Tag Archives: Sensory deficit

Chronic Idiopathic Axonal Polyneuropathy CIAP

Diagnosis: Diagnosis by exclusion. Clinical features: Chronic, sensory or sensorimotor polyneuropathy Findings on investigations: NCS/EMG: Axonal polyneuropathy No evidence of demyelination Related articles: Approach to weakness, approach to sensory deficits, neuromuscular disease patterns, polyneuropathy,

Idiopathic Sensory Ganglionopathy

Synonyms: a.k.a. chronic ataxic neuropathy Diagnosis: Clinical features +NCS/EMG findings +Exclusion of other causes: Sjogren syndrome, paraneoplastic syndrome, paraproteinemia, Refsum disease etc. Clinical features: Global sensory loss (especially proprioception), sensory ataxia (pseudoathetosis), normal power Exclusion of other causes: Sjogren syndrome, paraneoplastic syndrome, paraproteinemia, Refsum disease etc. Findings on investigations: +NCS +EMG: Axonal Sensory neuropathy (may … Continue reading Idiopathic Sensory Ganglionopathy →

Hereditary sensory neuropathy HSN (Hereditary sensory autonomic polyneuropathy HSAN)

Synonyms: Hereditary sensory neuropathy HSN or Hereditary sensory autonomic polyneuropathy HSAN Diagnosis: Clinical +genetic testing Clinical features: All have sensory neuropathy without motor features HSN1 a.k.a. Denny-Brown neuropathy: Clinical features: Loss of pain and temperature sensation, ulceration of feet and hands, Charcot joints. No autonomic features Findings on investigations: NCS/EMG: axonal HSN2 a.k.a. congenital sensory … Continue reading Hereditary sensory neuropathy HSN (Hereditary sensory autonomic polyneuropathy HSAN) →

Fabry Disease: alpha-galactosidase A deficiency

Synonyms: a.k.a. alpha-galactosidase A deficiency a.k.a. Angiokeratoma corporis diffusum Clinical features: Rash: Telangiectasia on lower trunk and upper legs Peripheral neuropathy: painful Stroke Cardiac and renal dysfunction Genetics: X-linked recessive Findings on investigations: Leukocyte alpha-galactosidase A: low CT: pulvinar hyperdensity (mineralization) MRI brain: Pulvinar sign T1 hyperintensity Other tests: Basic metabolic panel: creatinine: renal failure … Continue reading Fabry Disease: alpha-galactosidase A deficiency →

Tangier disease: Familial alpha-lipoprotein deficiency

Synonyms: High density lipoprotein HDL deficiency, Clinical features: Enlarged orange tonsils Impaired pain and temperature sensation Findings on investigations: Lipid profile: low to absent HDL Related articles: Approach to weakness, approach to sensory deficits, neuromuscular disease patterns, polyneuropathy, Small fibre sensory neuropathy,

Sjogren Syndrome Neuropathy

Diagnosis: Clinical features of neuropathy plus diagnosis of Sjogren syndrome (by serology or biopsy) plus exclusion of other causes. Vasculitis may occur. Clinical features: Features of neuropathy: small fibre neuropathy, or sensory axonal polyneuropathy, facial palsy CN VII may occur, Findings on Investigations: NCS/EMG: Features of the underlying pattern: small fibre sensory neuropathy, or sensory … Continue reading Sjogren Syndrome Neuropathy →

Coeliac Disease Neuropathy

Synonyms: a.k.a. gluten neuropathy Diagnosis: Diagnosis of Coeliac disease or serology positive for coeliac disease, plus clinical or electrodiagnostic neuropathy and exclusion of other causes Clinical features: Different patterns: sensorimotor neuropathy >mononeuropathy multiplex >motor neuropathy >small fibre neuropathy. Findings on investigations: +NCS/EMG: Axonal neuropathy pattern, different patterns (sensorimotor neuropathy >mononeuropathy multiplex >motor neuropathy >small fibre … Continue reading Coeliac Disease Neuropathy →

