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Weakness and Hypotonia. Dr. William W. C. Young Pediatric Neurologist. Unending List of Causes of Weakness. Duchenne muscular dystrophy Becker’s muscular dystrophy Pompe’s disease McArdle’s disease Carnitine palmitoyl transferase deficiency Medium chain acyl-coA DHG def

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Weakness and hypotonia

Weakness and Hypotonia

Dr. William W. C. Young

Pediatric Neurologist


Unending list of causes of weakness
Unending List of Causes of Weakness

Duchenne muscular dystrophy

Becker’s muscular dystrophy

Pompe’s disease

McArdle’s disease

Carnitine palmitoyl transferase deficiency

Medium chain acyl-coA DHG def

Cushing’s disease

Nemaline rod myopathy

Dermato myositis

Meningitis

Hypoxic ischemic encephalopathy

Cerebral palsy

MELAS

MERRF

Adrenoleukodystrophy

Gliomatosis cerebri

Obstructive hydro cephalus

Status epilepticus

Substance abuse

Uremia

Liver failure

Multiple sclerosis

Hyothyroidism

Tay Sach’s disease

Botulism

Myasthenia gravis

Organophospha toxicity

Magnesium toxicity

Eaton Lambert syndrome

Endplate AchR deficiency

Endplate AchE deficiency

Choline acetyl transferase deficiency

Sea snake venom

Gentamycin toxicity

Viper venom

Guillain Barre syndrome

Diphtheria

Vincristine neuropthy

Diabetic neuropthy

Polyarteritis nodosa

Metachromatic leuko dystrophy

Cockayne syndrome

Charcot Marie Tooth disease

Refsum’s disease

Vitamin E deficiency

Spinal muscular atrophy

Poliomyelitis

Werdnig Hoffman disease

Kugelberg Welander disease

Cold exposure

Radiation sickness

Fabry’s disease

Herpes zoster

Lyme disease

Hepatitis B

Porphyria

Mechanical trauma

Acromegaly

Thallium toxicity

Arsenic toxicity

Syringomyelia

Transverse myelitis

C1-C2 sub luxation

Spinal cord infarction

Vertebral injury

Spinal canal hematoma

AV malform

Tabes dorsalis

Subacute combine degener

Varicella myelopthy

Syringobulbia

Devic’s disease

Acute Dissem EncephM


Systematic neurologic evaluation
Systematic Neurologic Evaluation

  • Identify the problem accurately

  • Localize the lesion

  • Derive a differential diagnosis


E g abnormal movements
e.g., Abnormal Movements

  • Identify the problem accurately

    • Seizures?

    • Dyskinesias? (tremors, tics, chorea)

    • Unsteadiness?

    • Stereotypic movements?

  • Localize the lesion

  • Derive a differential diagnosis


E g abnormal movements1
e.g., Abnormal Movements

  • Identify the problem accurately

  • Localize the lesion

    • Central or peripheral or spinal cord?

    • Nerve, neuromuscular junction, or muscle?

  • Derive a differential diagnosis


E g abnormal movements2
e.g., Abnormal Movements

  • Identify the problem accurately

  • Localize the lesion

  • Derive a differential diagnosis

    • Acute (infectious, traumatic, toxic-metabolic)


E g abnormal movements3
e.g., Abnormal Movements

  • Identify the problem accurately

  • Localize the lesion

  • Derive a differential diagnosis

    • Acute (epileptic, infectious, traumatic, toxic-metabolic, vascular-ischemic)

    • Chronic (endocrine, degenerative, neoplastic, chronic toxicity, nutritional, autoimmune, congenital, systemic)


