The more specific findings of intradural flow voids on T2-weighted images, and/or serpentine enhancement on MRA and T1 images, are seen in 85 to 90 percent of patients.
From: Uptodate
From: Uptodate
Friday, March 16, 2012
Sensory Neuronopathies
http://neuromuscular.wustl.edu/antibody/sneuron.html
Causes of Sensory Neuronopathies
NB: CANVAS = Cerebellar ataxia, Neuropathy, Vestibular areflexia syndrome
Causes of Sensory Neuronopathies
- Immune mediated - paraneoplastic (anti Hu), Sjogren's, acute sensory neuronopathy
- Idiopathic - pan-sensory, small fiber, CANVAS
- Drugs - Pyridoxine, cisplatin, doxorubicin
- Hereditary
- Localized - zoster
NB: CANVAS = Cerebellar ataxia, Neuropathy, Vestibular areflexia syndrome
Hereditary Motor Syndromes with Multisystem Involvement
http://neuromuscular.wustl.edu/synmot.html
Hexosaminidase A (Late onset GM2 Gangliosidosis - Tay Sach's disease)
Motor neuropathy +/- CNS (LMN dominant)
Cramps, paresthesias at onset
Symmetric proximal > distal weakness
Fasciculations
+/- CNS involvement - spasticity, ataxia, dementia, dystonia
Adult onset Polyglucosan body disease
CNS + Neuropathy
Dementia in 2/3rd
Cord - spasticity
Nerve - Motor and sensory
Hexosaminidase A (Late onset GM2 Gangliosidosis - Tay Sach's disease)
Motor neuropathy +/- CNS (LMN dominant)
Cramps, paresthesias at onset
Symmetric proximal > distal weakness
Fasciculations
+/- CNS involvement - spasticity, ataxia, dementia, dystonia
Adult onset Polyglucosan body disease
CNS + Neuropathy
Dementia in 2/3rd
Cord - spasticity
Nerve - Motor and sensory
Another cause for myeloneuropathy
Copper deficiency can cause myeloneuropathy. Zinc excess or malabsorption syndrome are the common cause for same. Denture creams containing excess zinc can cause copper deficiency.
In fact zinc is used in Wilson's disease because of its ability to induce metallothionein, which binds copper and reduces its absorption.
Clioquinol, the cause for SMON (myeloneuropathy with optic nerve involvement), is an avid chelator. It has been used in acrodermatitis enteropathica, a childhood disease due to a hereditary defect in zinc transport. Clioquinol binds zinc and improves the transport into the circulation.
From:
Schaumburg H. Copper deficiency myeloneuropathy: A clue to clioquinol-induced subacute myelo-optic neuropathy? Neurology 2008; Vol 71, Issue 9
In fact zinc is used in Wilson's disease because of its ability to induce metallothionein, which binds copper and reduces its absorption.
Clioquinol, the cause for SMON (myeloneuropathy with optic nerve involvement), is an avid chelator. It has been used in acrodermatitis enteropathica, a childhood disease due to a hereditary defect in zinc transport. Clioquinol binds zinc and improves the transport into the circulation.
From:
Schaumburg H. Copper deficiency myeloneuropathy: A clue to clioquinol-induced subacute myelo-optic neuropathy? Neurology 2008; Vol 71, Issue 9
Dermatomyositis and polymyositis
DM - Humoral immunity directed against vessels, children + adults, CK can be normal
PM - Cytotoxic T cell mediated immune activation against muscle membrane, mostly adults, abnormal CK
Bradley 5th Ed 2008
PM - Cytotoxic T cell mediated immune activation against muscle membrane, mostly adults, abnormal CK
Bradley 5th Ed 2008
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