Idiopathic Sensory Perineuritis

Diagnosis: Clinical features of polyneuropathy, mononeuropathy multiplex, biopsy findings of perineuritis and exclusion of secondary causes Clinical features: sensory patchy neuropathy, sensorimotor forms exist. Pathology, nerve biopsy: Perineurium: inflammatory lymphocytic infiltrate, thickening of perineurium Exclusion of other causes Treatment: Consider corticosteroids Related articles: Approach to weakness, approach to sensory deficits, neuromuscular disease patterns, polyneuropathy, Diabetic … Continue reading Idiopathic Sensory Perineuritis →

Migrant sensory neuritis (Wartenberg Syndrome)

Synonyms: a.k.a. Wartenberg Syndrome: Clinical features: decreased sensation in individual cutaneous nerves. i.e. sensory mononeuropathy multiplex. Precipitated by stretching the limb and preceded by pain. Normal motor Findings on investigations: +NCS: axonal pattern in involved nerves Pathology, nerve biopsy: Endoneurium: increased connective tissue, fascicular Wallerian degeneration or inflammatory infiltrate Related articles: Approach to weakness, approach … Continue reading Migrant sensory neuritis (Wartenberg Syndrome) →

Nonhereditary Amyloid Neuropathy

Diagnosis: Appropriate clinical features plus evidence of amyloid on biopsy Clinical features: Small fibre neuropathy +/-autonomic failure (see under small fibre neuropathy) Findings on investigations: NCS/EMG: Normal Or features of carpel tunnel syndrome Pathology, sural nerve biopsy: Eosinophilic deposits in the endoneurium and vessel walls Congo red: apple green birefringence Sirius red: stains amyloid red … Continue reading Nonhereditary Amyloid Neuropathy →

Mycobacterium leprae Neuropathy

Synonyms: a.k.a. Leprosy a.k.a. Hansen’s disease a.k.a. leprous polyneuritis Diagnosis: Clinical features of neuropathy plus evidence of Mycobacterium leprae infection Clinical features: Skin changes (hyperpigmentation, papule) +loss of touch and pain (anesthesia) Spared reflexes Or multiple compression neuropathies (may mimic mononeuritis multiplex) in tuberculoid leprosy Thickened nerves Pathology, nerve biopsy: Acid fast bacilli in perineurium … Continue reading Mycobacterium leprae Neuropathy →

HIV Neuropathy

Synonyms: a.k.a. HIV associated polyneuropathy: Diagnosis: HIV infection with clinical features of neuropathy (many forms) with exclusion of other causese Many forms: CIDP, GBS, mononeuritis multiplex, sensory ataxic neuropathy, lumbosacral plexopathy, polyneuropathy Clinical features: Depends on the underlying subtype (see above) Findings on investigations: NCS/EMG, same as respective idiopathic forms Note: Guillain-Barre Syndrome occurs at … Continue reading HIV Neuropathy →

Heavy Metal Neuropathy and Solvent Neuropathy

Diagnosis: Neuropathy +documented high heavy metal level +improvement of symptoms/signs/NCS/EMG after withdrawal of agent Clinical features: Clinical features of neuropathy: may be motor neuropathy, sensori-motor polyneuropathy Findings on investigations: NCS/EMG: Axonal pattern Specific causes: Lead neuropathy (see lead poisoning), Mercury (see mercury poisoning), Others, arsenic, thallium, gold, N-Hexane inhalation Related articles: Approach to weakness, approach … Continue reading Heavy Metal Neuropathy and Solvent Neuropathy →

Alcohol Neuropathy

It is unclear if alcoholism without associated nutritional deficiency can cause neuropathy Clinical features: Painful sensory polyneuropathy. Pain on light touch a.k.a. allodynia. Loss of ankle jerks Improvement with withdrawal of alcohol and B1 supplementation Related articles: Approach to weakness, approach to sensory deficits, neuromuscular disease patterns, polyneuropathy, Nutritional neuropathy,