Two cases
Two Cases

  • Two year old female that is stumbling

  • 18 month old male that is not walking


Stumbling two year old female
Stumbling two year old female

  • Onset three days ago, unchanged

  • Recent respiratory infection 4 weeks ago

  • Prior history unremarkable

  • No toxic exposure, no recent travel, no adventure in the woods, no med use

  • No history of trauma

  • Normal developmental milestones


Stumbling two year old female1
Stumbling two year old female

  • Speaking in short phrases

  • No dysmorphic features, normal head circ

  • Normal cranial nerves

  • No nystagmus, no unsteadiness

  • No abnormal movements

  • Normal reflexes

  • Decreased movements in left leg, pain in left calf with squeezing


Stumbling two year old female2
Stumbling two year old female

  • Identify the problem accurately

  • Localize the lesion

  • Derive a differential diagnosis


Stumbling two year old female3
Stumbling two year old female

  • Identify the problem accurately

    • Weakness


Stumbling two year old female4
Stumbling two year old female

  • Identify the problem accurately

    • Weakness

    • Pain

    • Ataxia

    • Vertigo


Stumbling two year old female5
Stumbling two year old female

  • Identify the problem accurately

    • Weakness in left leg

    • Pain in left calf

    • Ataxia

    • Vertigo


Stumbling two year old female6
Stumbling two year old female

  • Identify the problem accurately

    • Weakness and pain in left leg

  • Localize the lesion

    • Central

    • Spinal cord

    • Neuromuscular


Stumbling two year old female7
Stumbling two year old female

  • Identify the problem accurately

    • Weakness and pain in left leg

  • Localize the lesion

    • Central

    • Spinal cord

    • Neuromuscular (anterior horn cell, nerve, neuromuscular junction, muscle?)


Stumbling two year old female8
Stumbling two year old female

  • Identify the problem accurately

    • Weakness and pain in left leg

  • Localize the lesion

    • Central

    • Spinal cord

    • Neuromuscular (anterior horn cell, nerve, neuromuscular junction, muscle?)


Stumbling two year old female9
Stumbling two year old female

  • Identify the problem accurately

    • Weakness and pain in left leg

  • Localize the lesion

    • Neuromuscular (muscle—serum CK 2000)

  • Derive a differential diagnosis


Stumbling two year old female10
Stumbling two year old female

  • Identify the problem accurately

    • Weakness and pain in left leg

  • Localize the lesion

    • Neuromuscular (muscle—serum CK 2000)

  • Derive a differential diagnosis

    • Acute vs Chronic?


Stumbling two year old female11
Stumbling two year old female

  • Identify the problem accurately

    • Weakness and pain in left leg

  • Localize the lesion

    • Neuromuscular (muscle—serum CK 2000)

  • Derive a differential diagnosis

    • Acute (epileptic, infectious, traumatic, toxic-metabolic, vascular-ischemic)


Stumbling two year old female12
Stumbling two year old female

  • Not Chronic

    • dystrophinopathy (Duchenne, Becker’s)

    • congenital myopathy (Nemaline rod, centronuclear)

    • inflammatory myopathy (dermatomyositis)

    • metabolic myopathy (MELAS, MERFF, MCAD def, CPT deficiency, Pompe’s, McArdle’s)


Stumbling two year old female13
Stumbling two year old female

  • Differential diagnosis

    • anterior horn cell (poliomyelitis)

    • neuropathy (Guillain Barre syndrome)

    • neuromuscular junction (Myasthenic crisis, Botulism, organophosphate poisoning)

    • Muscle (trauma, inflammation, infection, ischemia)


Stumbling two year old female14
Stumbling two year old female

  • Identify the problem accurately

    • Weakness and pain in left leg

  • Localize the lesion

    • Neuromuscular (muscle—serum CK 2000)

  • Derive a differential diagnosis

    • Acute (epileptic, infectious, traumatic, toxic-metabolic, vascular-ischemic)

  • POST INFECTIOUS MYOSITIS


Delayed walking 18 month male
Delayed walking 18 month male

  • No acute changes

  • Normal prenatal and birth history

  • No chronic medical problems, no meds

  • No hospitalizations, no surgeries

  • No toxic exposure, no recent travel, no adventure in the woods, no med use

  • No recent history of trauma or infection


Delayed walking 18 month male1
Delayed walking 18 month male

  • Normal head size, no dysmorphic features, no neurocutaneous markers

  • Normal cranial nerves

  • Has 20 word vocabulary, understands verbal

  • No abnormal movements or postures

  • Cruising along furniture

  • Can appose thumbs to radii, can dorsiflex ankles, has vertical slip, some draping with horizont suspension

  • Normal reflexes, mild head lag


Delayed walking 18 month male2
Delayed walking 18 month male

  • Identify the problem accurately

  • Localize the lesion

  • Determine the mechanism of action (to derive a reasonable differential diagnosis


