – Autonomic dysfunction – Heart disease

Niacin Common in corn eaters (tryptophan deficiency) Common in corn eaters (tryptophan deficiency) Deficiency causes pellagra Deficiency causes pellagra – Dermatitis – Dementia – Diarrhea Similar syndrome caused by carcinoid tumors Similar syndrome caused by carcinoid tumors

Vitamin B 12 Deficiency causes Deficiency causes – Pernicious anemia – Tumors – Infection – Parasites – Nitrous Oxide Signs & Symptoms Signs & Symptoms – Megaloblastic anemia – Glossitis – Anorexia – Diarrhea – Impaired vibration sense, proprioception, paraplegia Spinal Cord Involvement Spinal Cord Involvement – Subacute combined degeneration – Lower C-Spine/Upper T-Spine posterior & lateral columns – Spongiform demyelination Diagnosis Diagnosis – Hypersegmented PMNs – B12 assay – Increased methylmalonic acid & homocysteine

Others Vitamin A Vitamin A – Deficiency: decreased vision – Toxicity: pseudotumor & increased ICP Vitamin B 6 Vitamin B 6 – Deficiency: lower limb paresthesia, pain & weakness Vitamin D Vitamin D – Deficiency: Rickets, decreased bone strength Vitamin E Vitamin E – Deficiency: thick dystrophic axons in posterior columns, polyneuropathy.