Chronic Renal Failure Neuropathy

Synonyms: a.k.a. uremic neuropathy Diagnosis: Clinical or electrodiagnostic features of neuropathy plus chronic renal failure and exclusion of other cause Clinical features: Distal sensorimotor neuropathy +chronic renal failure +resolves with dialysis or treatment of chronic renal failure Findings on investigations: EMG/NCS: Distal Sensorimotor neuropathy In some cases carpel tunnel syndrome Pathology/Nerve biopsy: Axonal loss Related … Continue reading Chronic Renal Failure Neuropathy →

Hypophosphatemia Neuropathy

Diagnosis: Clinical features of neuropathy plus phosphate deficiency and exclusion of other causes Clinical features: Acute, Sensorimotor, areflexia, dysarthria Phostphate levels: <2.4 mg/dl Findings on investigations: NCS/EMG: Demyelinating pattern (Prolonged distal latencies, reduced conduction velocities) [case report] In some cases axonal pattern [case reports] F-waves absent Decreased recruitment (neurogenic recruitment) Pathology/Nerve biopsy: Sub-perineural edema, mild … Continue reading Hypophosphatemia Neuropathy →

Nutritional Deficiency Neuropathy

Synonyms: a.k.a. nutritional neuropathy Diagnosis: The diagnosis is based on clinical features of neuropathy supported by electrodiagosis and confirmation of nutritional deficiency with exclusion of other causes +improvement of symptoms/signs/NCS/EMG after supplementation Clinical features: Usually features of polyneuropathy. Most commonly sensori-motor neuropathy +documented deficiency Findings on investigations: NCS/EMG: Usually Axonal pattern Pathology, nerve biopsy: Perineuritis … Continue reading Nutritional Deficiency Neuropathy →

Charcot-Marie-Tooth disease CMT

Synonyms: Hereditary Motor and Sensory Neuropathy HMSN Now the whole group is called Charcot-Marie-Tooth disease CMT Diagnosis: Clinical +electrodiagnostics (EMG/NCS) +Genetic testing: Clinical features: Abnormal LMN features: weakness Atrophy: champagne-glass legs, stork legs Deformity: claw toe, Friedreich’s foot, kyphoscoliosis Sensory features: loss of vibration and later on loss of proprioception Palpable nerves Genetics: CMT1 a.k.a. … Continue reading Charcot-Marie-Tooth disease CMT →

Acromegaly Neuropathy

Diagnosis: Clinical features of neuropathy plus diagnosis of acromegaly and exclusion of other causes Clinical features: Carpal tunnel syndrome, tarsal tunnel syndrome Polyneuropathy Findings on investigations: NCS/EMG: Carpal tunnel syndrome, tarsal tunnel syndrome Polyneuropathy Pathology, Nerve biopsy: Initially: demyelination Onion bulb formation in end stage Related articles: Approach to weakness, neuromuscular disease patterns, polyneuropathy,

Cranial Neuropathies

Here are some lists to help with the cranial neuropathies. Please see the underlying sections. Also see the section on vertigo for cranial nerve VIII diseases. Synonyms: Cranial neuropathy 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 … Continue reading Cranial Neuropathies →

Diabetic Neuropathy

There are various types of diabetic neuropathy Diabetic polyneuropathy (sensory type and sensorimotor type): Diagnosis: Features of polyneuropathy and meeting criteria for diabetes mellitus Clinical features: Symmetric sensory polyneuropathy, loss of vibration, pain, touch and temperature sensation and in some cases proprioception loss and Charcot joints Areflexia Painful May become sensorimotor Pathology, nerve biopsy: Nonspecific … Continue reading Diabetic Neuropathy →