Delayed walking 18 month male3
Delayed walking 18 month male

  • Identify the problem accurately

    • Weakness

    • Pain

    • Ataxia

    • Vertigo


Delayed walking 18 month male4
Delayed walking 18 month male

  • Identify the problem accurately

    • Not weak

    • Not pain

    • Not ataxic

    • No vertigo

    • Hypotonic


Delayed walking 18 month male5
Delayed walking 18 month male

  • Identify the problem accurately

    • Weakness

    • Pain

    • Ataxia

    • Vertigo

    • Hypotonia?

    • Physiologic?


Delayed walking 18 month male6
Delayed walking 18 month male

  • Identify the problem accurately

    • Hypotonia without weakness

  • Localize the lesion

    • Central

    • Spinal cord

    • Neuromuscular


Delayed walking 18 month male7
Delayed walking 18 month male

  • Identify the problem accurately

    • Hypotonic without weakness

  • Localize the lesion

    • Not central (normal verbal and social development, normal head circ, no dysmorphic features, no neurocutaneous markers )


Delayed walking 18 month male8
Delayed walking 18 month male

  • Identify the problem accurately

    • Hypotonic without weakness

  • Localize the lesion

    • Not central (normal verbal and social development, normal head circ, no dysmorphic features, no neurocutaneous markers )

    • Not spinal cord (no paraplegia, no weakness)


Delayed walking 18 month male9
Delayed walking 18 month male

  • Identify the problem accurately

    • Hypotonic without weakness

  • Localize the lesion

    • Not central (normal verbal and social development, normal head circ, no dysmorphic features, no neurocutaneous markers )

    • Not spinal cord (no paraplegia, no weakness)

    • Neuromuscular? (no weakness, normal reflexes, normal muscle bulk)


Delayed walking 18 month male10
Delayed walking 18 month male

  • Identify the problem accurately

    • Hypotonia without weakness

  • Localize the lesion

    • Not central, not spinal cord, ?neuromuscular

      • Serum CK 40 normal

      • TSH normal

      • ESR 3 normal

      • Lactate 1.2 normal


Delayed walking 18 month male11
Delayed walking 18 month male

  • Identify the problem accurately

    • Hypotonic without weakness

  • Localize the lesion

    • Not central, not spinal cord, ?neuromuscular

  • Derive a differential diagnosis

    • Acute vs chronic?


Delayed walking 18 month male12
Delayed walking 18 month male

  • Identify the problem accurately

    • Hypotonic without weakness

  • Localize the lesion

    • Not central, not spinal cord, ?neuromuscular

  • Derive a differential diagnosis

    • Acute vschronic?


Delayed walking 18 month male13
Delayed walking 18 month male

  • Differential diagnosis

    • Muscle (dystrophinopathy, congenital myopathy, inflammatory myopathy, metabolic myopathy) (Duchenne, Becker’s)

    • Neuromuscular junction (chronic Botulism, myasthenia, organophosphate poisoning)

    • Nerve (Guillain Barre syndrome, diphtheria, poliomyelitis, Charcot Marie Tooth)

    • Brain (genetic disorders, hypotonic cerebral palsy, microcephaly, macrocephaly, hypothyroidism)


Delayed walking 18 month male14
Delayed walking 18 month male

  • Identify the problem accurately

    • Hypotonic without weakness

  • Localize the lesion

    • Not central, not spinal cord, ?neuromuscular

  • Derive a differential diagnosis

    • Acute vschronic?


Delayed walking 18 month male15
Delayed walking 18 month male

  • Identify the problem accurately

    • Hypotonic without weakness

  • Localize the lesion

    • Not central, not spinal cord, ?neuromuscular

  • Derive a differential diagnosis

    • Acute vschronic?

  • Something distal to the muscle?


Delayed walking 18 month male16
Delayed walking 18 month male

  • Identify the problem accurately

    • Hypotonic without weakness

  • Localize the lesion

    • Not central, not spinal cord, ?neuromuscular

  • Derive a differential diagnosis

    • Acute vschronic?

