Neuromuscular

Home, Search, Index, Links, Pathology, Molecules, Syndromes,
Muscle, NMJ, Nerve, Spinal, Ataxia, Antibody & Biopsy, Patient Info

ATAXIAS: General

Classification

Congenital

Hereditary
  Dominant
  Recessive
  X-linked

  Congenital
  DNA repair defects
  Episodic
  Metabolic disorders
  Mitochondrial
  Multisystem disorders

  Comparative pathology

Immune
  Acute cerebellar ataxia
  Miller-Fisher
  Multiple sclerosis
  Opsoclonus-Myoclonus
  Paraneoplastic
Infections: CNS
  Acute ataxia: Viral
  Creutzfeldt-Jakob
  Meningitis

Mass lesion
  Abscess
  Neoplasm
  Sarcoid

Paroxysmal
  Epilepsy
  Febrile
  Hereditary
  Migraine

Polyneuropathy
  Anti-MAG
  GALOP
  Sensory Neuron
  Large fiber

Supratentorial
  Gait disorders
    Elderly
    Extrapyramidal
  Hydrocephalus

Systemic
  Amyloid
  Autoimmune
    GAD antibodies
  Endocrine
    Hypoparathyroid
    Thyroid
  GI disorders
    Celiac disease; Sprue
    Vitamin E malabsorb
    Whipple's

Trauma

Vascular

Vestibular
Toxins & Drugs
  Drugs
    Amiodarone
    Cyclosporine
    Isoniazid
    Lithium
    Metronidazole
    Phenytoin
    Platins
      Carbo
      Oxali
  Toxins
    Buckthorn fruit
    Cyanide (Sensory)
    Ethanol
    Heavy metals
      Lead
      Mercury
    Methyl bromide
    Podophyllin (Sensory)
    Solvents
    TOCP

From: R Bucelli
Post-infectious Cerebellitis (T1 MRI)


Ataxia: Differential diagnostic testing 1


CEREBELLAR DYSFUNCTION: ANATOMY

Cerebellar lesionSigns or Function
Posterior
(Flocculo-nodular lobe; Archicerebellum)
Eye movement disorders: Nystagmus; Vestibulo-ocular reflex (VOR)
Postural and gait dysfunction
Midline
(Vermis; paleocerebellum)
Truncal & gait ataxia
Hemisphere
(Neocerebellum)
Limb ataxia: Dysmetria, Dysdiadochokinesis, "Intention" tremor
Dysarthria
Hypotonia
Afferent defect: Mossy fibers
  Vestibulopontine pathway
  Spinocerebellar system (Clarke)
    via: Inferior cerebellar peduncle
Dysmetria
Impaired stance & gait
Information about body parts in space
Vestibulocerebellar system
  Vestibular nucleus to Flocculonodular lobe
Situation & location of head
Outflow pathways: Dentate nucleus Postural instability
Limb ataxia
Inferior olive Disturbed teaching error signals
Ataxia developing over time:
  Especially trained limb movements (writing)

Ataxias: Comparative Pathology 2

Disease Dorsal root
ganglion
Clarke's
column
Vestibular
nucleus
Pontine
nucleus
Granule
cells
Purkinje
cells
Dentate
nucleus
Red
nucleus
Inferior
olive
DRPLA + +++ +
SCA6 + +++ ++ +
SCA31 +++
MSA + +++ + +++
SCA2 ++ +++ +++ +++ ++ + +++
SCA1 ++ + + ++ ++ ++ ++
SCA3 +++ ++ ++ + +++
FRDA +++ +++ ++ + +++
AOA1 +++ +++ + +++ + +


Patient Resources
Patient information

Return to Neuromuscular Syndromes
Return to Neuromuscular Home Page

References
1. Lancet Neurol 2010;9:94104
2. J Neurol Neurosurg Psychiatry 2015 Jan 30

11/17/2015