Paraneoplastic Sensory Neuropathy

Diagnosis: The diagnosis is made based on clinical features of neuropathy supported by electrodiagnostic testing and isolation of the underlying neoplasm Clinical features: Painful from: spontaneous pain and mechanical hyperalgesia i.e. painful sensation provoked or exacerbated by pinprick sensation a.k.a. pinprick hyperalgesia or gentle tactile stimulation a.k.a. allodynia Ataxic form: Romberg positive, impaired vibration and … Continue reading Paraneoplastic Sensory Neuropathy →

Paraneoplastic Vasculitic Neuropathy PVN

Synonyms: or paraneoplastic neuromyopathy Diagnosis: A subtype of nonsystemic vasculitic neuropathy, requires features of neuropathy on clinical exam or electrodiagnostic studies plus evidence of vasculitis and underlying neoplastic disorder Clinical features: Weakness +/-sensory abnornalities, often with features of mononeuropathy multiplex Findings on investigations: +Nerve conduction studies +EMG: Polyneuropathy or mononeuritis multiplex, axonal neuropathy +/-evidence of … Continue reading Paraneoplastic Vasculitic Neuropathy PVN →

Nonsystemic vasculitic neuropathy NSVN

Diagnosis: This is based on clinical features +nerve/muscle biopsy findings of vasculitis +exclusion of systemic causes Clinical features: Clinical features: usually subacute, painful, distal, asymmetric, Findings on investigations: +Nerve conduction studies +EMG: Axonal neuropathy +CSF analysis: increased protein +ESR: might be increased +ANCA negative Pathology, nerve biopsy: Sural nerve or superficial peroneal nerve biopsy +/-muscle … Continue reading Nonsystemic vasculitic neuropathy NSVN →

Systemic Vasculitic Neuropathy

Diagnosis: The diagnosis is made based on clinical features +Peripheral neuropathy, +evidence of vasculitis, +evidence of other organ involvement Clinical features: acute, mononuritis multiplex or less commonly mononeuritis simplex or symetric polyneuropathy, sensorimotor +Peripheral neuropathy +evidence of vasculitis +evidence of other organ involvement Findings on investigations: +NCS/EMG: NCS: Axonal neuropathy Conduction block: often transient CMAP: … Continue reading Systemic Vasculitic Neuropathy →

Anti-sulfatide Neuropathy

Synonyms: a.k.a. anti-chondroitin sulfate neuropathy: Diagnosis: The diagnosis is made based on clinical features supported by electrodiagnostic tests and antibodies Clinical features: Polyneuropathy (sensory, or sensorimotor, sensory>motor), ataxia may occur, Findings on investigations: Anti-sulfatide antibody (a.k.a. anti-chondroitin sulfate): positive, > 1:1000 titre SPEP +IFE: usually have monoclonal IgM NCS/EMG: Mainly demyelinating pattern with secondary axonal … Continue reading Anti-sulfatide Neuropathy →

Anti-GALOP syndrome: Gait ataxia, autoantibody, late onset polyneuropathy

Synonyms: a.k.a. gait ataxia and polyneuropathy GAPN Diagnosis: Clinical features: Late onset 70 year olds, gait ataxia (wide based with falls) and polyneuropathy (sensorimotor, sensory>motor, impaired proprioception) Anti-GALOP (IgM against central myelin antigen a.k.a. galopin): positive, SPEP +IFE: monoclonal IgM Treatment: Intravenous immunoglobulin IVIG cyclophosphamide Related articles: Approach to weakness, neuromuscular disease patterns, polyneuropathy, Polyneuropathy … Continue reading Anti-GALOP syndrome: Gait ataxia, autoantibody, late onset polyneuropathy →

