Nerve diseases

Diseases, , ,

Please start with the section on neuromuscular disease patterns for an introduction. Once you determined that the patient likely has a nerve disease you will need to consider which type to narrow down the differential diagnosis. As described in the previous section determining which of the main patterns cranial neuropathy, mononeuropathy, radiculopathy, polyneuropathy, mononeuropathy multiplex, polyradiculopathy, or autonomic neuropathy will help shorten the differential diagnosis tremendously. In this section we’ll start with some lists for cranial neuropathies and then move on to usefull lists and tables for the rest.
 

Cranial neuropathies:

Here are some lists to help with the cranial neuropathies. Also see the section on vertigo for cranial nerve VIII diseases.

Causes of Oculomotor nerve palsy (CN III): think of anatomy

Nuclear & fasciular:

  • Tumours: Glioma
  • Part of a brainstem stroke syndrome

Basilar area:

  • Meningitis:
    • Bacterial, Meningovascular syphilis
    • TB meningitis
    • Fungal meningitis
  • Basilar aneurysms
  • Posterior communicating artery PCOM aneurysm
  • Temporal lobe herniation (uncal herniation)

Cavernous sinus area:

  • Tumours: Intrasellar & extrasellar tumours e.g. pituitary, chordoma, meningioma, Nasopharymgeal tumours, craniopharygioma
  • Internal Carotid artery aneurysms
  • Cavernous sinus thrombosis
  • Mucormycosis

Superior orbital fissure & Orbital apex area:

  • Tumours: nasopharygeal, meningioma, hemangioma, glioma, sarcoma, Hand-Schuller-Christian disease, metastasis
  • AVMs
  • Tolosa-Hunt syndrome
  • Pseudotumour of the orbit

Others:

  • Idiopathic
  • Vascular:
    • Vasculopathy: diabetes mellitus, hypertension & atherosclerosis, giant cell arteritis
  • Wegner’s granulomatosus
  • Hodgkin’s disease, VZV, encephalitis, collagen vascular disease, Paget’s disease
  • Trauma

Causes of trochlear nerve palsy (CN IV):

Nuclear & fasciular:

  • Tumours: glioma, medulloblastoma
  • Part of a Brainstem stroke syndrome

Basilar area:

  • Meningitis:
    • Bacterial, Meningovascular syphilis
    • TB meningitis
    • Fungal meningitis
  • Basilar artery aneurysm

Cavernous sinus area:

  • Internal Carotid artery aneurysm
  • Cavernous sinus thrombosis

Superior orbital fissure & Orbital apex area:

  • Tumours: nasopharygeal, meningioma, hemangioma, glioma, sarcoma, Hand-Schuller-Christian disease, metastasis
  • AVMs
  • Tolosa-Hunt syndrome
  • Pseudotumour of the orbit

Others:

  • Idiopathic
  • Vasculopathy:
    • Atheroma, Hypertension, Diabetes mellitus
    • Giant cell arteritis
  • Trauma

Causes of abducens nerve palsy (CN VI):

Nuclear & fasciular:

  • Tumours: glioma
  • Part of a Brainstem stroke syndrome
  • Multiple sclerosis

Basilar area:

  • Meningitis:
    • Bacterial, Meningovascular syphilis
    • TB meningitis
    • Fungal meningitis
  • Basilar artery aneurysm

Petrous tip area:

  • Raised intracranial pressure ‘false localising sign’
  • Hydrocephalus
  • Mastoiditis
  • Nasophareygeal tumours, paranasal sinus tumours
  • Lateral sinus thrombosis

Cavernous sinus area:

  • Internal Carotid artery aneurysm
  • Cavernous sinus thrombosis
  • Tumours: Intrasellar & extrasellar tumours e.g. pituitary, chordoma, meningioma, Nasopharymgeal tumours, craniopharygioma

Superior orbital fissure & Orbital apex area:

  • Tumours: nasopharygeal, meningioma, hemangioma, glioma, sarcoma, Hand-Schuller-Christian disease, metastasis
  • AVMs
  • Tolosa-Hunt syndrome
  • Pseudotumour of the orbit

Others:

  • Idiopathic
  • Vasculopathy:
    • Atheroma, Hypertension, Diabetes mellitus
    • Giant cell arteritis
  • Wegner’s granulomatosus

Causes of isolated facial nerve palsy (CN VII):

Upper motor neuron lesion:

  • Stroke, most common
  • Vasculitis
  • Syphilis
  • HIV

Lower motor neuron lesion:

  • Bell’s palsy a.k.a. Idiopathic (but HSV-1 is implicated), most common
  • VZV a.k.a. Herpes zoster, Ramsay Hunt Syndrome
  • Otitis media
  • Cholesteatoma
  • Tumours:
    • Cerebellopontine angle, acoustic or facial neuroma
    • Glomus tumour
    • Parotid tumour
  • Temporal bone fracture
  • Diabetes mellitus
  • Lyme disease
  • Sarcoidosis
  • Amyloidosis
  • AIDS
  • Sjogren’s syndrome
  • Lesions of the facial nucleus (usually affects other nerves as well)

Recurrent or bilateral lower motor neuron facial palsy:

  • Base of the skull tumour e.g. Lymphoma
  • Lyme disease
  • Sarcoidosis
  • Gullian-Barre syndrome
  • If immunocompromised, VZV

DDx. of bilateral facial palsy (compare with causes of bilateral facial palsy above):

  • Myasthenia gravis

Causes of cavernous sinus syndrome:

  • Sepsis
  • Tumour
  • Carotid artery aneurysms
  • Wegener’s granulomatosis

Causes of skull base syndromes including jugular foramen syndrome:

Intracranial:

  • Neoplastic:
    • Extension of cerebellopontine angle tumour
    • Meningioma
    • Cholesteotoma
    • Neurofibroma
  • Guillain-Barre syndrome & variants
  • Chronic tuburculosis
  • Syphilis
  • Diabetes mellitus

Skull:

  • Fractured base of the skull
  • Paget’s disease

Extracranial:

  • Neoplastic:
    • Lymphoma
    • Carotid body tumour
    • Glomus jugulare turmour
    • Nasopharyngeal carcinoma
    • Metastatic Squamous cell carcionoma, others
  • Jugular vein thrombosis
  • Carotid dissection

 

 
 

Nerve diseases etiology by onset and involved modality:

Motor Sensorimotor Sensory
Acute -GBS Patients with sensory
symptoms tend not to present acutely
-Porphyria
Subacute/chronic -CIDP motor forms -CIDP -Diabetic polyneuropathy
-Multifocal motor neuropathy MMN -Lewis-Somner syndrome
LSS/MADSAM (asymmetric CIDP)		 -HIV associated distal
polyneuropathy
-Coeliac disease a.k.a. gluten neuropathy (usually sensorimotor) -Vasculitic neuropathy -Migrant sensory neuritis a.k.a. Wartenberg Syndrome
-Paraproteinemic neuropathy -Sjogren syndrome
-Amyloid neuropathy -Idiopathic sensory ganglionopathy a.k.a. chronic ataxic neuropathy
-Mulfocal diabetic
neuropathy a.k.a.		 -Drugs: antiretrovirals,
-Diabetic amyotrophy
-Lyme disease
-Coeliac disease a.k.a. gluten
neuropathy
-Sjogren syndrome (usually sensory)
-Arsenic poisoning
-HMSN a.k.a. Charcot-Marie Tooth
disease

 

Causes of peripheral neuropathy/peripheral nerve disease & some radiculopathies (the full list):

Idiopathic inflammatory neuropathy:

  • Acute idiopathic polyneuropathy
  • Chronic inflammatory demyelinating polyneuropathy CIDP

Infective & granulomatous:

  • AIDS
  • Leprosy
  • Diphtheria
  • Sepsis & multiorgan failure
  • Sarcoidosis

Vasculitic:

  • Systemic necrotizing vasculitis:
  • Polyarteritis nodosa
  • Allegic angiitis & granulomatosis a.k.a. Churg Strauss syndrome
  • Wegener’s granulomatosis
  • Giant cell arteritis
  • Collagen vascular disease:
  • Rheumatoid arthritis
  • Systemic lupus erythematosus
  • Sjogren’s syndrome
  • Progressive systemic sclerosis
  • Mixed connective tissue disease

Endocrine:

  • Diabetes mellitus
  • Hypothyroidism
  • Acromegaly

Metabolic & nutritional:

  • Uremia
  • Liver disease: Primary biliary cirrhosis & chronic liver disease
  • Vitamin B12 deficiency
  • Porphyria

Drug induced & toxic:

  • Alcohol
  • Other drugs
  • Heavy metals
  • Organophosphates & hexacarbons

Neoplastic & paraproteinemic neuropathies:

  • Compression/infiltration
  • Paraneoplastic syndromes
  • Paraproteinemias
  • Amyloidosis

Hereditary:

  • Hereditary motor & sensory neuropathies:
  • Charcot-Marie-Tooth CMT
  • Dejerine-Sottas disease
  • Hereditary sensory & autonomic neuropathies HSAN types I-IV
  • Friedreich’s ataxia
  • Familial Amyloidosis
  • Metabolic:
    • Porphyria
    • Some Leukodystrophies:
    • Metachromatic leukodystrophy a.k.a. arylsufatase A deficiency
    • Krabbe’s disease a.k.a. globoid cell leukodystrophy a.k.a. galactocerebroside b-galactosidase deficiency
    • Abetalipoproteinemia
    • Tangier disease
    • Refsum’s disease
    • Fabry’s disease

Entrapment neuropathies:

  • Carpel tunnel syndrome (see below)

 

Causes of polyneuropathy:

Axonal:

  •  Acute:
    • Porphyria
    • Toxins
    • Axonal form of Guillain Barre syndrome
  •  Subacute:
    • Metabolic & Toxic
  •  Chronic:
    • Metabolic & Toxic
    • Hereditary
    • Diabetic
    • Dysproteinamia

Demyelinating:

  • Acute:
    • Guillain Barre syndrome
  •  Subacute:
    •  CIDP
  •  Chronic:
    • Hereditary
    • Inflammatory, autoimmune
    • Dysproteinemia
    • Metabolic, toxic

Types of mononeuropathy:

  • Median nerve compression a.k.a. median neuropathy:
    • Carpal tunnel syndrome (median neuropathy at the wrist)
    • Entrapment at ligament of Struthers, very rare
    • Pronator syndromeAnterior interossesous nerve AIN syndrome (a.k.a. Kiloh-Nevin syndrome)
  • Ulnar nerve and ulnar neuropathy:
    • Ulnar nerve compression at the wrist
    • Ulnar nerve compression at the Elbow
  • Radial nerve and radial neuropathy:
    • Radial nerve compression at the axilla
    • Radial nerve compression at the spiral groove
    • Posterior interosseous nerve neuropathy (PIN)
    • Superficial radial nerve neuropathy
  • Long thoracic nerve a.k.a. Thoracic nerve of Bell
  • Obturator nerve palsy a.k.a. obturator neuropathyFemoral nerve palsy a.k.a. femoral neuropathy
  • Sciatic nerve a.k.a. sciatic neuropathy
  • Peroneal nerve a.k.a. peroneal neuropathy
  • Tibial nerve neuropathy, rare
  • Tarsal tunnel syndrome (posterior tibial nerve compression in tarsal tunnel)
  • Miscellaneous syndromes:
    • Suprascapular nerve: Spinoglenoid notch
    • Lateral fermoral cutaneous nerve (Meralgia paresthetica; at the Inguinal ligament)
    • Obturator nerve at the Obturator canal
    • Plantar branches of the posterior tibial nerve (Morton metatarsalgia, plantar fasica and heads of 3rd and 4th metatarsals)

Causes of Mononeuropathy multiplex a.k.a. mononeuritis multiplex:

Vasculitis: (axonal), common

  • Rheumatoid arthritis (axonal)
  • SLE, systemic sclerosis
  • Polyarteritis nodosa
  • Wegener’s granulomatosis
  • Cryoglobulinemia (rare)

Infections:

  • HIV(axonal)
  • Lyme disease
  • Mycobacterium leprae (Leprosy)

Demyelination:

  • Mutifocal form of CIDP (demyelinating)
  • Multifocal motor neuropathy MMN (demyelinating)

Others:

  • Diabetes mellitus, common
  • Sarcoidosis, rarely
  • Amyloidosis
  • Hypereosinophilia syndrome
  • Sickle cell disease
  • Subacute asymmetric idiopathic polyneuropathy
  • Migrant sensory neuritis a.k.a. Wartenberg’s disease (pure sensory, axonal)

 

Causes of mononeuritis multiplex with lymphocytic meningitis:

  • Lyme Neuroborreliosis
  • Neurosarcoidosis
  • Zoster sine herpete
  • Uveo-meningeal syndromes

Causes of autonomic neuropathy:

Central:

  • Parkinson disease
  • Primary selective autonomic failure ?a.k.a. Shy Dragger syndrome
  • ?Wernicke encephalopathy
  • Syringomyelia/syringobulia

Peripheral:

  • Guillain-Barre syndrome
  • Diabetes mellitus
  • Infections/related:
  • Toxic/nutritional:
    • Alcoholic neuropathy
  • Drugs: amiodarone, cisplatin, vincristine, paclitaxel
  • Paraneoplastic:
    • Lambert-Eaton myasthenic syndrome
  • Familial autonomic failure a.k.a. Riley-Day syndrome
  • Familial amyloidosis
  • Hereditary neuropathies:
    • Hereditary sensory and autonomic neuropathies HSAN
    • Fabry disease
    • Triple A syndrome a.k.a. allgrove syndrome
    • Navajo Indian neuropathy
    • Tangier disease
    • MEN type 2b