  • LIGAMENTOUS LAXITY

    • Physiologic

    • Ehlers Danlos syndrome, Cutis Laxa


Hypotonia archaic terms
Hypotonia (archaic terms)

  • Infantile progressive spinal muscular atrophy (Wernig 1891, Hoffman 1893)

  • Myotonia congenita (Oppenheim 1900)

  • Amyotonia congenita (Collier and Wilson 1908)

  • Benign congenital myopathy (Batten 1903, turner 1940)

  • Congenital universal muscular hypoplasia (Krabbe 1947)

  • Infantile muscular atrophy (Greenfield and Stern 1927)

  • Amyotonia congenita symptom complex (Brandt 1950)

  • Primary (essential) hypotonia (Sobel 1926)

  • Benign congenital hypotonia (Walton 1956)


Non neuromuscular causes of hypotonia
Non-Neuromuscular Causes of Hypotonia

  • Disorders of the central nervous system

  • Connective tissue disorders

  • Genetic disorders

  • Metabolic; nutritional; endocrine


Non neuromuscular causes of hypotonia1
Non-Neuromuscular Causes of Hypotonia

  • Disorders of the central nervous system

    • Non-specific mental deficiency

    • Birth trauma, intracranial hemorrhage, neonatal hypoxic ischemic encephalopathy

    • Hypotonic cerebral palsy

    • Metabolic disorders; lipidoses; leukodystrophies, mucopolysaccharidoses, aminoacidurias, mitochondrial disorders


Non neuromuscular causes of hypotonia2
Non-Neuromuscular Causes of Hypotonia

  • Connective tissue disorders

    • Congenital laxity of ligaments

    • Ehlers-Danlos syndromes

    • Cutis laxa

    • Marfan syndrome

    • Osteogenesis imperfecta

    • Arachnodactyly

    • Loeys-Dietz syndrome

    • Camurati-Engelman syndrome


Ehlers danlos syndromes
Ehlers Danlos syndromes

  • EDS I – severe

  • EDS II – mild

  • EDS III – hypermobile joints

  • EDS IV – vascular

  • EDS V – X linked type

  • EDS VI – ocular, scoliosis

  • EDS VII –arthrochalasis multiplex congenita

  • EDS VIII – periodontitis

  • EDS IX – copper transport disorder; X-linked cutis laxa

  • EDS X – fibronectin abnormality

  • EDS XI – familial hypermobility syndromes






Non neuromuscular causes of hypotonia3
Non-Neuromuscular Causes of Hypotonia

  • Genetic disorders

    • Down syndrome

    • Prader-Willi syndrome

    • Angelman syndrome

    • William syndrome

    • Miller-Dieker syndrome

    • Smith Lemli Opitz syndrome

    • Other structural chromosomal abnormalities


Non neuromuscular causes of hyotonia
Non-Neuromuscular Causes of Hyotonia

  • Metabolic; nutritional; endocrine

    • Organic acidemias

    • Hyperacalcemia

    • Rickets

    • celiac disease

    • Hypothyroidism

    • renal tubular acidosis


Maxims
Maxims

  • Most children who are weak, are hypotonic (not all)

  • Most children who are hypotonic, are not weak


Maxims1
Maxims

  • Most children who are weak, are hypotonic (not all)

    • exception is spastic cerebral palsy (spastic quadriplegia, spastic diplegia)

  • Most children who are hypotonic, are not weak

    • ligamentous laxity is the most common cause of hypotonia

    • Genetic conditions are not always associated with weakness (Down syndrome, PraderWilli syndrome, William syndrome, Smith Magenis)


Duchenne muscular dystrophy
Duchenne Muscular Dystrophy

  • Calf pseudohypertrophy (fat and connective tissue replaces normal muscle)

  • Positive Gower’s sign

  • Genetic deficiency of muscle protein dystrophin

  • 1:3500 male births

  • Large protein, with many sites for deletions, duplications, sequencing abnormalities

  • X-linked recessive inheritance

  • Serum CK over 10,000

  • Xp21.2

  • Possible complications

    • Cardiomyopathy

    • Congestive heart failure (rare)

    • Deformities

    • Heart arrhythmias (rare)

    • Mental impairment (varies, usually minimal)