Lymphoma Associated Neuropathy

Diagnosis: Clinical and electrodiagnostic features supported by isolation of lymphoma Clinical features: Sensorimotor neuropathy, polyradiculopathy in carcinomatous meningitis Findings on investigations: CSF analysis: cytology and flow cytometery in carcinomatous meningitis NCS/EMG: Sensorimotor neuropathy Or polyradiculopathy in carcinomatous meningitis Pathology revealing lymphoma: Cytology, flow cytometery Bone marrow biopsy Lymphnode excitional biopsy Pathology, Nerve biopsy: Axonal neuropathy … Continue reading Lymphoma Associated Neuropathy →

POEMS syndrome: Polyneuropathy Organomegally Endocrinopathy M protein and Skin changes

Diagnosis: A paraneoplastic syndrome to osteoclastic plasmacytoma Pathology: Muscle actin stain; increased vessels in nerves VEGF: elevated serum levels Criteria/Features: Polyneuropathy: Demyelinating sensorimotor polyneuropathy (motor>sensory), usually painless, length dependent Monoclonal gammopathy: Serum protein electrophoresis with immunofixation IFE: M protein, Lambda light chain, immunofixation is necessary to avoid false negatives Osteoclastic plasmacytoma i.e. note lytic Skin … Continue reading POEMS syndrome: Polyneuropathy Organomegally Endocrinopathy M protein and Skin changes →

Polyeuropathy Associated with Paraproteinemia

Diagnosis: This is a category of different neuropathies. Neuropathy due to a gammopathy includes anti-MAG syndrome, POEMS, cryoglobulinemia, IgM MGUS polyneuropathy, Anti-GALOP (IgM against central myelin antigen): Gait Disorder, Autoantibody Late-age Onset Polyneuropathy Evidence of paraproteinemia e.g. SPEP: paraproteinemia Pathology, nerve biopsy: Light microscopy: Nonspecific, loss of myelin and/or axons No inflammation except in cryoglobulinemia … Continue reading Polyeuropathy Associated with Paraproteinemia →

Multifocal Acquired Demyelinating Sensory And Motor neuropathy MADSAM

Synonyms: Lewis Sumner syndrome LSS Diagnosis: Clinical features supported by +NCS/EMG Clinical features: Chronic, asymmetric, distal, sensorimotor, arms >legs, can affect cranial nerves Findings on investigations: NCS/EMG: Multifocal conduction block in affected nerves, Widespread sensory abnormalities: helps distinguish from MMN, Asymmetric, helps distinguish it from CIDP Serum anti-GM1 antibody: negative, helps distinguish from MMN CSF: … Continue reading Multifocal Acquired Demyelinating Sensory And Motor neuropathy MADSAM →

Subacute Inflammatory Demyelinating Polyneuropathy

Synonyms: a.k.a. subacute IDP Diagnosis: Clinical +NCS/EMG Similar to Guillain Barre Syndrome GBS and Chronic inflammatory demyelinating polyradiculopathy CIDP but lasting 4-8 weeks. Related articles: Approach to weakness, neuromuscular disease patterns, polyradiculopathy, Guillain Barre syndrome GBS, Chronic inflammatory demyelinating polyradiculopathy CIDP,

Radiation Induced Radiculopathy

Synonyms: Formerly radiation induced lower motor neuron syndrome: Diagnosis: Clinical exposure to radiation in lower spinal cord and weakness +/-minor sensory involvement MRI +GAD: enhancement of nerve roots Related articles: Polyradiculopathy,

Idiopathic Lumbosacral Plexitis

Synonyms: Idiopathic Lumbosacral plexitis a.k.a. lumbosacral plexitis a.k.a. idiopathic neuralgic amyotrophy a.k.a. Lumbosacral plexopathy Diagnosis: The diagnosis is based on clinical features, electrophysiological findings and exclusion of competing causes. Clinical features: Lumbosacral distribution of pain followed by weakness See lumbosacral plexopathy Findings on investigations: +EMG Localises the lesion to the lumbar/sacral plexus Localises the lesion … Continue reading Idiopathic Lumbosacral Plexitis →