 

Causes of Motor Neuropathy (& motor neuron disease)

Acute:

  •  Poliomyelitis
  • GBS
  • Porphyria

Chronic:

  • MND
  • CIDP motor forms
  • Multifocal motor neuropathy MMN
  • Lewis Somner syndrome LSS
  • Lead toxicity

 

DDx. Of amyotrophic lateral sclerosis:

  • Myathemia gravis
  • Cervical spondylosis
  • Polyradiculopathy with myelopathy
  • Paraneoplastic motor neuropathy
  • Syringobulbia
  • Post polio syndrome
  • Hexaminidase A deficiency
  • Lead intoxication

 

Causes of Axonal Neuropathy:

Immune:

  • Guillain-Barre syndrome GBS axonal forms:
    • Acute motor axonal neuropathy AMAN
    • Acute motor sensory axonal neuropathy AMSAN
    • Chronic idiopathic axonal polyneuropathy CIAP
  • Connective tissue diseases:
    • Sjogren’s syndrome, systemic lupus erythematosus, rheumatoid arthritis

Medications:

  • Vincristine, cisplatinum, organophosphate
  • Metronidazole, dapsone

Toxins:

  • Alcohol, pyridoxine toxicity

Infectious:

  • Leprosy
  • Borrelia burgdorferi
  • HIV, HTLV1

Nutritional:

  • B1 deficiency, folic acid deficiency, vitamin E deficiency

Endocrine:

  • Diabetes mellitus, Hypothyroidism

Paraproteinemia:

  • Waldenstorm’s disease, Benign monoclonal gammopathy
  • Multiple myeloma

Hereditary neuropathy:

  • CMT 2 and CMT X
  • Familial amyloid neuropathies

Others:

  • Chronic renal failure
  • Paraneoplastic neuropathy: lung cancer, ovarian carcinoma

Causes of sensory neuropathy (most are chronic because of the nature of sensory symptoms):

  •  CIDP
  • Sarcoidosis
  • Hereditary sensory neuropathy
  • Chronic renal failure
  • Diabetes mellitus (predominantly sensory)
  • Thiamine B1 defeciency (predominantly sensory)
  • Leprosy

 

Causes of Chronic inflammatory demyelinating polyneuropathy CIDP:

CIDP alone:

  • CIDP, idiopathic

CIDP with an associated condition:

  • CIDP with paraproteinemia, includes:
    MGUS, Waldenstrom’s macroglobulinemia, multiple myeloma, plasmacytoma, POEMS syndrome, B cell lymphoma, CLL,
  • CIDP with SLE, inflammatory bowel disease, sarcoidosis, chronic active hepatitis,
  • CIDP with HIV
  • CIDP with hepatitis B, hepatitis C
  • CIDP with Diabetes mellitus, thyrotoxicosis
  • CIDP with hereditary demyelinating neuropathies
  • CIDP with bone marrow & organ transplantation

 

Causes of small fibre neuropathy:

  • Chronic idiopathic small fibre sensory neuropathy
  • Sjogren’s syndrome
  • Amyloidosis
  • Diabetes mellitus
  • Hereditary sensory and autonomic neuropathies
  • Fabry’s disease
  • Tangier disease

 

Causes of neuromyopathy:

  • Vasculitis
  • Paraneoplastic
  • Drugs:
    • Chloroquine & Hydroxychloroquine
    • Amiodarone, Colchicine
    • Vincristine, Perhexiline

 

Causes of palpable nerves (thickened nerves/enlarged nerves/hypertrophied nerves):

  • Amyloidosis
  • Acromegaly
  • Leprosy
  • Sarcoidosis
  • Neurofibromatosis
  • Some Hereditary neuropathies
  • Refsum’s disease
  • CMT
  • CIDP

 
 

IgM gammopathy associated peripheral neuropathy:

  • Cryoglobulinemia (type 1 & 2)
  • Macroglobulinemia
  • CLL

 

IgA or IgG gammopathy associated peripheral neuropathy:

  • Multiple myeloma
  • POEMS
  • Primary amyloidosis
  • Chronic inflammation

 

Causes of head drop/neck drop:

  • Myasthenia gravis
  • Polymyositis
  • Dermatomyositis
  • Motor neuron disease