    • Permanent, progressive disability

      • Decreased mobility

      • Decreased ability to care for self

    • Pneumonia or other respiratory infections

    • Respiratory failure


Guillain barre syndrome
Guillain Barre Syndrome

  • Acute inflammatory demyelinatingpolyneuropathy

  • Areflexic

  • Weakness, paresthesia, limb pain

  • Cytoalbuminogenic dissociation (CSF protein up to 1 gm after one week)

  • EMG with delayed F waves

  • IVIg or plasmaphoresis for severe or rapidly progressive cases

  • Dysautonomia risk

  • Respiratory failure risk

  • Ascending paralysis

  • Autoimmune disorder


Myasthenia gravis
Myasthenia Gravis

  • Autoimmune disorder

  • Thymectomy

  • Paralysis from myasthenic crisis and cholinergic crisis

  • Palliation and control w/acetylcholinesterase inhibitor

  • AchR antibody levels (80-90%)

  • Muscle fatiguability

  • Bulbar symptoms prominent

  • Different from congenital myasthenic syndromes


Dermatomyositis
Dermatomyositis

  • Heliotrope rash and Gottron’s papules

  • malar erythema, poikiloderma in a photosensitive distribution, violaceous erythema on the extensor surfaces, and periungual and cuticular changes.

  • Perifascicular atrophy

  • Calcinosis


Dermatomyositis1
Dermatomyositis

  • Joints (arthralgia)

  • Reticuloendothelial (lymphadenopathy, splenomegaly, hepatomegaly)

  • Respiratory(acute respiratory distress from lung parenchymal involvement)

  • Gastrointestinal (ulcers of stomach, intestines)

  • Cardiovascular (murmurs, friction rubs, EKG changes

  • Renal (albuminuria)


Pompe s disease
Pompe’s Disease

  • Autosomal recessive, 17q23

  • Glycogen storage disease type II

  • Acid maltase deficiency

  • Cardiomegaly and hepatomegaly

  • EKG with characteristic gigantic QRS complexes and very short P-R interval

  • Pseudomyotonic bursts on EMG

  • Infantile form generally fatal


Charcot marie tooth disease
Charcot Marie Tooth disease

  • Hereditary sensory motor neuropathy

  • Champagne bottle legs

  • Areflexia (neuropathy)

  • Most common inherited neurologic disorder

  • CMT1A accounts for about 60% of all autosomal dominant neuropathies, CMT2 accounts for about 22%, X-linked Charcot-Marie Tooth disease (CMTX) for about 16%, and CMT1B for approximately 1.6%.


Charcot marie tooth disease1
Charcot Marie Tooth disease

  • More common types

    • CMT1A, CMT2 (CMT2A, CMT2B, CMT2C, CMT2D, CMT2E, CMT2F, CMT2L, CMT2Po), CMT1B, CMTX

  • Rarer types

    • CMT4A, CMT4B, CMT4B2 CMT4C, CMT4D(lom), CMT4E, CMT 4F, AR-CMT2, AR-CMT2A, AR-CMT2B

  • Most associated with abnormalities of specific gene loci


Melas
MELAS

  • Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke

  • seizures, diabetes mellitus, hearing loss, cardiac disease, short stature, endocrinopathies, exercise intolerance, and neuropsychiatric dysfunction

  • Multisystem involvement: CNS, skeletal muscle, eye, cardiac muscle, and, more rarely, the GI and renal systems


Melas1
MELAS

  • Mitchondrial t-RNA abnormalities

  • 3243 A → G mutation (80% of cases) produces a severe combined respiratory chain defect in myoblasts, with almost complete lack of assembly of complex I, IV, and V, and a slight decrease of assembled complex III.

