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Transcript of Johns Hopkins University School of Medicine Clinico-Pathological Conference Benjamin M. Greenberg,...
Johns Hopkins University Johns Hopkins University School of MedicineSchool of Medicine
Clinico-Pathological ConferenceClinico-Pathological Conference
Benjamin M. Greenberg, M.D., M.H.S.Benjamin M. Greenberg, M.D., M.H.S.
Assistant ProfessorAssistant Professor
Department of NeurologyDepartment of Neurology
Johns Hopkins School of MedicineJohns Hopkins School of Medicine
Differential Diagnosis at This Differential Diagnosis at This PointPoint
Cortex
BrainstemSpinal CordNerve RootPlexusNerveNeuromuscular JunctionMuscle
CerebellumSubcortex
Differential Diagnosis at This Differential Diagnosis at This PointPoint
Cortex
BrainstemSpinal CordNerve RootPlexusNerveNeuromuscular JunctionMuscle
CerebellumSubcortex
Differential Diagnosis at This Differential Diagnosis at This PointPoint
Cortex
BrainstemSpinal CordNerve RootPlexusNerveNeuromuscular JunctionMuscle
CerebellumSubcortex
White Matter
Deep Gray
Differential Diagnosis at This Differential Diagnosis at This PointPoint
Cortex
BrainstemSpinal CordNerve RootPlexusNerveNeuromuscular JunctionMuscle
CerebellumSubcortex
Differential Diagnosis at This Differential Diagnosis at This PointPoint
Cortex
BrainstemSpinal CordNerve RootPlexusNerveNeuromuscular JunctionMuscle
CerebellumSubcortex
Differential Diagnosis at This Differential Diagnosis at This PointPoint
Cortex
BrainstemSpinal CordNerve RootPlexusNerveNeuromuscular JunctionMuscle
CerebellumSubcortex
Differential Diagnosis at This Differential Diagnosis at This PointPoint
Cortex
BrainstemSpinal CordNerve RootPlexusNerveNeuromuscular JunctionMuscle
CerebellumSubcortex
Differential Diagnosis at This Differential Diagnosis at This PointPoint
Cortex
BrainstemSpinal CordNerve RootPlexusNerveNeuromuscular JunctionMuscle
CerebellumSubcortex
Differential Diagnosis at This Differential Diagnosis at This PointPoint
Cortex
BrainstemSpinal CordNerve RootPlexusNerveNeuromuscular JunctionMuscle
CerebellumSubcortex
Differential Diagnosis at This Differential Diagnosis at This PointPoint
Cortex
BrainstemSpinal CordNerve RootPlexusNerveNeuromuscular JunctionMuscle
CerebellumSubcortex
Differential Diagnosis at This Differential Diagnosis at This PointPoint
Cortex
BrainstemSpinal CordNerve RootPlexusNerveNeuromuscular JunctionMuscle
CerebellumSubcortex
Time course is important for definingpathologic process, not localization.
Double vision = brainstem or cranial nerve
Personality changes = Frontal > Temporal Lobe
Parkinsonism = substantia nigra (deep gray)
? opsoclonus
Associated symptoms are important fordefining the pathology, not localization
Oculomotor Limbic Extrapyramidal
Midbrain Frontal Lobe Basal Ganglia
Subacute Central Nervous System Degeneration Subacute Central Nervous System Degeneration with Parkinsonism, Eye Movement Abnormalities with Parkinsonism, Eye Movement Abnormalities
and Frontal Love Dysfunctionand Frontal Love Dysfunction
““Degenerative”Degenerative”– Parkinson’s DiseaseParkinson’s Disease– Progressive Supranuclear Progressive Supranuclear
PalsyPalsy– Corticobasal DegenerationCorticobasal Degeneration– Diffuse lewy body diseaseDiffuse lewy body disease
Toxic/MetabolicToxic/Metabolic– Wilson’sWilson’s– ManganeeseManganeese– MPTPMPTP
InfectiousInfectious– Whipple’sWhipple’s– Prion DiseasesPrion Diseases– FlavivirusesFlaviviruses
Mitochondrial DisordersMitochondrial Disorders
NutritionalNutritional– DeficienciesDeficiencies
B12B12ThiamineThiamine
Medications/DrugsMedications/Drugs– Dopamine AntagonistsDopamine Antagonists
Immune MediatiedImmune Mediatied– ParaneoplasticParaneoplastic– ADEMADEM– Multiple SclerosisMultiple Sclerosis
NeoplasticNeoplastic– CNS lymphomaCNS lymphoma– Intravascular lymphomaIntravascular lymphoma– GliomaGlioma
Subacute Central Nervous System Degeneration Subacute Central Nervous System Degeneration with Parkinsonism, Eye Movement Abnormalities with Parkinsonism, Eye Movement Abnormalities
and Frontal Love Dysfunctionand Frontal Love Dysfunction
““Degenerative”Degenerative”– Parkinson’s DiseaseParkinson’s Disease– Progressive Supranuclear Progressive Supranuclear
PalsyPalsy– Corticobasal DegenerationCorticobasal Degeneration– Diffuse lewy body diseaseDiffuse lewy body disease
Toxic/MetabolicToxic/Metabolic– Wilson’sWilson’s– ManganeeseManganeese– MPTPMPTP
InfectiousInfectious– Whipple’sWhipple’s– Prion DiseasesPrion Diseases– FlavivirusesFlaviviruses
Mitochondrial DisordersMitochondrial Disorders
NutritionalNutritional– DeficienciesDeficiencies
B12B12ThiamineThiamine
Medications/DrugsMedications/Drugs– Dopamine AntagonistsDopamine Antagonists
Immune MediatiedImmune Mediatied– ParaneoplasticParaneoplastic– ADEMADEM– Multiple SclerosisMultiple Sclerosis
NeoplasticNeoplastic– CNS lymphomaCNS lymphoma– Intravascular lymphomaIntravascular lymphoma– GliomaGlioma
Whipple’s DiseaseWhipple’s Disease
First described in 1907 by George Hoyt Whipple, First described in 1907 by George Hoyt Whipple, a JHH pathologista JHH pathologist– He developed recurring arthropathy, weight loss, and He developed recurring arthropathy, weight loss, and
steatorrhoea, became worse, and died. At necropsy steatorrhoea, became worse, and died. At necropsy the intestine and mesenteric lymph glands were the intestine and mesenteric lymph glands were infiltrated by mononuclear cells and deposits of fat.infiltrated by mononuclear cells and deposits of fat.
Weakly gram positive rod, Weakly gram positive rod, ActinomycetesActinomycetes NeurologicallyNeurologically– Cognitive declineCognitive decline– Vertical Gaze difficultyVertical Gaze difficulty– Oculomasticatory myorhythmia Oculomasticatory myorhythmia – Movement disordersMovement disorders
ParaneoplasticParaneoplastic
Subacute presentationsSubacute presentationsOpsoclonus Opsoclonus – small cell lung cancersmall cell lung cancer– NeuroblastomaNeuroblastoma– Gynecologic malignanciesGynecologic malignancies
Limbic Encephalitis Limbic Encephalitis – small cell lung cancer (anti-Hu)small cell lung cancer (anti-Hu)– Germ cell tumorsGerm cell tumors– can have brainstem encephalitis.can have brainstem encephalitis.
Can precede malignancy by yearsCan precede malignancy by years
Paraneoplastic SyndromeParaneoplastic Syndrome
(Whipple’s is a close second)(Whipple’s is a close second)