Neuropathies with HIV Infection

Seroconversion

Guillain-Barre syndrome

Chronic inflammatory demyelinating polyneuropathy (CIDP).

Symptomatic stage: Mononeuritis multiplex axonal type subacute or chronic

Late symptomatic stage: Distal symmetrical sensory polyneuropathy, most common neuropathy

frequently coexists with symptomatic encephalopathy and myelopathy

Toxic polyneuropathy (drugs)

Subacute asymmetrical polyneuropathy of cauda equina, caused by cytomegalovirus.

HEREDITARY NEUROPATHIES

Neuropathy is the sole or primary part of the disease Neuropathy is part of a more generalized neurological or multisystem disorder

Charcot-Marie-tooth disease—CMT1 (demyelinating) and

CMT2 (axonal)

HMSN-III (or Dejerine–Sottas neuropathy)

Hereditary sensory and autonomic neuropathy (HSAN)

Spinocerebellar atrophy (SCA)—Friedreich ataxia (FA)

Hereditary spastic paraplegia neuropathy (i.e. complicated HSP,

HMSN 5)

Familial amyloid (transthyretin, gelsolin, ApoA1)

Distal hereditary motor neuropathy (dHMN)

Hereditary brachial plexus neuropathy (HBPN)

Hereditary neuropathy with liability to pressure palsies

(HNPP)

Leukodystrophy

Lipoprotein deficiency

Porphyrias

APPROACH TO A PATIENT WITH PARKINSON’S DISEASE

Idiopathic Parkinson’s Disease (Paralysis Agitans)

It is a chronic, progressive disorder in which idiopathic parkinsonism occurs without evidence of more

widespread neurologic involvement.

Clinical Manifestations

Motor symptoms: Always asymmetrical in onset and become bilateral within a year (Table 6E.8).

Tremor is an early and presenting symptom in 70% of patients.

Frequency is 4–6 Hz tremor and is typically most prominent at rest and worsens with emotional

stress.

Typically tremor starts with the fingers and hands at rest.

Often described as pill rolling of finger and wrist, because the patient appears to be rolling

something between thumb and forefinger.

Disappears on voluntary movement and sleep.

Rigidity:

Stiffness on passive limb movement is described as “lead pipe” rigidity because the increase in

muscle tone is present throughout the range of movement. Unlike spasticity, it is not dependent

on speed of movement. When tremor is superimposed on the rigidity, a ratchet like jerkiness is felt, described as

“cogwheel” rigidity.

Akinesia or bradykinesia

Poverty/slowing of movement is the hallmark of parkinson’s disease (PD). Slowness/difficulty of

initiating voluntary movement and an associated reduction in automatic movements, such as

swinging of the arms when walking.

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There is fixity of facial expression (facial immobility—mask like face) with widened palpebral

fissures and infrequent blinking.

Repetitive tapping (at about 2 Hz) over the glabella (glabellar tap) produces a sustained blink

response (Myerson’s sign), in contrast to the response of normal subject.

Postural changes: A stooped posture is a characteristic feature.

Gait changes: Slow shuffling, freezing and reduced arm swing, small stride length, slow turns,

festinating gait (tendency to advance rapid short steps) and catching center of gravity. Feet may be

glued to floor. Postural instability and freezing may result in fall forward.

Reduced eye blink.

Table 6E.8: Nonmotor symptoms of Parkinson’s disease.

Autonomic dysfunction

Orthostatic hypotension

Urinary incontinence

Constipation

Sexual problems

Neuropsychiatric

Anxiety

Depression

Apathy

Psychosis

Dementia

Sensory problems

Reduced sense of smell (hyposmia)

Pain

Sleep disorders

Restless legs

Insomnia

Daytime somnolence

Rheumatological

Frozen shoulder

Periarthritis

Swan neck deformity

Other

Seborrhea

Flowchart 6E.4: Classification of Parkinsonsian disorder.

(MPTP: manganese, 1-methyl 4-phenyl tetrahydropyridine; HIV: human immunodeficiency virus)

Table 6E.9: Hoehn and Yahr stage of Parkinson’s disease.

Stage Disease state

I Unilateral involvement only, minimal or no functional impairment

II Bilateral or midline involvement, without impairment of balance

III First sign of impaired righting reflex, mild to moderate disability

IV Fully developed, severely disabling disease; patient still able to walk and stand unassisted

V Confinement to bed or wheelchair unless aided

Table 6E.10: Causes of secondary Parkinsonism.