  • Altered mental status, schizophrenia, bipolar symptoms, autistic spectrum disorder


Merrf
MERRF

  • Myoclonic Epilepsy with Ragged Red Fibers

  • Mitochondrial encephalomyopathy

  • Ataxia, lactic acidosis

  • Less often: dysarthria, optic atrophy, short stature, hearing loss, dementia, and nystagmus

  • Progressive multisystem disorder

  • Mitochondrial DNA , mutation in A8344G most common, T8356C less often


Merrf1
MERRF

Normal muscle (H & E)

Ragged Red Fibers (Gomori Trichrome)


Mcardle s disease
McArdle’s disease

  • Autosomal recessive, 11q13

  • Glycogen storage disease Type V

  • Muscle phosphorylase deficiency

  • Cramps on exertion, sometimes myoglobinuria

  • Easy fatiguability in childhood and adolescence, diagnosis usually in adulthood

  • No cardiac involvement


Botulism
Botulism

  • Dysphagia, ptosis

  • Fixed dilated pupils

  • Neuromuscular junction blockade

  • Clostridium botulinum toxin A

  • Intestinal colonization under 12 months of age

  • Diplopia, dysarthria, dry mouth, sore throat, dysphonia, nystagmus, ataxia, paresthesia

  • Paralytic ileus, constipation, urinary retention, poor anal sphincter tone


Mcad deficiency
MCAD deficiency

  • Medium chain acyl-coA DHG, chrom 1p31

  • Fatty acid metabolism difficulty

  • Exaggerated lethargy accompanied by vomiting and acidosis with previous viral illness, quick recovery with IV fluids

  • No ketones on urinalysis

  • increased preprandial irritability, lethargy, jitteriness, sweating, and, possibly, seizures, which are all symptomatic of hypoglycemia.


Spinal muscular atrophy
Spinal Muscular Atrophy

  • Werdnig Hoffman (type 1), onset 2-3 months

  • Kugelberg Welander (type 3)

  • Autosomal recessive, 5q11-q13

  • Tongue fasciculations, areflexia, dysphagia

  • Facial muscles spared

  • Diaphragmatic breathing

  • Mild arthrogryposis


ADEM

  • Somewhat different than Multiple Sclerosis, 10% do progress to Multiple Sclerosis

  • Postinfectious from Herpes simplex, HHV6, CMV, EBV, varicella, Mycoplasma

  • Immunizations for rabies, pertussis, measles, mumps, tetanus, influenza

  • White matter demyelination at nexus with cortical gray matter

  • 1.5% mortality, seizures at onset in 25%

  • Treatment with IVIg, IV cyclosporin, high dose steroids, plasmapharesis

  • Emotional lability (other psychopathology) leads to limbic encephalitis

  • Cannot use CSF myelin basic protein and IgG index to separate from MS


Poliomyelitis
Poliomyelitis

  • Poliomyelitis is an enteroviral infection that can manifest in 4 different forms: inapparent infection (90-95%), inapparent abortive disease (5-10%), nonparalytic poliomyelitis (5%), and paralytic poliomyelitis (5%)

  • Poliovirus is an RNA virus that is transmitted through the oral-fecal route or by ingestion of contaminated water. Three serotypes are able to cause human infection. The incubation period for poliovirus is 5-35 days. The viral particles initially replicate in the nasopharynx and GI tract and then invade lymphoid tissues, with subsequent hematologic spread. After a period of viremia, the virus becomes neurotropic and produces destruction of the motor neurons in the anterior horn and brainstem. The destruction of motor neurons leads to the development of flaccid paralysis, which may be bulbar or spinal in distribution.


Poliomyelitis1
Poliomyelitis

  • A 4-fold increase in the immunoglobulin G (IgG) antibody titers or a positive anti-immunoglobulin M (IgM) titer during the acute stage is diagnostic.

  • Patients who have recovered from poliomyelitis occasionally develop a postpoliomyelitis syndrome, in which recurrences of weakness or fatigue are observed and which usually involve groups of muscles that were initially affected. This postpolio syndrome may develop 20-40 years after infection with poliovirus.


Transverse myelitis
Transverse Myelitis

  • More common, older than 5 years of age

  • Initial discomfort and pain, with weakness

  • 80% thoracic, 10% cervical

  • Some with fever and meningismus

  • Paraplegia, sensory loss, sphincter dysfunction, bilateral weakness, sensory level

  • MRI shows T2 increased signal in affected level of spinal cord, swelling

  • CSF pleocytosis in 25%, increased CSF protein in 50%

  • Steroids, IVIg, plasmapharesis do not help

  • 80-90% recovery, 50% with excellent recovery

  • Consider Devic’s disease when accompanied with optic neuritis


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