Johns Hopkins University School of Medicine Clinico-Pathological Conference

1
Johns Hopkins University School of
MedicineClinico-Pathological Conference

• Benjamin M. Greenberg, M.D., M.H.S.
• Assistant Professor
• Department of Neurology
• Johns Hopkins School of Medicine

2
(No Transcript)
3
Differential Diagnosis at This Point
Cortex
Subcortex
Cerebellum
Brainstem
Spinal Cord
Nerve Root
Plexus
Nerve
Neuromuscular Junction
Muscle
4
Differential Diagnosis at This Point
Cortex
Subcortex
Cerebellum
Brainstem
Spinal Cord
Nerve Root
Plexus
Nerve
Neuromuscular Junction
Muscle
5
Differential Diagnosis at This Point
Cortex
White Matter
Subcortex
Cerebellum
Deep Gray
Brainstem
Spinal Cord
Nerve Root
Plexus
Nerve
Neuromuscular Junction
Muscle
6
Differential Diagnosis at This Point
Cortex
Subcortex
Cerebellum
Brainstem
Spinal Cord
Nerve Root
Plexus
Nerve
Neuromuscular Junction
Muscle
7
Differential Diagnosis at This Point
Cortex
Subcortex
Cerebellum
Brainstem
Spinal Cord
Nerve Root
Plexus
Nerve
Neuromuscular Junction
Muscle
8
Differential Diagnosis at This Point
Cortex
Subcortex
Cerebellum
Brainstem
Spinal Cord
Nerve Root
Plexus
Nerve
Neuromuscular Junction
Muscle
9
Differential Diagnosis at This Point
Cortex
Subcortex
Cerebellum
Brainstem
Spinal Cord
Nerve Root
Plexus
Nerve
Neuromuscular Junction
Muscle
10
Differential Diagnosis at This Point
Cortex
Subcortex
Cerebellum
Brainstem
Spinal Cord
Nerve Root
Plexus
Nerve
Neuromuscular Junction
Muscle
11
Differential Diagnosis at This Point
Cortex
Subcortex
Cerebellum
Brainstem
Spinal Cord
Nerve Root
Plexus
Nerve
Neuromuscular Junction
Muscle
12
Differential Diagnosis at This Point
Cortex
Subcortex
Cerebellum
Brainstem
Spinal Cord
Nerve Root
Plexus
Nerve
Neuromuscular Junction
Muscle
13
Differential Diagnosis at This Point
Cortex
Subcortex
Cerebellum
Brainstem
Spinal Cord
Nerve Root
Plexus
Nerve
Neuromuscular Junction
Muscle
14
(No Transcript)
15
Time course is important for defining pathologic
process, not localization.
16
Double vision brainstem or cranial nerve
17
Personality changes Frontal gt Temporal Lobe
18
Parkinsonism substantia nigra (deep gray)
19
? opsoclonus
20
Associated symptoms are important for defining
the pathology, not localization
21
Oculomotor
Limbic
Extrapyramidal
Frontal Lobe
Basal Ganglia
Midbrain
22
(No Transcript)
23
(No Transcript)
24
Subacute Central Nervous System Degeneration with
Parkinsonism, Eye Movement Abnormalities and
Frontal Love Dysfunction

• Degenerative
• Parkinsons Disease
• Progressive Supranuclear Palsy
• Corticobasal Degeneration
• Diffuse lewy body disease
• Toxic/Metabolic
• Wilsons
• Manganeese
• MPTP
• Infectious
• Whipples
• Prion Diseases
• Flaviviruses
• Mitochondrial Disorders

• Nutritional
• Deficiencies
• B12
• Thiamine
• Medications/Drugs
• Dopamine Antagonists
• Immune Mediatied
• Paraneoplastic
• ADEM
• Multiple Sclerosis
• Neoplastic
• CNS lymphoma
• Intravascular lymphoma
• Glioma

25
(No Transcript)
26
(No Transcript)
27
(No Transcript)
28
(No Transcript)
29
Subacute Central Nervous System Degeneration with
Parkinsonism, Eye Movement Abnormalities and
Frontal Love Dysfunction

• Degenerative
• Parkinsons Disease
• Progressive Supranuclear Palsy
• Corticobasal Degeneration
• Diffuse lewy body disease
• Toxic/Metabolic
• Wilsons
• Manganeese
• MPTP
• Infectious
• Whipples
• Prion Diseases
• Flaviviruses
• Mitochondrial Disorders

• Nutritional
• Deficiencies
• B12
• Thiamine
• Medications/Drugs
• Dopamine Antagonists
• Immune Mediatied
• Paraneoplastic
• ADEM
• Multiple Sclerosis
• Neoplastic
• CNS lymphoma
• Intravascular lymphoma
• Glioma

30
Whipples Disease

• First described in 1907 by George Hoyt Whipple, a
  JHH pathologist
• He developed recurring arthropathy, weight loss,
  and steatorrhoea, became worse, and died. At
  necropsy the intestine and mesenteric lymph
  glands were infiltrated by mononuclear cells and
  deposits of fat.
• Weakly gram positive rod, Actinomycetes
• Neurologically
• Cognitive decline
• Vertical Gaze difficulty
• Oculomasticatory myorhythmia
• Movement disorders

31
Paraneoplastic

• Subacute presentations
• Opsoclonus
• small cell lung cancer
• Neuroblastoma
• Gynecologic malignancies
• Limbic Encephalitis
• small cell lung cancer (anti-Hu)
• Germ cell tumors
• can have brainstem encephalitis.
• Can precede malignancy by years

32
(No Transcript)
33
Paraneoplastic Syndrome

• (Whipples is a close second)