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MOTOR SYNDROMES

Motor
  Differential diagnosis
  Hereditary

Amyotrophic lateral sclerosis
  Hereditary
  Sporadic
  ALS variants
    EOM & Extrapyramidal change
    Primary lateral sclerosis (PLS)
    Primary muscular atrophy (PMA)
    Western Pacific ALS
Amyotrophy
  Lower extremity, benign
  Monomelic
Diabetic amyotrophy
Hand weakness
Hopkins
Infections
Insulinoma
Mitochondrial: SCO2
Motor Neuron Disorders
  Hereditary
  Bulbar
  Lower motor neuron
  Upper motor neuron
  Asymmetric
  Symmetric
Motor Neuropathy
  Distal
    Hereditary
    Antibodies
      IgG vs GM1
      IgM vs GM1
      IgM vs GalNAc-GD1a
      IgM vs NS6S
  Multifocal motor (MMN)
Myopathies
Painful
Paraneoplastic
  Lymphoma
  Breast
Poliomyelitis & Post-polio syndrome
Rapid onset
  Acute Axonal Motor Neuropathy
  Poliomyelitis
  Porphyria
Spinal muscular atrophy: Hereditary
  SMN (5q)
  Androgen Receptor (Bulbar SMA)
  Distal SMA (Distal HMN)
  Hexosaminidase A (Tay-Sachs)
  Scapuloperoneal
  Spinal muscular atrophy 2
  X-linked Infant & Arthrogryposis
Spastic paraparesis
Toxic

From: Quain



Motor Neuron Disorders: Differential Diagnoses



Paraneoplastic Motor Neuropathy


Ramon y Cajal

Paraneoplastic Lower Motor Neuron Syndrome8



HOPKINS' SYNDROME: Acute post-asthmatic amyotrophy



Post-Polio Syndrome

Old Polio
Asymmetric atrophy & weakness

Atrophic right leg (arrow) in patient
with paralytic polio 70 years in past

Monomelic Amyotrophy


Amyotrophy: Benign Lower Extremity11

Benign lower limb amyotrophy: MRI

From: M Al-Lozi
Legs: Involvement of posterior muscle groups

From: M Al-Lozi
Thigh: Long head of biceps involvement


Gowers
Primary muscular atrophy
Wasting: Includes
  paraspinous muscles.

Putnam

"Primary Muscular Atrophy" (PMA) 15




Primary Lateral Sclerosis 2

Dominant, Adult onset
  4p16
  FIG4: 6q21
PLS + FTD
Recessive, Childhood onset
  ALS2
  ERLIN2
  SIGMAR1
  SPG11
Sporadic, Adult onset

From Bramwell: Diseases of the Spinal Cord

PLS: "Sclerosis" of pyramidal tracts (Upper thoracic cord)

PLS : Sporadic, Adult onset 14

PLS: Recessive, Juvenile onset9
  Alsin (ALS2) ; Chromosome 2q33.1; Recessive

PLS: Dominant, Adult onset
  Chromosome 4p16; Dominant

PLS with Frontotemporal dementia12
  Sporadic
Patient information

Return to Polyneuropathy Index

References
1. Spine 1997;22:486-492
2. J Neurol Sci 1999;170:5-10, JNNP 2001;71:615-620
3. J Neurol 1999;246:1069-1074
4. Clin Neurol Neurosurg 2000;102:109-112
5. JNNP 2000;69:257-261
6. J Neurol 2000;247:654-655
7. Acta Neurol Scand 1997;96:14-21
8. Neurology 1999;53:852-855, PNAS 2001;98:6945-6950
9. Nature Genetics 2001;29:160-165, Neuropediatrics 1995;26:313-319, Neurology 2009; Online January
10. Acta Neurol Scand 2003;107:215220, Muscle Nerve 2008 Feb 20
11. J Neurol Sci 1994;107:153-161, Acta Neurol Scand 1992;85-397-400
12. Neurology 2007;69:1800-1801
13. Neurologist 2009; 15:156-160
14. Neurology 2009;72:19481952, Muscle Nerve 2012; Online August
15. Neurology 2009;73:16861692
16. Neuromusc Disord 2012;22:394-400
17. J Child Neurol 2011;26:1542-1547

10/22/2013