Radiation Induced Plexopathies (Radiation Plexopathy)

Diagnosis: The diagnosis is made based on findings of plexopathy clinically and on imaging supported by exclusion of other causes. Clinical features: Exposure to radiation, exclusion of other causes Weakness, usually painless Clinical fasciculations and myokymia may occur Findings on investigations: NCS/EMG: Fasciculations and myokymia may occur SNAP and CMAP: slowed conduction velocity is common … Continue reading Radiation Induced Plexopathies (Radiation Plexopathy) →

Heredofamilial Brachial Plexopathy

Synonyms: a.k.a. hereditary brachial plexus neuropathy a.k.a. hereditary neuralgic amyotrophy HNA, rare: Clinical features: Usually Painless Attacks of weakness with recovery over weeks +some cranial involvement Genetics: Mapped to chr. 17q24-25 Autosomal dominant Findings on investigations: NCS/EMG: may be normal between attacks Pathology, nerve biopsy: Semithin plastic sections: Tomacula (sausage like excess myelin) Teased fibres: … Continue reading Heredofamilial Brachial Plexopathy →

Carcinomatous Plexopathy

Diagnosis: The diagnosis is confirmed by isolation of the causative neoplasm and electrophysiological and clinical findings Clinical features: Painful, Features of brachial plexopathy or those of lumbosacral plexopathy: Weakness in lower plexus C8, T1 distribution +atrophy Associated with Horner’s syndrome Asymmetric leg weakness, reduced reflexes Findings on investigations: NCS/EMG: Denervation (fibrillations and positive sharp waves … Continue reading Carcinomatous Plexopathy →

Acute Brachial Plexus Neuritis

Synonyms: a.k.a. Parsonage-Turner syndrome formerly brachial neuralgic amyotrophy Diagnosis: This is a clinical diagnosis supported by electrophysiological findings and consistent imaging Clinical features: Brachial distribution of pain followed in 3-10 days by weakness and atrophy with areflexia In some cases sensory loss may occur May be bilateral Findings on investigations: +EMG: Localised to brachial plexus, … Continue reading Acute Brachial Plexus Neuritis →

Neurogenic Thoracic Outlet Syndrome

This is a type of cervical rib syndrome. There is a related vascular thoracic outlet syndrome. Diagnosis: The diagnosis is made by clinical and electrophysiological (NCS/EMG) and then imaging to identify the underlying case. Clinical features: Pain: C8 andT1 Sensory loss C8 and T1 Weakness: abductor pollicis brevis Puling on the arm (down) reproduces sensory … Continue reading Neurogenic Thoracic Outlet Syndrome →

Autonomic Neuropathy

Synonyms: Autonomic neuropathy/autonomic failure/autonomic dysfunction Diagnosis: Autonomic dysfunction may be central (CNS) or peripheral (PNS) Clinical features: Helps define distribution and extent of disease: Sympathetic, parasympathetic Adrenergic, cholinergic Helps define presents of associated CNS disease Helps define associated systemic disease Autonomic function testing: Baroreceptor function testing a.k.a. Cardiovagal testing by HR variability: Beat to beat … Continue reading Autonomic Neuropathy →

Radiculopathy

Diagnosis: This is a clinical diagnosis supported by electrophysiological findings on NCS/EMG Clinical features of radiculopathy in general: Motor or sensory involvement restricted to the distribution of an isolated nerve root level e.g. C5 nerve rooth or L5 nerve root. The motor involvement may include weakness, atrophy or rarely faciculations in a myotome distribution. The … Continue reading Radiculopathy →

Polyradiculopathy

Synonyms: Polyradiculoneuropathy Diagnosis: The diagnosis is clinical supported by electrophysiological studies (NCS/EMG) Clinical features: A syndrome with involvement of multiple nerve roots and peripheral nerves. Usually equal proximal and distal weakness with reduced reflexes Findings on investigations: +NCS: Symmetrical i.e. <50% difference between sides Decreased SNAP Decreased CMAP Motor conduction velocities: mild decrease (remaining >75% … Continue reading Polyradiculopathy →