Toxin: Manganese, 1-methyl 4-phenyl -1,2,3,6-tetrahydropyridine

(MPTP), carbon monoxide, manganese, mercury, carbon disulfide,

cyanide, methanol

Viral: Encephalitis lethargica, Creutzfeldt-Jakob disease

Metabolic: Wilson’s disease

Drugs: Dopamine receptor blocking drugs, reserpine,

tetrabenazine, alpha methyl dopa, lithium, flunarizine,

cinnarizine

Vascular: Multi-infarct, Binswangers disease

Trauma: Pugilistic encephalopathy

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Head injury: Punch drunk syndrome

Infectious: Postencephalitic, human immunodeficiency virus (HIV),

subacute sclerosing panencephalitis (SSPE), Prion diseases

Others: Parathyroid abnormalities, hypothyroidism, brain

tumors, paraneoplastic, normal pressure hydrocephalus

(NPH), psychogenic

Table 6E.11: Parkinson plus syndromes and its features.

Syndrome Features

Progressive

supranuclear palsy

(PSP, SteeleRichardson-Olszewski

syndrome)

Slow ocular saccades, eyelid apraxia, and restricted eye movements with particular impairment of

downward gaze and reptilian stare [Fig. 6D(iii).37]. Frequently experience hyperextension of the neck

with early gait disturbance and falls. MRI may reveal a characteristic atrophy of the midbrain with relative

preservation of the pons (the ‘hummingbird sign’ on midsagittal images)

Multiple-system

atrophy (MSA)

Parkinsonian (MSAP) or striatonigral

degeneration

Cerebellar (MSA-C)

or

olivopontocerebellar

atrophy

Autonomic (MSA-A)

form or Shy-Drager

syndrome

Parkinsonism in conjunction with cerebellar signs and/or early and prominent autonomic dysfunction,

usually orthostatic hypotension.

Cerebellar and brainstem atrophy (the pontine ‘hot cross buns’ sign in MSA-c)

Corticobasal

ganglionic

degeneration

(Rebeitz-KolodnyRichardson

syndrome)

Asymmetric dystonic contractions and clumsiness of one hand coupled with cortical sensory

disturbances manifest as apraxia, agnosia, focal myoclonus, or alien limb phenomenon

Dementia with lewy

bodies

Early onset dementia, visual hallucinations

Parkinsonismdementia complex of

Guam

Motor neuron disease plus Parkinson’s

Guadeloupean

parkinsonism

Levodopa-unresponsive parkinsonism, postural instability with early falls, and pseudobulbar palsy

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A. CASE SHEET FORMAT

HISTORY TAKING

Name:

Age:

Sex:

Residence:

Occupation:

Chief Complaints

________ × days

________ × days

________ × days

History of Presenting Illness

Joint pain:

Duration:

Onset:

No. of joints involved:

Symmetry:

Progression:

Variation:

Aggravating factors:

Relieving factors:

Morning stiffness:

Duration of stiffness:

Onset:

Progression:

Variation:

Aggravating:

Relieving factors:

Deformities:

Duration:

Onset:

Ulcers:

Duration:

Onset:

Progression:

Fever:

Episodic or continuous

Grade

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Chill and rigors

Aggravating factors

Relieving factors

Variation

Diurnal variation

History of:

Petechiae

Purpura

Other bleeding manifestations

Breathing difficulty

Dyspnea on exertion

Numbness and tingling of legs

Skin lesions

Endocrine abnormalities

Past history:

Asthma

Chronic obstructive airway disease

Tuberculosis

History of contact with tuberculosis

Diabetes mellitus (DM)

Hypertension (HTN)

Ischemic heart disease (IHD)

Seizure disorder

Family history:

(Draw pedigree chart representing three generations)

Personal history:

Bowel habits

Bladder habits

Appetite

Loss of weight

Occupational exposure

Sleep

Dietary habits and taboo

Food allergies

Smoking (in smoking Index or Pack years)

Alcohol history (__ grams of alcohol/day or ___ units of alcohol/week)

Menstrual and obstetric history:

G__P__L__A__

Age of menarche __

Menopause at __

Flow – Amenorrhea/Oligomenorrhea/Menorrhagia

Summarize:

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