Brachial Plexopathy

Diagnosis: The diagnosis is made based on clinical features supported by neurophysiology (NCS/EMG) Findings on investigations: Neurophysiology (NCS/EMG) SNAP: More sensitive than CMAP Normal conduction velocity and distal latency Decreased amplitude in affected nerve (may be normal initially). CMAP: Indicates severe injury Decreased amplitude Conduction block distal to Erb’s point (i.e. amplitude is reduced at … Continue reading Brachial Plexopathy →

Small Fibre Sensory Neuropathy SFSN

Synonyms: Small fibre sensory neuropathy SFSN a.k.a. small fibre neuropathy SFN Clinical features: Small fibre involvement: Neuropathic pain; allodynia, hyperalgesia Reduced sensation to pin prick and temperature. Absence of large fibre involvement: light touch, vibratory, proprioceptive sensory loss or absent deep tendon reflexes Normal motor exam Findings on Investigations: +Electrodiagnosis NCS (sensory +motor +F waves) … Continue reading Small Fibre Sensory Neuropathy SFSN →

Polyneuropathy

Synonyms: Distal symmetric polyneuropathy Diagnosis: This is a clinical diagnosis supported by electrophysiologic (nerve conduction studies and electromyography) testing. The underlying cause may be determined based on blood tests and other testing. Clinical features: Subtypes: sensory, senosrimotor, autonomic or combination. It may be axonal or demyelinating. Axonal forms are more common. Typical features: Distal symmtric … Continue reading Polyneuropathy →

Mononeuropathy

Synonyms: Mononeuropathy, including Compressive neuropathy a.k.a. nerve compression Diagnosis: This is a clinical diagnosis supported by electrophysiology (NCS/EMG) Clinical features: Dysfunction limited to one isolated peripheral nerve for example the median nerve, the ulnar nerve, the radial nerve, the femoral nerve, the peroneal nerve etc. Only one nerve involved. This distinguishes the syndrome from mononeuropathy … Continue reading Mononeuropathy →

Cryoglobulinemia, Neurological Manifestations

Diagnosis: Clinical features plus isolation of cryoglobulins Clinical features: Hepatitis Purpura (legs), arthralgia, glomerulonephritis Raynaud’s phenomenon Peripheral neuropathy, precipitated by cold +serum Cryoglobulins: positive Type I cryoglobulin: Monoclonal protein without rheumatoid factor activity Associated with plasma cell dyscrasia Type II mixed cryoglobulin MC (formerly essential mixed cryoglobulinemia): Monoclonal protein with rheumatoid factor activity +polyclonal component … Continue reading Cryoglobulinemia, Neurological Manifestations →

Lumbar spinal stenosis

Synonyms: formerly ‘pseudoclaudication’ Diagnosis: Any developmental or acquired narrowing of the spinal canal (spinal canal stenosis), nerve root canal, or intervertebral foramina a.k.a. neural foramina, that results in compression of neural elements Clinical features: (must be present) Pain & numbness in lower back, buttocks & legs on walking or lumbar extension History: Painful gait, Absence … Continue reading Lumbar spinal stenosis →

Spondylolisthesis and Spondylolysis

Diagnosis: These are imaging findings. Whether they are the cause of deficits requires clinical correlation plus electrophysiology as necessary Spondylolisthesis: Slipping of one vertebra upon another Spondylolysis: a bony defect (fracture) in the pars interarticularis (connects the pedicle and lamina on axial plane, connects superior and inferior articular processes of the facet joints on sagittal … Continue reading Spondylolisthesis and Spondylolysis →

Cervical Spondylosis

Note this can cause myelopathy and radiculopathy Synonyms: a.k.a. osteoarthropathy of the cervical spine Diagnosis: Imaging confirms the presence of degenerative changes but doesn’t confirm that it is the cause of radiculopathy or myelopathy X-ray spine: Osteophytes, narrowed disk spaces, narrowed framina This confirms the presence of degenerative changes but doesn’t confirm that it is … Continue reading Cervical Spondylosis →

Subacute Combined Degeneration of the Spinal Cord

Diagnosis: Low B12 level or normal lower limit B12 with high methylmalonic acid MRI T2: expanded spinal cord, high signal in posterior columns Pathology: Shrunken thoracic cord, discoloured posterior & lateral columns Microscopically: Thoracic cord, bilateral symmetrical white matter vacuolar degeneration “spongy appearance” affecting the long tracts, lipid-laden macrophages, Wallarian degeneration of some axons. Gliosis … Continue reading Subacute Combined Degeneration of the Spinal Cord →

Syringomyelia

Diagnosis: Clinical features plus MRI Clinical features: Suspended sensory level (loss of pain & temperature at a level on both sides), Later on absent reflexes & weakness Scoliosis, foot deformity (pes cavus, equinovarus), Charcot joint at the shoulder may occur Findings on Investigations: MRI: Cavity within the spinal cord, same signal characteristics as CSF Associated … Continue reading Syringomyelia →

Chronic Inflammatory Demyelinating Polyradiculopathy

Synonyms: Chronic inflammatory demyelinating polyradiculoneuropathy a.k.a. Chronic inflammatory demyelinating polyneuropathy, CIDP Diagnosis: Is by clinical findings supported by +NCS/EMG and occasionally nerve biopsy Clinical features: Clinical: >2 months, gradual onset, usually sensorimotor, arms & legs, proximal weakness & distal weakness, usually spares the cranial nerves, thickened nerves may occur. NCS/EMG Nerve conduction studies: Key features: … Continue reading Chronic Inflammatory Demyelinating Polyradiculopathy →

Lumbosacral Plexopathy

Diagnosis: This is a clinical diagnosis supported by neurophysiological (electrophysiology NCS/EMG) tests Clinical features: Weakness and sensory loss in lumbosacral distribution that spares the paraspinal muscles and is usually asymmetric in a distribution localizing to the plexus. Lumbar plexopathy: Loss of knee reflex Weakness: hip flexors, hip adductors, knee extensors, Reverse straight leg sign may … Continue reading Lumbosacral Plexopathy →

Guillain-Barre Syndrome

Guillain-Barre Syndrome (GBS) is an acute inflammatory polyradiculopathy. It is immune mediated and causes ascending weakness in the limbs, respiratory failure and autonomic instability. Synonyms: Landry-Guillain-Barre-Strohl syndrome Clinical features: Progressive ascending weakness in both legs & arms & areflexia Relative symmetry, autonomic dysfunction, mild sensory symptoms Findings on Investigations: CSF analysis: Albuminocytological dissociation after one … Continue reading Guillain-Barre Syndrome →

Nerve 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, … Continue reading Nerve diseases →

Multiple Sclerosis

This is an autoimmune disease of the central nervous system white matter. There is some evidence that the grey matter may be involved in the disease, but it is best to think of it as a type of white matter disease. The disease is multifocal i.e. it affects non-adjacent parts of the white matter. The … Continue reading Multiple Sclerosis →

Myelopathy

  Approaches: Localization by level of lesion: Cervical Thoracic Lumbar (Conus medullaris and cauda equina syndrome) Localization by cross-sectional origin of pathology: Extradural Intradural extramedullary Intramedullary (within the spinal cord) Anatomical syndromes Anterior cord syndrome Central cord syndrome Transverse myelitis Brown-Sequard syndrome   Anatomical syndrome approach: Anterior cord syndrome: Anatomy: lesion at anterior aspects of … Continue reading Myelopathy →